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

M. S. Hershfield, N. M. Kredich, D. R. Ownby, H. Ownby, R. Buckley

Research output: Contribution to journalArticle

112 Citations (Scopus)

Abstract

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 the authors 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 3 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.

Original languageEnglish (US)
Pages (from-to)807-811
Number of pages5
JournalUnknown Journal
Volume63
Issue number4
DOIs
StatePublished - Jan 1 1979

Fingerprint

Adenosylhomocysteinase
Adenosine Deaminase
suicide
Erythrocytes
Suicide
cause
communication
Nucleosides
Catalytic Domain
2'-deoxyadenosine
Enzymes

ASJC Scopus subject areas

  • Medicine(all)

Cite this

In vivo inactivation of erythrocyte S-adenosylhomocysteine hydrolase by 2'-deoxyadenosine in adenosine deaminase-deficient patients. / Hershfield, M. S.; Kredich, N. M.; Ownby, D. R.; Ownby, H.; Buckley, R.

In: Unknown Journal, Vol. 63, No. 4, 01.01.1979, p. 807-811.

Research output: Contribution to journalArticle

@article{28a71f160ee3470890d5305f051cf2c5,
title = "In vivo inactivation of erythrocyte S-adenosylhomocysteine hydrolase by 2'-deoxyadenosine in adenosine deaminase-deficient patients",
abstract = "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 the authors 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 3 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.",
author = "Hershfield, {M. S.} and Kredich, {N. M.} and Ownby, {D. R.} and H. Ownby and R. Buckley",
year = "1979",
month = "1",
day = "1",
doi = "10.1172/JCI109367",
language = "English (US)",
volume = "63",
pages = "807--811",
journal = "Handbook of Behavioral Neuroscience",
issn = "0003-1348",
publisher = "JAI Press",
number = "4",

}

TY - JOUR

T1 - In vivo inactivation of erythrocyte S-adenosylhomocysteine hydrolase by 2'-deoxyadenosine in adenosine deaminase-deficient patients

AU - Hershfield, M. S.

AU - Kredich, N. M.

AU - Ownby, D. R.

AU - Ownby, H.

AU - Buckley, R.

PY - 1979/1/1

Y1 - 1979/1/1

N2 - 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 the authors 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 3 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.

AB - 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 the authors 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 3 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.

UR - http://www.scopus.com/inward/record.url?scp=0018765904&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0018765904&partnerID=8YFLogxK

U2 - 10.1172/JCI109367

DO - 10.1172/JCI109367

M3 - Article

C2 - 312296

AN - SCOPUS:0018765904

VL - 63

SP - 807

EP - 811

JO - Handbook of Behavioral Neuroscience

JF - Handbook of Behavioral Neuroscience

SN - 0003-1348

IS - 4

ER -