An in vitro model of ADA deficiency is selectively toxic to cartilage from immature rabbits with a greater effect on growth plate than articular cartilage. The selective toxicity observed appears to be the consequence of ATP depletion. These results support the hypothesis that the chondro-osseous dysplasia observed in patients with ADA deficiency is caused by the disordered metabolism that results from the enzyme deficiency.
|Original language||English (US)|
|Number of pages||3|
|Journal||Advances in experimental medicine and biology|
|Volume||195 Pt A|
|State||Published - Jan 1 1986|
ASJC Scopus subject areas
- Biochemistry, Genetics and Molecular Biology(all)