Mutations in COL1A1

This page is now frozen. No new data will be added. No further corrections will be made.

The COL1A1 mutation data are now in a new database.

New data last added: 15 Febraury, 2008
Corrections and other changes 1 August, 2008


As far as possible, the mutations are reported according to current guidelines, though some old data still need to be re-formatted.

Please refer to the nomenclature page for a complete description of how mutations in this gene are numbered.

An anotated cDNA and protein sequence alignment using both the systematic and historical numbering of the amino acids is available for download.

The data are organised in tables representing the four major classes of mutation. Click on one of the following to move directly to mutations of a particular type.

Amino acid substitutions
RNA splicing mutations
Deletions, insertions, duplications, and frameshifts
Polymorphisms


Table 1. Amino acid substitutions in COL1A1.

Return to the start of COL1A1.


Mutation                  Phenotype      Reference(s)
GGC->CGC                  OI II          Pollitt et al. Hum Mutat Mutation in Brief #901 2006 Online
c.64G>C
p.Gly22Arg


Gly13Asp                  OI IV/EDS      Cabral et al. J Biol Chem E-pub 2005
GGC->GAC
c.572G>A
p.Gly191Asp


Gly13Ala                  Cervical       Mayer et al. Neurology 47:552-556 1996
GGC->GCC                  artery
c.572G>C                  dissections
p.Gly191Ala


Gly16Arg                  OI I           Lee et al. Human Mutat Mutation in Brief #894 2006 Online
GGT->CGT
c.580G>C
p.Gly194Arg


Gly19Cys                  Osteoporosis   Nicholls et al. IV Int Conf on OI 48 1990 (abstract)
GGT->TGT                  OI I           Lund et al. J Craniofac Genet Dev Biol 18:30-37 1998
c.589G>T
p.Gly197Cys


Gly19Arg                  OI IV          Pollitt et al. Hum Mutat Mutation in Brief #901 2006 Online
GGT->CGT
c.589G>C
p.Gly197Arg


Gly25Val                  OI IV/EDS      Cabral et al. J Biol Chem E-pub 2005
GGT->GTT                  OI IV/EDS      Cabral et al. J Biol Chem E-pub 2005
c.608G>T                  OI III         Venturi et al. Clin Genet 70:131-139 2006
g.3930G>T
p.Gly203Val


Pro27Ala                  Osteopaenia(?) Spotila et al. J Bone Mineral Res 9:923-932 1994
CCT->GCT                  Osteopaenia(?) Spotila et al. J Bone Mineral Res 9:923-932 1994
c.613C>G
p.Pro205Ala


Gly34Arg                  OI III/IV/EDS  Cabral et al. J Biol Chem E-pub 2005
GGA->AGA
c.634G>A
p.Gly212Arg


Arg42Stop                 OI I           Körkkö et al. Am J Hum Genet 62:98-110 1998
CGA->TGA
c.658C>T
p.Arg220X


Gly43Cys                  OI I           Shapiro et al. J Clin Invest 89:567-573 1992
GGT->TGT
c.661G>T
p.Gly221Cys


Gly46Cys                  OI I           Byers et al. J Med Genet 28:433-442 1991
GGT->TGT
c.670G>T
p.Gly224Cys


Gly64Asp                  OI ?           Lee et al. Human Mutat Mutation in Brief #894 2006 Online
GGT->GAT
c.725G>A
p.Gly242Asp


Arg75Stop                 OI I           Ries-Levavi et al. Hum Mutat Mutation in Brief #701 2004 Online
CGA->TGA                  OI I           Venturi et al. Clin Genet 70:131-139 2006
c.757C>T
g.4856C>T
p.Arg253X


Gly76Glu                  OI III         Cabral et al. Mol Genet Metab 72:326-335 2001
GGA->GAA                  OI III/EDS     Cabral et al. J Biol Chem E-pub 2005
c.761G>A
p.Gly254Glu


Gly79Arg                  OI I           Redford-Badwal et al. J Clin Invest 97:1035-1040 1996
GGA->AGA                  OI I           Gat-Yablonski et al. Hum Genet 101:22-25 1997
c.769G>A                  OI IA          Benušienė and Kučinskas J Appl Genet 44:95-102 2003
g.4868G>A                 OI IA          Benušienė and Kučinskas J Appl Genet 44:95-102 2003
p.Gly257Arg               OI III         Benušienė and Kučinskas J Appl Genet 44:95-102 2003
                          OI I           Ries-Levavi et al. Hum Mutat Mutation in Brief #701 2004 Online
                          OI IV          Lee et al. Human Mutat Mutation in Brief #894 2006 Online
                          OI IV          Venturi et al. Clin Genet 70:131-139 2006

Gly85Arg                  OI I           Deak et al. J Biol Chem 266:21827-21832 1991
GGA->AGA
c.787G>A
p.Gly263Arg


Gly85Val                  OI I           Valli et al. Eur J Biochem 217:77-82 1993
GGA->GTA
c.788G>T
p.Gly263Val


Gly88Glu                  OI III/IV/EDS  Cabral et al. J Biol Chem E-pub 2005
GGA->GAA                  OI I           Kataoka et al. Pediatr Int 49:564-569 2007
c.797G>A
p.Gly266Glu


Gly94Cys                  OI I           Starman et al. J Clin Invest 84:1206-1214 1989
GGT->TGT
c.814G>T
p.Gly272Cys


Gly97Asp                  OI II          Lightfoot et al. J Biol Chem 267:25521-25528 1992
GGT->GAT
c.824G>A
p.Gly275Asp


Gly109Ser                 OI I           Kataoka et al. Pediatr Int 49:564-569 2007
GGT>AGT
c.859G>A
p.Gly199Ser

Gly121Arg                 OI IV          Hartikka et al. Hum Mutat 24:147-154 2004
GGT->AGT
c.895G>A
p.Gly299Arg


Gly127Arg                 OI I           Hartikka et al. Hum Mutat 24:147-154 2004
GGC->CGC
c.913G>C
p.Gly305Arg
Note: this mutation is mis-reported in Hartikka et al. (2004)


Gly127Ser                 OI IV          Ward et al. Hum Mutat Mutation in Brief #414 2001 Online
GGC->AGC
c.913G>A
p.Gly305Ser


Gly133Arg                 OI IV          Ward et al. Hum Mutat Mutation in Brief #414 2001 Online
GGT->AGT
c.931G>C
p.Gly311Arg


Arg134Cys                 EDS Classical  Nuytinck et al. Am J Hum Genet 66:1398-1402 2000
CGC->TGC                  EDS Classical  Nuytinck et al. Am J Hum Genet 66:1398-1402 2000
c.934C>T                  EDS Classical  Malfait et al. Hum Mutat 28:387-395 2007
p.Arg312Cys


Gly142Val                 OI I           Pollitt et al. Hum Mutat Mutation in Brief #901 2006 Online
GGT->GTT
c.959G>T
p.Gly320Val


Gly154Arg                 OI III         Pruchno et al. Hum Genet 87:33-40 1991
GGG->AGG                  OI III         Pruchno et al. Hum Genet 87:33-40 1991
c.994G>A                  OI I           Zhuang et al. Am J Med Genet 61:111-116 1996
p.Gly332Arg


Gly154Ala                 OI III         Krakow et al. Am J Hum Genet 59S:A266 1996 (abstract)
GGG->GCG
c.995G>C
p.Gly332Ala


Gly160Cys                 OI IV          Pollitt et al. Hum Mutat Mutation in Brief #901 2006 Online
GGC->TGC
c.1012G>T
p.Gly338Cys


Gly172Arg                 OI III         Mackay et al. Hum Mutat 3:324-326 1994
GGT->CGT
c.1048G>C
p.Gly350Arg


Gly175Cys                 OI III/IV      Wirtz et al. Connect Tissue Res  29:1-11 1993
GGT->TGT
c.1057G>T
p.Gly353Cys


Gly175Ser                 OI IV          Kataoka et al. Pediatr Int 49:564-569 2007
GGT->AGT
c.1057G>A
p.Gly353Ser


Gly178Cys                 OI I           Valli et al. J Biol Chem 266:1872-1878 1991
GGT->TGT
c.1066G>T
p.Gly356Cys


Arg183Stop                OI I           Körkkö et al. Am J Hum Genet 62:98-110 1998
CGA->TGA                  OI I           Benušienė and Kučinskas J Appl Genet 44:95-102 2003
c.1081C>T                 OI I           Ries-Levavi et al. Hum Mutat Mutation in Brief #701 2004 Online
p.Arg361X


Gly205Cys                 OI I           Byers Trends Genet 6:293-300 1990
GGC->TGC
c.1147G>T
p.Gly383Cys


Gly211Arg                 OI II          Sztrolovics et al. Bone 15:321-328 1994
GGT->CGT
c.1165G>C
p.Gly389Arg


Gly211Cys                 OI III/IV(?)   Wilcox et al. Am J Hum Genet 55S:A367 1994 (abstract)
GGT->TGT
c.1165G>T
p.Gly389Cys


Gly220Ala                 OI IV          Sarafova et al. Hum Mutat 11:395-403 1998
GGT->GCT
c.1193G>C
p.Gly398Ala


Gly220Asp                 OI II          Culbert et al. Biochem J 311:815-820 1995
GGT->GAT
c.1193G>A
p.Gly398Asp


Gly223Cys                 OI I           Byers. Trends Genet 6:293-300 1990
GGT->TGT                  OI IV          Cohen et al. Am J Hum Genet 59S:A253 1996 (abstract)
c.1201G>T
p.Gly401Cys


Gly226Cys                 OI III/IV(?)   Wilcox et al. Am J Hum Genet 55S:A367 1994 (abstract)
GGT->TGT
c.1210G>T
p.Gly404Cys


Gly229Ser                 OI III         Ward et al. Hum Mutat Mutation in Brief #414 2001 Online
GGT->AGT
c.1219G>A
p.Gly407Ser


Arg237Stop                OI I           Willing et al. Am J Hum Genet 59:799-809 1996
CGA->TGA                  OI I           Redford-Badwal et al. J Clin Invest 97:1035-1040 1996
c.1243C>T                 OI I           Ries-Levavi et al. Hum Mutat Mutation in Brief #701 2004 Online
p.Arg415X                 OI IV          Reis et al. J Appl Genet 46:105-108 2005


Pro239Ala                 OI I           Reis et al. J Appl Genet 46:105-108 2005
CCC->GCC
c.1249C>G
p.Pro417Ala


Gly244Cys                 OI II          Fertala et al. Biochem J 289:195-199 1993
GGC->TGC
c.1264G>T
p.Gly422Cys


Gly247Ser                 OI II/III      Mackay et al. Hum Mol Genet 2:1155-1160 1993
GGC->AGC
c.1273G>A
p.Gly425Ser


Gly256Val                 OI II          Patterson et al. J Biol Chem 264:10083-10087 1989
GGT->GTT
c.1301G>T
p.Gly434Val


Arg294Stop                OI I           Pollitt et al. Hum Mutat Mutation in Brief #901 2006 Online
CGA->TGA
c.1414C>T
p.Arg472X


Gly298Arg                 OI II          Byers et al. J Med Genet 28:433-442 1991
GGA->CGA
c.1426G>C
p.Gly476Arg


Gly349Cys                 OI IV          Cohen et al. VI Int Conference on OI 1996 (abstract)
GGT->TGT                  OI IV          Sarafova et al. Hum Mutat 11:395-403 1998
c.1579G>T
p.Gly527Cys


Gly349Ala                 OI I           Hartikka et al. Hum Mutat 24:147-154 2004
GGT->GCT
c.1580G>C
p.Gly527Ala


Gly352Cys                 OI IV          Cohen et al. Am J Hum Genet 59S:A253 1996 (abstract)
GGT->TGT
c.1588G>T
p.Gly530Cys


Gly352Ser                 OI IVB         Bateman et al. Biochem J 288:131-135 1992
GGT->AGT                  OI IV          Marini et al. J Biol Chem 268:2667-2673 1993
c.1588G>A                 OI III/IV      Marini et al. Am J Hum Genet 53S:A1199 1993 (abstract)
p.Gly530Ser               OI II          Mackay et al. Hum Mol Genet 2:1155-1160 1993


Gly355Asp                 OI II          Raghunath et al. Pediatr Res 36:441-448 1994
GGT->GAT
c.1597G>A
p.Gly533Asp


Pro377Arg                 OI I           Pollitt et al. Hum Mutat Mutation in Brief #901 2006 Online
CCC->CGC
c.1664C>G
p.Pro555Arg


Gly382Arg                 OI II          Cohen-Solal et al. Mol Cell Probes 10:219-225 1996
GGT->CGT
c.1678G>C
p.Gly560Arg


Gly382Ser                 OI IV          Mackay et al. Hum Mol Genet 2:1155-1160 1993
GGT->AGT                  OI IV          Reis et al. J Appl Genet 46:105-108 2005
c.1678G>A
p.Gly560Ser


Gly382Cys                 OI IV          Byers Trends Genet 6:293-300 1990
GGT->TGT
c.1678G>T
p.Gly560Cys


Gly388Arg                 OI II          Galicka et al. Acta Biochim Pol 49:443-450 2002
GGA->AGA
c.1696G>A
p.Gly566Arg


Gly391Arg                 OI II          Bateman et al. J Biol Chem 262:7021-7027 1987
GGC->CGC
c.1705G>C
p.Gly569Arg


Arg396Cys                 EDS            Malfait et al. Hum Mutat 28:387-395 2007
CGT->TGT
c.1720C>T
p.Arg574Cys 


Gly403Arg                 OI II          Pollitt et al. Hum Mutat Mutation in Brief #901 2006 Online
GGA->AGA
c.1741G>A
p.Gly581Arg


Gly415Cys                 OI III/IV      Nicholls et al. J Med Genet  28:757-764 1991
GGC->TGC
c.1777G>T
p.Gly593Cys


Gly415Ser                 OI III/IVB     Bateman et al. Biochem J 288:131-135 1992
GGC->AGC                  OI II/III      Mottes et al. Hum Mutat 2:196-204 1993
c.1777G>A
p.Gly593Ser


Arg420Stop                OI I           Siegart et al. Klin Pädiatr 216:91-93 2004
CGA->TGA
c.1792C>T
p.Arg598X


Gly424Arg                 OI II(?)       Allan Lund, personal communication
GGA->AGA
c.1804G>A
p.Gly602Arg


Gly448Cys                 OI II          Pepin et al. Prenat Diagn 17:559-570 1997
GGT->TGT
c.1876G>T
p.Gly626Cys


Gly448Ser                 OI IV          Pepin et al. Prenat Diagn 17:559-570 1997
GGT->AGT
c.1876G>A
p.Gly626Ser


Gly451Ser                 OI II          Pepin et al. Prenat Diagn 17:559-570 1997
GGC->AGC
c.1885G>A
p.Gly629Ser


Gly469Ser                 OI I/IV        Pollitt et al. Hum Mutat Mutation in Brief #901 2006 Online
GGT->AGT
c.1939G>A
p.Gly647Ser


Gly478Ser                 OI II          Mottes et al. Hum Mutat 12:71-72 1998 Online
GGC->AGC
c.1966G>A
p.Gly656Ser


Gly481Ala                 OI III         Benušienė and Kučinskas J Appl Genet 44:95-102 2003
GGT->GCT
c.1976G>C
p.Gly659Ala


Gln483Leu                 OI I           Ries-Levavi et al. Hum Mutat Mutation in Brief #701 2004 Online
CAG->CTT
c.[1982A>T; 1983G>T]
p.Gln661Leu


Glu500Stop                OI I           Benušienė and Kučinskas J Appl Genet 44:95-102 2003
GAG->TAG
c.2032G>T
p.Glu678X


Gly505Ser                 OI IV          Venturi et al. Clin Genet 70:131-139 2006
GGC->AGC
c.2047G>A
g.10038G>A
p.Gly683Ser
Note: this mutation is incorrectly described for patient 3 in Table 2 of Venturi et al.


Gly511Ser                 OI III         Galicka et al. Mol Cell Biochem 248:49-56 2003
GGT->AGT
c.2065G>A
p.Gly689Ser

Arg519Stop                OI I           Willing et al. Am J Hum Genet 59:799-809 1996
CGA->TGA                  OI I           Körkkö et al. Am J Hum Genet 62:98-110 1998
c.2089C>T
p.Arg697X


Gly523Cys                 OI IV          Sarafova et al. Hum Mutat 11:395-403 1998
GGT->TGT
c.2101G>T
p.Gly701Cys


Gly526Cys                 OI III         Starman et al. J Clin Invest 84:1206-1214 1989
GGC->TGC                  OI III         Pepin et al. Prenat Diagn 17:559-570 1997
c.2110G>T
p.Gly704Cys


Gly526Ser                 OI III         Ward et al. Hum Mutat Mutation in Brief #414 2001 Online
GGC->AGC
c.2110G>A
p.Gly704Ser


Gly541Ser                 OI III         Mackay et al. Hum Mol Genet 2:1155-1160 1993
GGT->AGT
c.2155G>A
p.Gly719Ser


Gly541Asp                 OI II          Zhuang et al. Am J Hum Genet 48:1186-1191 1991
GGT->GAT
c.2156G>A
p.Gly719Asp

Gly544Ser                 OI I           Lee et al. Human Mutat Mutation in Brief #894 2006 Online
GGC->AGC
c.2164G>A
p.Gly722Ser


Gly550Arg                 OI II          Wallis et al. Am J Hum Genet 46:1034-1040 1990
GGA->AGA
c.2182G>A
p.Gly728Arg


Gly556Val                 OI II          Pollitt et al. Hum Mutat Mutation in Brief #901 2006 Online
GGT->GTT
c.2201G>T
p.Gly734Val


Gly559Cys                 OI I & DGI     Pallos et al. Arch Oral Biol 46:459-470 2001
GGT->TGT
c.2209G>T
p.Gly737Cys


Gly559Asp                 OI II          Cohn et al. IV Int Conf on OI 47 1990
GGT->GAT
c.2210G>A
p.Gly737Asp


Gly565Ser                 OI II          Bateman et al. Biochem J 288:131-135 1992
GGT->AGT
c.2227G>A
p.Gly743Ser


Gly565Val                 OI II          Mackay et al. Hum Genet 91:439-444 1993
GGT->GTT
c.2228G>T
p.Gly743Val


Gly586Val                 OI II          Lund et al. Hum Mutat 9:431-436 1997
GGT->GTT
c.2291G>T
p.Gly764Val


Gly589Ser                 OI III         Forlino et al. Hum Mol Genet 3:2201-2206 1994
GGC->AGC                  OI IV(?)       Zhuang et al. Hum Mutat 7:89-99 1996
c.2299G>A                 OI III         Lund et al. Acta Paediatr 86:711-718 1997
p.Gly767Ser               OI III         Ries-Levavi et al. Hum Mutat Mutation in Brief #701 2004 Online
                          OI III         Hartikka et al. Hum Mutat 24:147-154 2004
                          OI III         Lee et al. Human Mutat Mutation in Brief #894 2006 Online


Gly592Ser                 OI III         Reis et al. J Appl Genet 46:105-108 2005
GGT->AGT
c.2308G>A
p.Gly770Ser


Gly595Ser                 OI III(?)      Chamberlain et al. Science 303:1198-1201 2004
GGC->AGC
c.2317G>A
p.Gly773Ser


Gly598Ser                 OI II          Westerhausen et al. J Biol Chem 265:13995-14000 1990
GGT->AGT
c.2326G>A
p.Gly776Ser


Gly601Ser                 OI III/IV      Lund et al. Acta Paediatr 86:711-718 1997
GGT->AGT
c.2335G>A
p.Gly779Ser


Gly610Cys                 OI IV          Hartikka et al. Hum Mutat 24:147-154 2004
GGC->TGC
c.2362G>T
p.Gly788Cys


Arg618His                 Connective     Superti-Furga et al. Matrix Biol 14:385 (abst) 1994
CGT->CAT                  tissue
c.2387G>A                 weakness
p.Arg796His


Gly631Ser                 OI II          Westerhausen et al. J Biol Chem 265:13995-14000 1990
GGC->AGC
c.2425G>A
p.Gly809Ser


Gly637Val                 OI II          Tsuneyoshi et al. J Biol Chem 266:15608-15613 1991
GGC->GTC
c.2444G>T
p.Gly815Val


Gly643Ser                 OI III         Lund et al. Hum Mutat 9:378-382 1997
GGT->AGT                  OI I           Wang et al. Zhonghua Yi Xue Yi Chuan Xue Za Zhi 23:192-194 2006
c.2461G>A                 OI ?           Lee et al. Human Mutat Mutation in Brief #894 2006 Online
g.11808G>A                OI III         Venturi et al. Clin Genet 70:131-139 2006
p.Gly821Ser

Gln644Stop                OI I           Liu et al. Mol Vis 13:360-365 2007
CAA->TAA
c.2464C>T
g.11811C>T
p.Gln822X


Gly649Asp                 OI IIA         Reis et al. J Appl Genet 46:105-108 2005
GGC->GAC
c.2480G>A
p.Gly827Asp


Gly655Asp                 OI II          Aerts et al. Prenat Diagn 26:393-394 2006
GGT->GAT
c.2498G>A
p.Gly833Asp


Gly661Ser                 OI III         Nuytink et al. Hum Genet 97:324-329 1996
GGC->AGC
c.2515G>A
p.Gly839Ser


Gly664Arg                 OI II          Culbert et al. Biochem J 311:815-820 1995
GGG->CGG
c.2524G>C
p.Gly842Arg


Gly667Arg                 OI II          Bateman et al. J Biol Chem 263:11627-11630 1988
GGA->AGA
c.2533G>A
p.Gly845Arg


Gly673Asp                 OI II          Cohn et al. IV Int Conf on OI 47 1990
GGC->GAC
c.2552G>A
p.Gly851Asp

Gly688Cys                 OI III         Ward et al. Hum Mutat Mutation in Brief #414 2001 Online
GGC->TGC
c.2596G>T
p.Gly866Cys


Gly688Ser                 OI III         Horwitz et al. Nat Med 5:309-313 1999
GGC->AGC                  OI III         Lund et al. Hum Mutat 13:503 1999 Online
c.2596G>A
p.Gly866Ser


Gly691Cys                 OI II          Steinmann et al. Biochem J 279:747-752 1991
GGT->TGT
c.2605G>T
p.Gly869Cys


Arg704Stop                OI I           Hartikka et al. Hum Mutat 24:147-154 2004
CGA->TGA
c.2644C>T
p.Arg882X


Gly718Cys                 OI II          Starman et al. J Clin Invest 84:1206-1214 1989
GGC->TGC
c.2686G>T
p.Gly896Cys


Gly748Cys                 OI II          Vogel et al. J Biol Chem 262:14737-14744 1987
GGT->TGT
c.2776G>T
p.Gly926Cys


Gln779Stop                OI I           Ries-Levavi et al. Hum Mutat Mutation in Brief #701 2004 Online
CAG->TAG
c.2869C>T
p.Gln957X


Arg780Leu                 OI I(?)        Sun et al. ASHG Conference 2006
CGT->CTT
c.2873G>T
p.Arg958Leu


Gly802Val                 OI II          Bonaventure et al. Hum Genet 89:640-646 1992
GGT->GTT
c.2939G>T
p.Gly980Val


Gly832Ser                 OI IV          Marini et al. J Biol Chem 264:11893-11900 1989
GGT->AGT
c.3028G>A
p.Gly1010Ser


Arg836Cys                 Caffey         Gensure et al. J Clin Invest 115:1250-1257 2005
CGT->TGT                  Disease        Gensure et al. J Clin Invest 115:1250-1257 2005        
c.3040C>T                                Gensure et al. J Clin Invest 115:1250-1257 2005
p.Arg1014Cys                             Suphapeetiporn et al. Clin Genet 71:280-284 2007
                                         Kamoun-Goldrat et al. Am J Med Genet A 146A:1820-1824 2008

Gly844Ser                 OI III         Pack et al. J Biol Chem 264:19694-19699 1989
GGT->AGT
c.3064G>A
p.Gly1022Ser


Gly844Val                 OI II          Körkkö et al. Hum Mutat 9:148-156 1997
GGT->GTT                  OI II          Allan Lund, personal communication
c.3065G>T
p.Gly1022Val


Gly847Arg                 OI II          Wallis et al. J Biol Chem 265:18628-18633 1990
GGA->AGA
c.3073G>A
p.Gly1025Arg


Arg848Stop                OI I           Ries et al. Prenat Diagn 20:876-878 2000
CGA->TGA                  OI I           Ries-Levavi et al. Hum Mutat Mutation in Brief #701 2004 Online
c.3076C>T                 OI I           Ries-Levavi et al. Hum Mutat Mutation in Brief #701 2004 Online
p.Arg1026X                OI I           Hartikka et al. Hum Mutat 24:147-154 2004
                          OI I           Hartikka et al. Hum Mutat 24:147-154 2004


Arg858Cys                 OI/EDS         Lund et al. Clin Genet 73:97-101 2008
CGT->TGT
c.3106C>T
p.Arg1036Cys


Gly862Ser                 OI IIa         Virdi et al. Hum Genet 93:287-290 1994
GGC->AGC                  OI III         Namikawa et al. Hum Genet 95: 666-670 1995
c.3118G>A                 OI III         Zhuang et al. Hum Mutat 7:89-99 1996
p.Gly1040Ser              OI III         Lund et al. Hum Mutat 9:378-382 1997
                          OI III/IV      Chamberlain et al. Science 303:1198-1201 2004


Gly868Cys                 OI III         Pepin et al. Prenat Diagn 17:559-570 1997
GGT->TGT
c.3136G>T
p.Gly1046Cys


Gly871Ser                 OI III         Lund et al. Hum Mutat 9:378-382 1997
GGT->AGT
c.3145G>A
p.Gly1049Ser


Gly880Ser                 OI IV          Lund et al. Hum Mutat 9:378-382 1997
GGT->AGT                  OI IV          Lee et al. Human Mutat Mutation in Brief #894 2006 Online
c.3172G>A
p.Gly1058Ser


Gly883Ser                 OI IV          Lightfoot et al. J Biol Chem 269:30352-30357 1994
GGC->AGC
c.3181G>A
p.Gly1061Ser


Gly883Asp                 OI II          Cohn et al. Am J Hum Genet 46:591-601 1990
GGC->GAC
c.3182G>A
p.Gly1061Asp


Arg888Cys                 OI/EDS         Cabral et al. Hum Mutat 28:396-405 2007
CGT->TGT
c.3196C>T
p.Arg1006Cys


Gly898Ser                 OI III         Lund et al. Hum Mutat 9:378-382 1997
GGT->AGT
c.3226G>A
p.Gly1076Ser


Gly901Ser                 OI I           Mottes et al. Hum Genet 89:480-484 1992
GGC->AGC                  OI I           Hartikka et al. Hum Mutat 24:147-154 2004
c.3235G>A                 OI IV          Allan Lund, personal communication
p.Gly1079Ser


Gly904Cys                 OI II          Constantinou et al. J Clin Invest 83:574-584 1989
GGC->TGC
c.3244G>T
p.Gly1082Cys


Gly910Ala                 OI II          Valli et al. Eur J Biochem 211:415-419 1993
GGA->GCA
c.3263G>C
p.Gly1088Ala


Gly913Ser                 OI II          Cohn et al. Matrix 10:236 (abst) 1990
GGC->AGC
c.3271G>A
p.Gly1091Ser


Arg915Cys                 EDS            Malfait et al. Hum Mutat 28:387-395 2007
CGT->TGT
c.3277C>T
p.Arg1093Cys

Gly928Ala                 OI II          Lamande et al. J Biol Chem 264:15809-15812 1989
GGC->GCC
c.3317G>C
p.Gly1106Ala


Gly946Cys                 OI II          Kurosaka et al. J Biochem 115:853-857 1994
GGC->TGC
c.3370G>T
p.Gly1124Cys


Arg963Stop                OI I           Willing et al. Am J Hum Genet  55:638-647 1994
CGA->TGA                  OI I           Körkkö et al. Hum Mutat 9:148-156 1997
c.3421C>T                 OI I           Körkkö et al. Hum Mutat 9:148-156 1997
g.14421C>T                OI I           Lee et al. Human Mutat Mutation in Brief #894 2006 Online
p.Arg1141X                OI I           Venturi et al. Clin Genet 70:131-139 2006


Gly964Ser                 OI II          Wallis et al. Am J Hum Genet 45S:A228 1989
GGT->AGT
c.3424C>A
p.Gly1142Ser


Gly973Ser                 OI III         Gomez-Lira et al. V Int Conf on OI 120 1993
GGC->AGC
c.3451G>A
p.Gly1151Ser


Gly973Val                 OI II          Lamande et al. J Biol Chem 264:15809-15812 1989
GGC->GTC
c.3452G>T
p.Gly1151Val


Gly976Arg                 OI II          Lamande et al. J Biol Chem 264:15809-15812 1989
GGA->CGA
c.3460G>C
p.Gly1154Arg


Gly979Asp                 OI I           Wang et al. Zhonghua Yi Xue Za Zhi 86:170-173 2006
GGT->GAT
c.3470G>A
p.Gly1157Asp


Gly988Cys                 OI II          Cohn et al. Proc Natl Acad Sci USA 83:6045-6047 1986
GGT->TGT
c.3496G>T
p.Gly1166Cys


Gly994Asp                 OI IIC         Mottes et al. Hum Mutat 12:71-72 1998 Online
GGT->GAT
c.3515G>A
p.Gly1172Asp


Gly1003Ser                OI II          Pruchno et al. Hum Genet 87:33-40 1991
GGC->AGC                  OI II          Pruchno et al. Hum Genet 87:33-40 1991
c.3541G>A
p.Gly1181Ser


Gly1006Val                OI II          Lamande et al. J Biol Chem 264:15809-15812 1989
GGA->GTA
c.3551G>T
p.Gly1184Val


Gly1009Ser                OI III         Cohn et al. Matrix 10:236 (abst) 1990
GGT->AGT
c.3559G>A
p.Gly1187Ser


Gly1009Val                OI II          Cohn et al. Matrix 10:236 (abst) 1990
GGT->GTT
c.3560G>T
p.Gly1187Val


Gly1017Cys C-telopeptide  OI I           Labhard et al. Mol Biol Med 5:197-207 1988
GGT->TGT                                 Cohn et al. J Biol Chem 263:14605-14607 1988
c.3583G>T                                Note: These papers refer to the same patient.
p.Gly1195Cys


(Asp1041Asn)              Dense OI       Lindahl et al. Calcif Tissue Int 80:Suppl 1 S101 2007
GAT->AAT
c.3655G>A
p.Asp1219Asn


(Asp1041Glu)              OI II          Pollitt et al. Hum Mutat Mutation in Brief #901 2006 Online
(GAT->GAG)
c.[3657T>G(+)862G>A]
p.[Asp1219Glu(+)Glu288Lys]


(Met1086Val C-propeptide) EDS/OI         Symoens et al. J Med Genet 41:e96 2004 Online
(ATG->GTG)
c.3790A>G
p.[Met1264Val; Lys1263fsX60]
r.[3790a>g; 3790_3814del]


(Trp1091Stop)             OI IV          Pollitt et al. Hum Mutat Mutation in Brief #901 2006 Online
(TGG->TAG)
c.3806G>A
p.Trp1269X


Asp1099His C-propeptide   OI II          Chessler et al. J Biol Chem 268:18218-18225 1993
GAC->CAC
c.3829G>C
p.Asp1277His


Cys1121Trp                OI I(?)        Pace et al. J Med Genet 38:443-449 2001
TGC->TGG
c.3897C>G
p.Cys1299Trp


Trp1134Cys C-propeptide   OI II          Lamande et al. J Biol Chem 270:8642-8649 1995
TGG->TGT
c.3936G>T
p.Trp1312Cys


His1145Tyr C-propeptide   OI I           Ries-Levavi et al. Hum Mutat Mutation in Brief #701 2004 Online
CAT->TAT
c.3967C>T
p.His1323Tyr


Trp1147Stop               OI I           Hartikka et al. Hum Mutat 24:147-154 2004
TGG->TAG
c.3974G>A
p.Trp1325X


Leu1210Arg C-propeptide   OI II          Chessler et al. J Biol Chem 268:18218-18225 1993
CTG->CGG
c.4163T>G
p.Leu1388Arg


Asp1235Asn                OI II          Pollitt et al. Hum Mutat Mutation in Brief #901 2006 Online
GAT-AAT
c.4237G>A
p.Asp1413Asn


Leu1259Gln                OI II          Rugolotto et al. Acta Paediatr 96:1855-1862 2007
CTG->CAG
c.4310T>A
g.16310T>A
p.Leu1437Gln

Asp1263Tyr C-propeptide   OI II-like     Pace et al. J Med Genet 39:23-29 2002
GAT->TAT
c.4321G>T
p.Asp1441Tyr


Leu1286Pro C-propeptide   OI III         Oliver et al. Hum Mutat 7:318-326 1996
CTG->CCG
c.4391T>C
p.Leu1464Pro
with variant
Thr1058Pro C-propeptide
ACC->CCC in a2(I)
c.3443A>C
p.Thr1148Pro


Table 2 RNA splicing mutations in COL1A1.

Return to the start of COL1A1.


Mutation                  Phenotype      Reference(s)
Splicing variant          OI I           Hartikka et al. Hum Mutat 24:147-154 2004
exon 1
c.103+1G>A
r.spl?

Splicing variant          OI I           Hartikka et al. Hum Mutat 24:147-154 2004
exon 2
c.104-3C>G
r.spl?

Splicing variant          OI I           Ries-Levavi et al. Hum Mutat Mutation in Brief #701 2004 Online
exon 3
c.333_333+1GG>TC
r.spl?

Splicing variant          OI I           Hartikka et al. Hum Mutat 24:147-154 2004
exon 3
c.333+2T>C
r.spl?

Splicing variant          OI I           Hartikka et al. Hum Mutat 24:147-154 2004
exon 4
c.334-2A>G
r.spl?

Splicing variant          OI I           Pollitt et al. Hum Mutat Mutation in Brief #901 2006 Online
exon 5
c.471+1G>A
r.spl?

Splicing variant          OI I           Körkkö et al. Am J Hum Genet 62:98-110 1998
exon 6
c.472-2A>G
r.spl?

Splicing variant          EDS VIIA       Byers et al. Am J Med Genet 72:94-105 1997
exon 6                    EDS VIIA       Nicholls et al. J Med Genet 37:33e 2000 Online
c.472-1G>A
r.spl?

Splicing variant          EDS VIIA       Weil et al. EMBO J 8:1705-1710 1989
exon 6                    EDS VIIA       D'Alessio et al. Am J Hum Genet 49:400-406 1991
c.543G>A
r.spl?

Splicing variant          OI III/IV/EDS  Cabral et al. J Biol Chem 280:19259-19269 2005
exon 7
c.588+4A>T
r.spl?

Splicing variant          OI I           Pollitt et al. Hum Mutat Mutation in Brief #901 2006 Online
exon 8
c.589-2A>G
r.spl?

Splicing variant          OI IV          Schwarze et al. Am J Hum Genet 65:336-344 1999
exon 8
c.642+1G>C
results in several
splice products

Splicing variant          OI III/IV      Bateman et al. Biochem J 302: 729-735 1994
exon 8
c.642+5G>A
r.spl?

Splicing variant          OI I           Lee et al. Human Mutat Mutation in Brief #894 2006 Online
exon 8
c.642+5G>A
r.spl?

Splicing variant          OI I           Lee et al. Human Mutat Mutation in Brief #894 2006 Online
exon 10
c.697-2delA
r.spl?

Splicing variant          OI I           Hartikka et al. Hum Mutat 24:147-154 2004
exon 10
c.697-2A>G
r.spl?

Splicing variant          OI IV          Pepin et al. Prenat Diagn 17:559-570 1997
exon 10
c.697-1G>T
r.spl?

Splicing variant          OI III         Ward et al. Hum Mutat Mutation in Brief #414 2001 Online
exon 11
c.804+3_804+6delGAGT
r.spl?

Splicing variant          OI I           Körkkö et al. Am J Hum Genet 62:98-110 1998
exon 12
c.858+1G>A
r.spl?

Splicing variant          OI III         Venturi et al. Clin Genet 70:131-139 2006
exon 13
c.859-2A>C
g.5374A>C
r.spl

Splicing variant          OI I           Schafer et al. Hum Genet 114:404 2004
exon 14
c.904-10T>A
new acceptor in
intron 13 and
frameshift

Splicing variant          OI II          Bonadio et al. J Biol Chem 265:2262-2268 1990
exon 14
c.957+5G>A
r.spl?

Splicing variant          OI I           Ward et al. Hum Mutat Mutation in Brief #414 2001 Online
exon 16
c.1004-44_1004-33delCTGCCATCTCTT
r.spl?

Splicing variant          OI I           Tamborlane et al. J Pediatr Orthop 24:620-622 2004
exon 17
c.1057-2A>C
r.spl?

Splicing variant          OI I           Willing et al. Am J Med Genet 45:223-227 1993
exon 17
c.1057-2A>G
r.spl?

Splicing variant          OI I           Venturi et al. Clin Genet 70:131-139 2006
exon 17
c.1155+1G>A
g.6338G>A
r.spl?

Splicing variant          OI I           Pepin et al. Prenat Diagn 17:559-570 1997
exon 17                   OI III         Hartikka et al. Hum Mutat 24:147-154 2004
c.1155+1G>C               OI I           Lee et al. Human Mutat Mutation in Brief #894 2006 Online
r.spl?

Splicing variant          OI I           Hartikka et al. Hum Mutat 24:147-154 2004
exon 17
c.1155+3_1155+6delAAGT
r.spl?

Splicing variant          OI I           Willing et al. Am J Hum Genet 55:638-647 1994
exon 18                   OI I           Willing et al. Am J Hum Genet 55:638-647 1994
c.1200+1G>A
Creates new splice site
at c.1193 causing
frameshift and termination
32 aa downstream

Splicing variant          OI I           Körkkö et al. Am J Hum Genet 62:98-110 1998
exon 19
c.1201-1G>A
r.spl?

Splicing variant          OI I           Benušienė and Kučinskas J Appl Genet 44:95-102 2003
exon 19
c.1299+1G>A
r.spl?

Splicing variant          OI IV          Cabral and Marini Am J Hum Genet 74:752-760 2004
exon 19
c.1299+1G>C
r.spl?

Splicing variant          OI I           Ward et al. Hum Mutat Mutation in Brief #414 2001 Online
exon 20
c.1353+2T>C
r.spl?

Splicing variant          OI I           Körkkö et al. Am J Hum Genet 62:98-110 1998
exon 21
c.1354-12G>A
r.spl?

Splicing variant          OI IA          Benušienė and Kučinskas J Appl Genet 44:95-102 2003
exon 21
c.1354-2A>G
r.spl?

Splicing variant          OI I           Venturi et al. Clin Genet 70:131-139 2006
exon 21
c.1461+1G>C
AF017178.2:g.7184G>C
r.spl?

Splicing variant          OI II          Byers et al. V Int Conf on OI 1993
exon 21
not defined

Splicing variant          OI I           Körkkö et al. Am J Hum Genet 62:98-110 1998
exon 22
c.1515+3A>G
r.spl?

Splicing variant          OI IB          Benušienė and Kučinskas J Appl Genet 44:95-102 2003
exon 23
c.1516-1G>T
r.spl?

Splicing variant          OI II          Lee et al. Human Mutat Mutation in Brief #894 2006 Online
exon 25
c.1669-2A>G
r.spl?

Splicing variant          OI I           Körkkö et al. Am J Hum Genet 62:98-110 1998
exon 26
c.1768-1G>A
r.spl?

Splicing variant          OI I           Stover et al. J Clin Invest 92:1994-2002 1993
exon 26                   OI I           Körkkö et al. Hum Mutat 9:148-156 1997
c.1821+1G>A               OI I           Johnson et al. J Cell Biol 150:417-431 2000
r.spl?                    OI ?           Qin et al. Yi Chuan Xue Bao 32:248-252 2005

Splicing variant          OI I           Körkkö et al. Hum Mutat 9:148-156 1997
exon 26
c.1821+1G>T
r.spl?

Splicing variant          OI II          Byers. Trends Genet 6:293-300 1990
exon 27                   OI II          Pepin et al. Prenat Diagn 17:559-570 1997
c.1822-2A>C
r.spl?

Splicing variant          OI I           Lee et al. Human Mutat Mutation in Brief #894 2006 Online
exon 28
c.1876-2delA
r.spl

Splicing variant          OI IV          Pollitt et al. Hum Mutat Mutation in Brief #901 2006 Online
exon 28
c.1876-2A>G
r.spl?

Splicing variant          OI IV          Ward et al. Hum Mutat Mutation in Brief #414 2001 Online
exon 28
c.1876-1G>C
r.spl?

Splicing variant          OI I           Venturi et al. Clin Genet 70:131-139 2006
exon 30
c.1984-5C>A
g.9877C>A
r.spl?

Splicing variant          OI III         Wallis et al. Am J Hum Genet 45S:A228 1989
exon 30
not defined

Splicing variant          OI I           Hartikka et al. Hum Mutat 24:147-154 2004
exon 30
c.2028+2T>G
r.spl?

Splicing variant          OI I           Hartikka et al. Hum Mutat 24:147-154 2004
exon 31
c.2127+2T>G
r.spl?

Splicing variant          OI III         Hartikka et al. Hum Mutat 24:147-154 2004
exon 33_34
nonsense-mediated
exon skipping?
c.2268_2269delTC
r.spl?

Splicing variant          OI I           Lee et al. Human Mutat Mutation in Brief #894 2006 Online
exon 37
c.2452-2A>G
r.spl?

Splicing variant          OI I           Hartikka et al. Hum Mutat 24:147-154 2004
exon 37
c.2452-1G>A
r.spl?

Splicing variant          OI I           Venturi et al. Clin Genet 70:131-139 2006
exon 39
c.2667+1G>A
g.12229G>A
r.spl?

Splicing variant          OI I           Hartikka et al. Hum Mutat 24:147-154 2004
exon 40
c.2829+3A>C
r.spl?

Splicing variant          OI III         Cabral et al. J Biol Chem 277:4215-4222 2002
exon 41
c.2937+4A>C
r.spl?

Splicing variant          OI II          Byers. Trends Genet 6:293-300 1990
exon 43
not defined

Splicing variant          OI I           Hartikka et al. Hum Mutat 24:147-154 2004
exon 43
c.3046-2A>G
r.spl?

Splicing variant          OI II          Byers. Trends Genet 6:293-300 1990
exon 44
c.3100-3C>?
r.spl?

Splicing variant          OI I           Hartikka et al. Hum Mutat 24:147-154 2004
exon 44
c.3207+5G>T
r.spl?

Splicing variant          OI III         Hartikka et al. Hum Mutat 24:147-154 2004
exon 45
c.3208-2A>C
r.spl?

Splicing variant          OI II          Wallis et al. IV Int Conf on OI 55 1990
exon 47
c.3423+1G>A
r.spl?

Splicing variant          OI III/IV      Pepin et al. Prenat Diagn 17:559-570 1997
exon 48
c.3424-3C>G
r.spl?

Splicing variant          OI I           Willing et al. Am J Hum Genet 55:638-647 1994
exon 48
c.3531+1G>A
retention of intron 48

Splicing variant          OI I           Lee et al. Human Mutat Mutation in Brief #894 2006 Online
exon 49
c.3532-4_3532-2delGCA
r.spl?


Table 3. Deletions, insertions duplications and frameshifts in COL1A1.

Return to the start of COL1A1.


Mutation                   Phenotype      Reference(s)
Frameshift                 OI I           Hartikka et al. Hum Mutat 24:147-154 2004
c.246delC
p.Gly83AlafsX182

Frameshift                 OI I           Hartikka et al. Hum Mutat 24:147-154 2004
c.299_300insC              OI I           Hartikka et al. Hum Mutat 24:147-154 2004
p.Glu100AspfsX69

Frameshift                 OI I           Pollitt et al. Hum Mutat Mutation in Brief #901 2006 Online
c.386dupC
p.Gly130TrpfsX39

Frameshift                 OI IV          Pollitt et al. Hum Mutat Mutation in Brief #901 2006 Online
c.386delC
p.Pro129LeufsX136

Frameshift                 OI I           Hartikka et al. Hum Mutat 24:147-154 2004
c.389_390insA
p.Arg131ProfsX38

Frameshift                 OI I           Pollitt et al. Hum Mutat Mutation in Brief #901 2006 Online
c.432delC
p.Gly145AspfsX120

Frameshift                 OI I           Ries-Levavi et al. Hum Mutat Mutation in Brief #701 2004 Online
c.459delT
p.Gly154AlafsX111

Frameshift                 OI I           Hartikka et al. Hum Mutat 24:147-154 2004
c.532delC
p.Pro178LeufsX87

Frameshift                 OI I           Venturi et al. Clin Genet 70:131-139 2006
c.579delT
g.3743delT
p.Gly194ValfsX71

Duplication                OI II          Cohn et al. Hum Mutat 2:21-27 1993
NG_007400.1:g.10328_11056dup
p.Gly320_Ala379dup

Frameshift                 OI I           Hartikka et al. Hum Mutat 24:147-154 2004
c.614delC
p.Pro205LeufsX60

Skip of exons 5 & 6        EDS VIIA       Nicholls et al. J Med Genet 37:33e 2000
NG_007400.1:g.7335_8215del881insT
p.Gly124_Met181del

Frameshift                 OI I           Willing et al. Am J Hum Genet 59:799-809 1996
c.775delG
p.Ala259LeufsX6

Frameshift                 OI I           Körkkö et al. Am J Hum Genet 62:98-110 1998
c.808delT
p.Phe270SerfsX271

Frameshift                 OI I           Ries-Levavi et al. Hum Mutat Mutation in Brief #701 2004 Online
c.1001dupC
p.Gly335TrpfsX18

Frameshift                 OI I           Lee et al. Human Mutat Mutation in Brief #894 2006 Online
c.997_998delCCinsG
p.Pro333AlafsX208

Frameshift                 OI I           Willing et al. Am J Hum Genet 59:799-809 1996
c.1127delC
p.Pro376LeufsX165

Frameshift                 OI I           Willing et al. Am J Hum Genet 59:799-809 1996
c.1127dupC
p.Gly377TrpfsX15

Frameshift                 OI I           Willing et al. Am J Hum Genet 59:799-809 1996
c.1128delT                                Roschger et al. Calcif Tissue Int 82:263-270 2008
p.Gly377AlafsX164

Frameshift                 OI IV          Pollitt et al. Hum Mutat Mutation in Brief #901 2006 Online
c.1379dupC
p.Gly461TrpfsX14

Frameshift                 OI I           Ward et al. Hum Mutat Mutation in Brief #414 2001 Online
c.1452delT
p.Gly485AlafsX56

Frameshift                 OI I           Willing et al. Am J Hum Genet 55:638-647 1994
c.1486_1487delCC
p.Pro496TrpfsX14

Deletion                   OI II          Chu et al. J Biol Chem 260:691-694 1985
NG_007400.1:g.12149_12797del              Barsh et al. Proc Natl Acad Sci USA 82:2870-2874 1985
p.Gly506_Ala589del                        Note: These papers refer to the same patient.

Frameshift                 OI IB          Benušienė and Kučinskas J Appl Genet 44:95-102 2003
c.1667dupC
p.Gly557TrpfsX30

Frameshift                 OI IB          Benušienė and Kučinskas J Appl Genet 44:95-102 2003
c.1668delT
p.Gly557ValfsX23

Frameshift                 OI I           Körkkö et al. Am J Hum Genet 62:98-110 1998
c.1668_1669insC
p.Gly557ArgfsX30

Frameshift                 OI I           Körkkö et al. Am J Hum Genet 62:98-110 1998
c.1719_1720insAC
p.Arg574ThrfsX7

Frameshift                 OI I           Lee et al. Human Mutat Mutation in Brief #894 2006 Online
c.1814delG
p.Gly605AlafsX161

Deletion/insertion         OI IV          Lund et al. Clin Genet 50:304-309 1996
c.1845_1847delGGC
p.Glu615_Ala616delinsAsp

Frameshift                 OI I           Lee et al. Human Mutat Mutation in Brief #894 2006 Online
c.1865delC
p.Pro622fs

Frameshift                 OI I           Redford-Badwal et al. J Clin Invest 97:1035-1040 1996
c.1866delT
p.Gly623AlafsX143

Frameshift                 OI I           Reis et al. J Appl Genet 46:105-108 2005
c.1885delG
p.Gly629AlafsX137

Frameshift                 OI I           Venturi et al. Clin Genet 70:131-139 2006
c.1920dupC
g.9256dupC
p.Gly641ArgfsX14
Note: This mutation is incorrectly described for patient 11 in table 2 of Venturi et al.

Frameshift                 OI I           Pollitt et al. Hum Mutat Mutation in Brief #901 2006 Online
c.1944delT
p.Ala649LeufsX117

Frameshift                 OI I           Hartikka et al. Hum Mutat 24:147-154 2004
c.1994delG
p.Gly665GlufsX101

Frameshift                 OI I           Ward et al. Hum Mutat Mutation in Brief #414 2001 Online
c.2010delT
p.Gly671AlafsX95

Frameshift                 OI IB          Benušienė and Kučinskas J Appl Genet 44:95-102 2003
c.2046_2047insCTCTCTAG
p.Gly683LeufsX86

Frameshift                 OI I           Ward et al. Hum Mutat Mutation in Brief #414 2001 Online
c.2072delC
p.Pro691LeufsX75

Frameshift                 OI I           Körkkö et al. Am J Hum Genet 62:98-110 1998
c.2073delT
p.Gly692ValfsX74

Frameshift                 OI I           Ries-Levavi et al. Hum Mutat Mutation in Brief #701 2004 Online
c.2097delC
p.Asn700ThrfsX66

Deletion                   OI III         Wang et al. J Biol Chem 271:28617-28623 1996
NG_007400.1:g.15815_16373del559
p.Gly746_Pro817del

Retention of first         OI II          Genovese et al. J Biol Chem 264:9632-9637 1989
75 bp of intron 35
NG_007400.1:g.16390C>T
p.Pro817_Gly818ins25

Frameshift                 OI I           Kataoka et al. Pediatr Int 49:564-569 2007
c.2444delG
p.Gly815AlafsX293

Frameshift                 OI I           Hartikka et al. Hum Mutat 24:147-154 2004
c.2451delT
p.Gly818ValfsX290

Frameshift                 OI I           Willing et al. Am J Hum Genet 59:799-809 1996
c.2451dupT
p.Gly818TrpfsX3

Frameshift                 OI IV          Ward et al. Hum Mutat Mutation in Brief #414 2001 Online
c.2523delT
p.Gly842AlafsX266

Frameshift                 OI I           Hartikka et al. Hum Mutat 24:147-154 2004
c.2550dupT
p.Gly851TrpfsX5

1aa duplication            OI III/IV      Pace et al. Hum Mutat 18:319-326 2001
c.2562_2564dupTAA
p.Asn855dup

Frameshift                 OI I           Willing et al. Am J Hum Genet 59:799-809 1996
c.2510_2525del
p.Asp837AlafsX266

Frameshift                 OI I           Willing et al. Am J Hum Genet 59:799-809 1996
c.2522dupC
p.Gly842TrpfsX14

Frameshift                 OI I           Körkkö et al. Am J Hum Genet 62:98-110 1998
c.2613delT
p.Gly872ValfsX236

Frameshift                 OI I           Hartikka et al. Hum Mutat 24:147-154 2004
c.2646_2647delAG
p.Val883ArgfsX29

Frameshift at Pro717       OI IV          Ward et al. Hum Mutat Mutation in Brief #414 2001 Online
c.2684delC
p.Pro895fsX213

Deletion                   OI II          Pace et al. Hum Mutat 18:319-326 2001
c.2722_2730delGGTCCCCGT
p.Gly908_Arg910del

Frameshift                 OI I           Ward et al. Hum Mutat Mutation in Brief #414 2001 Online
c.2784delT
p.Gly929AlafsX179

Frameshift                 OI I           Pollitt et al. Hum Mutat Mutation in Brief #901 2006 Online
c.3008delC                 OI IV          Pollitt et al. Hum Mutat Mutation in Brief #901 2006 Online
p.Pro1003fsX105

Frameshift                 OI I           Hartikka et al. Hum Mutat 24:147-154 2004
c.3064delG
p.Gly1022Valfs

Frameshift                 OI I           Körkkö et al. Am J Hum Genet 62:98-110 1998
c.3079delG
p.Asp1027ThrfsX81

Deletion                   OI II          Pace et al. Hum Mutat 18:319-326 2001
c.3129_3137delACCCCCTGG
p.Pro1044_Gly1046del

Deletion                   OI II          Pace et al. Hum Mutat 18:319-326 2001
c.3132_3140delCCCTGGTGC
p.Pro1045_Ala1047del

Deletion                   OI II          Kataoka et al. Pediatr Int 49:564-569 2007
c.3145_3153delGGTGCTCCT
p.Gly1049_Pro1051del
Note: this mutation is incorectly described (as deletion GlyProAla) for patient 13 in table 2 of Kataoka et al.

Duplication                OI II          Pace et al. Hum Mutat 18:319-326 2001
c.3145_3153dupGGTGCTCCT    OI II          Cabral et al. J Biol Chem 278:10006-10012 2003
p.Gly1049_Pro1051dup

Deletion                   OI II          Hawkins et al. J Biol Chem 266:22370-22374 1991
c.3150_3158delTCCTGGTGC
p.Pro1051_Ala1053del

Deletion                   OI II          Wallis et al. J Biol Chem 267:25529-25534 1992
c.3155_3163delGTGCCCCTG
p.Gly1052_Pro1054del

Insertion                  OI II          Ward et al. Hum Mutat Mutation in Brief #414 2001 Online
c.3157_3158insCTGGTGGTC
p.Ala1053_Pro1054insGlyGlyPro

Frameshift                 OI I           Hartikka et al. Hum Mutat 24:147-154 2004
c.3162delT
p.Gly1055AlafsX53

Frameshift                 OI I           Venturi et al. Clin Genet 70:131-139 2006
c.3201delT
g.13371delT
p.Glu1068ArgfsX40

Frameshift                 OI I           Willing et al. Am J Hum Genet 55:638-647 1994
c.3285_3286dupC
p.Lys1096GlnfsX3

Frameshift                 OI IV          Lee et al. Human Mutat Mutation in Brief #894 2006 Online
c.3307_3314delGGCGACAG
p.Gly1103fsArgfsX23

Frameshift                 OI I           Körkkö et al. Hum Mutat 9:148-156 1997
c.3313delA                                Nwosu et al. Ophthalmic Genet 26:135-138 2005
p.Arg1105GlufsX3

Frameshift                 OI I           Willing et al. Am J Hum Genet 55:638-647 1994
c.3402delC
p.Ser1135LeufsX104

6 aa deletion              OI II          Kataoka et al. Pediatr Int 49:564-569 2007
c.3478_3495delGGCCCCATTGGGCCCCCT
p.1160_1166delGlyProIleGlyProPro

3aa duplication            OI III/IV      Pace et al. Hum Mutat 18:319-326 2001
c.3559_3567dupGGTCCCCCT
p.Gly1190_Pro1192dupGlyProPro

Frameshift                 OI I           Hartikka et al. Hum Mutat 24:147-154 2004
c.3560delG
p.Gly1187ValfsX52

Frameshift                 OI III         Pollitt et al. Hum Mutat Mutation in Brief #901 2006 Online
c.3580_3581delGC
p.Ala1194TrpfsX25

Frameshift                 OI I           Pollitt et al. Hum Mutat Mutation in Brief #901 2006 Online
c.3584delG
p.Gly1195ValfsX44

Frameshift                 OI I           Willing et al. Am J Hum Genet 55:638-647 1994
c.3589_3590delGA
p.Asp1197LeufsX22

3aa deletion               OI III         Ries-Levavi et al. Hum Mutat Mutation in Brief #701 2004 Online
c.3652_3660delGCTGATGAT
p.Ala1218_Asp1220delAlaAspAsp

Frameshift                 OI I           Willing et al. Am J Hum Genet 59:799-809 1996
c.3782_3794del
p.Asp1261AlafsX66

Frameshift                 OI II          Bateman et al. J Biol Chem 264:10960-10964 1989
c.3969dupT
p.Val1324CysfsX105

Leu1352Stop                OI I           Pollitt et al. Hum Mutat Mutation in Brief #901 2006 Online
c.4054delC
p.Leu1352X

2aa deletion               OI II          Chessler et al. J Biol Chem 268:18218-18225 1993
c.4008_4013del
p.Glu1337_Tyr1338del

Frameshift                 OI IB          Benušienė and Kučinskas J Appl Genet 44:95-102 2003
c.4338dupC
p.Val1447ArgfsX104

Frameshift                 OI I           Willing et al. J Clin Invest 85:282-290 1990
c.4358_4362del
p.Glu1453ArgfsX96

Deletion                   OI I           Pollitt et al. Hum Mutat Mutation in Brief #901 2006 Online
Chr17q21.33_q23.1del


Table 4. Polymorphisms in COL1A1 cDNA.

Return to the start of COL1A1.


Polymorphism                      Reference
Arg59Arg   N-propeptide           Mackay et al. Hum Mol Genet 2:1155-1160 1993
CGG>CGT
c.177G>T

Pro338Pro                         Marini et al. J Biol Chem 268:2667-2673 1993
CCC>CCT
c.1548C>T

Arg386His                         Pruchno et al. Hum Genet 87:33-40 1991
CGC>CAC
c.1691G>A
p.Arg564His

Ala410Ala                         Mackay et al. Hum Mol Genet 2:1155-1160 1993
GCT>GCC
c.1764T>C

Pro423Pro                         Mirandola et al. Mol Cell Probes 14:329-332 2000
CCC>CCT
c.1803C>T

Gly517Gly                         Nicholls et al. J Med Genet 28:757-764 1991
GGA>GGT
c.2085A>T
p.=

Pro645Ala                         Mackay et al. Hum Mol Genet 2:1155-1160 1993
CCT>GCT
c.2467C>T
p.Pro823Ala

Pro800Pro                         Oliver et al. Hum Mutat 7:318-326 1996
CCC>CTT
c.2934
p.=

Ala897Thr                         Sokolov et al. Nucl Acids Res 19:4302 1991
GCC>ACC
c.3223G>A
p.Ala1075Thr

Pro899Pro                         Lamande et al. J Biol Chem 264:15809-15812 1989
CCT>CCC
c.3231T>C

Pro902Pro                         Lamande et al. J Biol Chem 264:15809-15812 1989
CCT>CCC
c.3240T>C

Val903Val                         Bateman et al. Am J Med Genet 45:233-240 1993
GTC>GTT
c.3243C>T

Asp975Asp                         Zhuang et al. Hum Mutat 7:89-99 1996
GAT<>GAC
c.3459T>C

Ser1215Ser                        Zhuang et al. Hum Mutat 7:89-99 1996
TCC>TCT
c.4179C>T

Ser1256Thr   C-propeptide         Mäkelä et al. Nucl Acids Res 16:349 1988
TCC>ACC
c.4300T>A
p.Ser1434Thr

3' untranslated                   Zhuang et al. Hum Mutat 7:89-99 1996
TCA>CCA   nt4602
c.4483T>C

3' untranslated                   Nuytinck et al. Matrix Biol 16:349-352 1997
4 nt insertion (AGCA)
following nt 5194
c.5075-5076insAGCA

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This collection of data is copyright © 1996 - 2008 Raymond Dalgleish, University of Leicester. Valid HTML 4.01


Last updated: 1 August, 2008
Raymond Dalgleish
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