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

Research output: Contribution to journal › Article › peer-review

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
Externally published	Yes

ASJC Scopus subject areas

Biochemistry, Genetics and Molecular Biology(all)

Access to Document

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

Fingerprint Dive into the research topics of 'Adenosine deaminase deficiency and chondro-osseous dysplasia.'. Together they form a unique fingerprint.

Cite this

@article{7e33c8a884c543cead685c55f6d6f18c,

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",

pages = "81--83",

journal = "Advances in Experimental Medicine and Biology",

issn = "0065-2598",

publisher = "Springer New York",

}

TY - JOUR

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.

UR - http://www.scopus.com/inward/record.url?scp=0022530045&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0022530045&partnerID=8YFLogxK

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

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

M3 - Article

C2 - 3487928

AN - SCOPUS:0022530045

VL - 195 Pt A

SP - 81

EP - 83

JO - Advances in Experimental Medicine and Biology

JF - Advances in Experimental Medicine and Biology

SN - 0065-2598

ER -