Table 2

Genetic disorders and the mutant cartilage matrix proteoglycans and proteins with which they are associated

Mutant matrix molecule
Associated disorder1

Aggrecan (impaired core
Cartilage matrix deficiency (in mice)
protein synthesis)
Nanomelia (in chickens)

Brachymorphism (in mice)
Aggrecan (impaired
Diastrophic dysplasia
glycosaminoglycan sulfation)
Achondrogenesis 1B

Atelosteogenesis type II
Perlecan
Chondrodystrophic myotonia

Dyssegmental dysplasia
SZP
Camptodactyly-arthropathy-

coxa vara-pericarditis syndrome
COMP
Pseudoachondroplasia

Multiple epiphyseal dysplasia

1Human disorder unless indicated otherwise. COMP = cartilage oligomeric protein; SZP = superficial zone protein.

Roughley Arthritis Res 2001 3:342   doi:10.1186/ar326