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

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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.

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
Externally published	Yes

ASJC Scopus subject areas

Biochemistry, Genetics and Molecular Biology(all)

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

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title = "Adenosine deaminase deficiency and chondro-osseous dysplasia.",

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.",

author = "Wortmann, {R. L.} and Veum, {J. A.} and Cheung, {H. S.}",

year = "1986",

doi = "10.1007/978-1-4684-5104-7_13",

language = "English (US)",

volume = "195 Pt A",

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journal = "Advances in Experimental Medicine and Biology",

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T1 - Adenosine deaminase deficiency and chondro-osseous dysplasia.

AU - Wortmann, R. L.

AU - Veum, J. A.

AU - Cheung, H. S.

PY - 1986

Y1 - 1986

N2 - 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.

AB - 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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U2 - 10.1007/978-1-4684-5104-7_13

DO - 10.1007/978-1-4684-5104-7_13

M3 - Article

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AN - SCOPUS:0022530045

VL - 195 Pt A

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JO - Advances in Experimental Medicine and Biology

JF - Advances in Experimental Medicine and Biology

SN - 0065-2598

ER -

Adenosine deaminase deficiency and chondro-osseous dysplasia.

Abstract

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