Adenosine deaminase deficiency and chondro-osseous dysplasia.

R. L. Wortmann; J. A. Veum; H. S. Cheung

doi:10.1007/978-1-4684-5104-7_13

Adenosine deaminase deficiency and chondro-osseous dysplasia.

R. L. Wortmann, J. A. Veum, H. S. Cheung

Biomedical Engineering

Research output: Contribution to journal › Article

1 Scopus citations

Abstract

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)
Pages (from-to)	81-83
Number of pages	3
Journal	Advances in experimental medicine and biology
Volume	195 Pt A
DOIs	https://doi.org/10.1007/978-1-4684-5104-7_13
State	Published - 1986

ASJC Scopus subject areas

Biochemistry, Genetics and Molecular Biology(all)

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Wortmann, R. L., Veum, J. A., & Cheung, H. S. (1986). Adenosine deaminase deficiency and chondro-osseous dysplasia. Advances in experimental medicine and biology, 195 Pt A, 81-83. https://doi.org/10.1007/978-1-4684-5104-7_13

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abstract = "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.",

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