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In vivo inactivation of erythrocyte S-adenosylhomocysteine hydrolase by 2'-deoxyadenosine in adenosine deaminase-deficient patients.

Publication ,  Journal Article
Hershfield, MS; Kredich, NM; Ownby, DR; Ownby, H; Buckley, R
Published in: J Clin Invest
April 1979

The cytotoxic nucleoside 2'-deoxyadenosine is excreted in excessive amounts by individuals with genetic deficiency of adenosine deaminase, and may be in part responsible for the severe combined immune dysfunction from which they suffer. Earlier studies from this laboratory showed that 2'-deoxyadenosine causes the irreversible inactivation of the enzyme S-adenosylhomocysteine hydrolase by an active site-directed, "suicide-like" process. In this communication we have demonstrated similar inactivation of S-adenosylhomocysteine hydrolase in hemolysate and in intact erythrocytes, as well as a striking deficiency of S-adenosylhomocysteine hydrolase activity in the erythrocytes of three adenosine deaminase-deficient patients. In vivo suicide-like inactivation of S-adenosylhomocysteine hydrolase by 2'-deoxyadenosine may contribute to the cytotoxicity of 2'-deoxyadenosine and to the immune dysfunction in adenosine deaminase deficiency.

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Published In

J Clin Invest

DOI

ISSN

0021-9738

Publication Date

April 1979

Volume

63

Issue

4

Start / End Page

807 / 811

Location

United States

Related Subject Headings

  • S-Adenosylhomocysteine
  • Nucleoside Deaminases
  • Male
  • Kinetics
  • Immunology
  • Hydrolases
  • Humans
  • Female
  • Erythrocytes
  • Deoxyadenosines
 

Citation

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MLA
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Hershfield, M. S., Kredich, N. M., Ownby, D. R., Ownby, H., & Buckley, R. (1979). In vivo inactivation of erythrocyte S-adenosylhomocysteine hydrolase by 2'-deoxyadenosine in adenosine deaminase-deficient patients. J Clin Invest, 63(4), 807–811. https://doi.org/10.1172/JCI109367
Hershfield, M. S., N. M. Kredich, D. R. Ownby, H. Ownby, and R. Buckley. “In vivo inactivation of erythrocyte S-adenosylhomocysteine hydrolase by 2'-deoxyadenosine in adenosine deaminase-deficient patients.J Clin Invest 63, no. 4 (April 1979): 807–11. https://doi.org/10.1172/JCI109367.
Hershfield MS, Kredich NM, Ownby DR, Ownby H, Buckley R. In vivo inactivation of erythrocyte S-adenosylhomocysteine hydrolase by 2'-deoxyadenosine in adenosine deaminase-deficient patients. J Clin Invest. 1979 Apr;63(4):807–11.
Hershfield, M. S., et al. “In vivo inactivation of erythrocyte S-adenosylhomocysteine hydrolase by 2'-deoxyadenosine in adenosine deaminase-deficient patients.J Clin Invest, vol. 63, no. 4, Apr. 1979, pp. 807–11. Pubmed, doi:10.1172/JCI109367.
Hershfield MS, Kredich NM, Ownby DR, Ownby H, Buckley R. In vivo inactivation of erythrocyte S-adenosylhomocysteine hydrolase by 2'-deoxyadenosine in adenosine deaminase-deficient patients. J Clin Invest. 1979 Apr;63(4):807–811.

Published In

J Clin Invest

DOI

ISSN

0021-9738

Publication Date

April 1979

Volume

63

Issue

4

Start / End Page

807 / 811

Location

United States

Related Subject Headings

  • S-Adenosylhomocysteine
  • Nucleoside Deaminases
  • Male
  • Kinetics
  • Immunology
  • Hydrolases
  • Humans
  • Female
  • Erythrocytes
  • Deoxyadenosines