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Titolo:
ADENOSINE-DEAMINASE-DEFICIENT MICE DIE PERINATALLY AND EXHIBIT LIVER-CELL DEGENERATION, ATELECTASIS AND SMALL-INTESTINAL CELL-DEATH
Autore:
MIGCHIELSEN AAJ; BREUER ML; VANROON MA; RIELE HT; ZURCHER C; OSSENDORP F; TOUTAIN S; HERSHFIELD MS; BERNS A; VALERIO D;
Indirizzi:
LEIDEN UNIV,SYLVIUS LABS,MOLEC CARCINOGENESIS LAB,GENE THERAPY WORKING GRP,WASSENAARSEWEG 72 2300 RA LEIDEN NETHERLANDS LEIDEN UNIV,SYLVIUS LABS,MOLEC CARCINOGENESIS LAB,GENE THERAPY WORKING GRP 2300 RA LEIDEN NETHERLANDS NETHERLANDS CANC INST,DIV MOLEC GENET 1066 CX AMSTERDAM NETHERLANDS TNO,BIOMED PRIMATE RES CTR 2280 HV RIJSWIJK NETHERLANDS ACAD HOSP LEIDEN,IMMUNOHEMATOL & BLOODBANK LEIDEN NETHERLANDS DUKE UNIV,MED CTR,DEPT RHEUMATOL & IMMUNOL DURHAM NC 27710 INTROGENE BV 2280 GG RYSWIJK NETHERLANDS
Titolo Testata:
Nature genetics
fascicolo: 3, volume: 10, anno: 1995,
pagine: 279 - 287
SICI:
1061-4036(1995)10:3<279:AMDPAE>2.0.ZU;2-I
Fonte:
ISI
Lingua:
ENG
Soggetto:
S-ADENOSYLHOMOCYSTEINE HYDROLASE; SEVERE COMBINED IMMUNODEFICIENCY; T-LYMPHOMA CELLS; DEOXYADENOSINE METABOLISM; HOMOCYSTEINE HYDROLASE; PATHOLOGIC FINDINGS; TISSUE ALTERATIONS; MOUSE; EXPRESSION; 2'-DEOXYCOFORMYCIN;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Science Citation Index Expanded
Citazioni:
61
Recensione:
Indirizzi per estratti:
Citazione:
A.A.J. Migchielsen et al., "ADENOSINE-DEAMINASE-DEFICIENT MICE DIE PERINATALLY AND EXHIBIT LIVER-CELL DEGENERATION, ATELECTASIS AND SMALL-INTESTINAL CELL-DEATH", Nature genetics, 10(3), 1995, pp. 279-287

Abstract

We report the generation and characterization of mice lacking adenosine deaminase (ADA). in humans, absence of ADA causes severe combined immunodeficiency. In contrast, ADA-deficient mice die perinatally with marked liver-cell degeneration, but lack abnormalities in the thymus. The ADA substrates, adenosine and deoxyadenosine, are increased in ADA-deficient mice. Adenine deoxyribonucleotides are only modestly elevated, whereas S-adenosylhomocysteine hydrolase activity is reduced more than 85%. Consequently, the ratio of S-adenosylhomocysteine (AdoMet) to S-adenosyl homocysteine (AdoHcy) is reduced threefold in liver. We conclude that ADA plays a more critical role in murine than human fetaldevelopment. me murine liver pathology may be due to AdoHcy-mediated inhibition of AdoMet-dependent transmethylation reactions.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 15/07/20 alle ore 15:04:58