Table 2

Structural gene mutations in cartilage that result in abnormal cartilage matrix

OMIM#
Gene name
Gene symbol
Diseases and disorders

12140
Collagen, type II α1
COL2A1
Achondrogenesis, type II



Achondrogenesis-hypochondrogenesis, type II



Epiphyseal dysplasia, multiple, with myopia and conductive deafness



Hypochondrogenesis



Kniest dysplasia



Osteoarthritis with mild dysplasia



Spondyloepiphyseal dysplasia, congenital type



Spondyloepiphyseal dysplasia, Namaqualand type



Spondyloepiphyseal dysplasia, Strudwick type



Spondyloepiphyseal dysplasia, various types



Spondyloepiphyseal dysplasia with precocious OA



Spondyloperipheral dysplasia



Stickler syndrome, type I



Wagner syndrome
120180
Collagen, type III α1
COL3A1
Arterial and aortic aneurysm



Ehlers–Danlos syndrome, types III and IV
120220
Collagen, type VI α1
COL6A1
Bethlem myopathy
120240
Collagen, type VI α2
COL6A2
Bethlem myopathy



Ullrich scleroatonic muscular dystrophy
120250
Collagen, type VI α3
COL6A3
Bethlem myopathy
120210
Collagen, type IX α1
COL9A1
Epiphyseal dysplasia, multiple, type 1



Intervertebral disk disease
120260
Collagen, type IX α2
COL9A2
Epiphyseal dysplasia, multiple, type 2



Intervertebral disk disease
120270
Collagen, type IX α3
COL9A3
Epiphyseal dysplasia, multiple, type 3



Epiphyseal dysplasia, multiple, with myopathy
120110
Collagen, type X α1
COL10A1
Metaphyseal chondrodysplasia, Schmid type



Spondylometaphyseal dysplasia, Japanese type
120260
Collagen, type XI α1
COL11A1
Stickler syndrome, type II



Marshall syndrome
120290
Collagen, type XI α2
COL11A2
Sensorineural deafness, autosomal dominant nonsyndromic



Otospondylomegaepiphyseal dysplasia



Stickler syndrome, type III



Weissenbacher–Zweymuller syndrome
600310
Cartilage oligomeric matrix protein
COMP
Pseudoachondroplasia



Epiphyseal dysplasia, multiple, Fairbanks type



Epiphyseal dysplasia, multiple, type 1
602109
Matrilin-3
MATN3
Multiple epiphyseal dysplasia, MATN3-related



Epiphyseal dysplasia, multiple, type 5
134797
Fibrillin
FBN1
Marfan syndrome, various type



Ectopia lentis, familial



Marfanoid skeletal syndrome



MASS syndrome



Shprintzen–Goldberg syndrome
154870
Matrix γ-carboxyglutamic acid protein
MGP
Keutel syndrome
142461
Perlecan
PLC
Schwartz–Jampel syndrome, type 1



Dyssegmental dysplasia, Silverman–Handmaker type



Chondrodystrophic myotonia
222600
Diastrophic dysplasia sulfate transporter
DTDST
Achondrogenesis IB



Atelostogenesis type II



Diastrophic dysplasia



Epiphyseal dysplasia, multiple type 4



Diastrophic dysplasia, broad-bone–platyspondylic variant
604283
Proteoglycan 4
PRG4
Camptodactyly–arthropathy–coxa vara–pericarditis syndrome
605145
ANK
ANKH
Craniometaphyseal dysplasia, autosomal dominant



Chondrocalcinosis 2

MASS, mitral valve, aorta, skeleton, skin; OA, osteoarthritis; OMIM, Online Mendelian Inheritance in Man™.

Reginato and Olsen Arthritis Res 2002 4:337   doi:10.1186/ar595