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Titolo:
HOMOZYGOUS C1Q DEFICIENCY CAUSES GLOMERULONEPHRITIS ASSOCIATED WITH MULTIPLE APOPTOTIC BODIES
Autore:
BOTTO M; DELLAGNOLA C; BYGRAVE AE; THOMPSON EM; COOK HT; PETRY F; LOOS M; PANDOLFI PP; WALPORT MJ;
Indirizzi:
IMPERIAL COLL SCI TECHNOL & MED,SCH MED,RHEUMATOL SECT,HAMMERSMITH CAMPUS LONDON W12 0NN ENGLAND IMPERIAL COLL SCI TECHNOL & MED,SCH MED,RHEUMATOL SECT LONDON W12 0NNENGLAND IMPERIAL COLL SCI TECHNOL & MED,SCH MED,DEPT HISTOPATHOL LONDON W12 0NN ENGLAND DEPT HISTOPATHOL LONDON W2 1NY ENGLAND INST MED MICROBIOL & HYG D-55101 MAINZ GERMANY MEM SLOAN KETTERING CANC CTR,DEPT HUMAN GENET NEW YORK NY 10021
Titolo Testata:
Nature genetics
fascicolo: 1, volume: 19, anno: 1998,
pagine: 56 - 59
SICI:
1061-4036(1998)19:1<56:HCDCGA>2.0.ZU;2-S
Fonte:
ISI
Lingua:
ENG
Soggetto:
SYSTEMIC LUPUS-ERYTHEMATOSUS; MOLECULAR-BASIS; KERATINOCYTES; EXPRESSION; NEPHRITIS; ANTIGENS; BINDING; FAMILY; GENES;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Science Citation Index Expanded
Citazioni:
20
Recensione:
Indirizzi per estratti:
Citazione:
M. Botto et al., "HOMOZYGOUS C1Q DEFICIENCY CAUSES GLOMERULONEPHRITIS ASSOCIATED WITH MULTIPLE APOPTOTIC BODIES", Nature genetics, 19(1), 1998, pp. 56-59

Abstract

The complement system plays a paradoxical role in the development andexpression of autoimmunity in humans. The activation of complement insystemic lupus erythematosus (SLE) contributes to tissue injury. In contrast, inherited deficiency of classical pathway components, particularly Clq (ref, 1), is powerfully associated with the development of SLE, This leads to the hypothesis that a physiological action of the early part of the classical pathway protects against the development of SLE (ref, 2) and implies that Clq may play a key role in this respect. Clq-deficient (C1qa(-/-)) mice were generated by gene targeting and monitored for eight months. C1qa(-/-) mice had increased mortality and higher titres of autoantibodies, compared with strain-matched controls. Of the C1qa(-/-) mice, 25% had glomerulonephritis with immune deposits and multiple apoptotic cell bodies. Among mice without glomerulonephritis, there were significantly greater numbers of glomerular apoptotic bodies in Clq-deficient mice compared with controls. The phenotype associated with Clq deficiency was modified by background genes. Thesefindings are compatible with the hypothesis that Clq deficiency causes autoimmunity by impairment of the clearance of apoptotic cells.

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Documento generato il 01/12/20 alle ore 16:46:20