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Titolo:
Stable and functional lymphoid reconstitution of common cytokine receptor gamma chain deficient mice by retroviral-mediated gene transfer
Autore:
Soudais, C; Shiho, T; Sharara, LI; Guy-Grand, D; Taniguchi, T; Fischer, A; Di Santo, JP;
Indirizzi:
Inst Pasteur, Unite Cytokines & Dev Lymphoide, F-75724 Paris, France Inst Pasteur Paris France F-75724 & Dev Lymphoide, F-75724 Paris, France Hop Necker Enfants Malad, INSERM, U429, Paris, France Hop Necker Enfants Malad Paris France alad, INSERM, U429, Paris, France Univ Tokyo, Dept Immunol, Tokyo, Japan Univ Tokyo Tokyo JapanUniv Tokyo, Dept Immunol, Tokyo, Japan
Titolo Testata:
BLOOD
fascicolo: 10, volume: 95, anno: 2000,
pagine: 3071 - 3077
SICI:
0006-4971(20000515)95:10<3071:SAFLRO>2.0.ZU;2-3
Fonte:
ISI
Lingua:
ENG
Soggetto:
SEVERE COMBINED IMMUNODEFICIENCY; HEMATOPOIETIC STEM-CELLS; IFN-GAMMA; T-CELLS; EXPRESSION; PROGENITORS; INFECTION; DISEASE; TRANSPLANTATION; DIFFERENTIATION;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Clinical Medicine
Life Sciences
Citazioni:
40
Recensione:
Indirizzi per estratti:
Indirizzo: Di Santo, JP Inst Pasteur, Unite Cytokines & Dev Lymphoide, 25 Rue Dr Roux, F-75724 Paris, France Inst Pasteur 25 Rue Dr Roux Paris France F-75724 ris, France
Citazione:
C. Soudais et al., "Stable and functional lymphoid reconstitution of common cytokine receptor gamma chain deficient mice by retroviral-mediated gene transfer", BLOOD, 95(10), 2000, pp. 3071-3077

Abstract

Mutations in the gene encoding the common cytokine receptor gamma chain (gamma(c)) are responsible for human X-linked severe combined immunodeficiency disease (SCIDX1), We have used a gamma(c)-deficient mouse model to test the feasibility and potential toxicity of gamma(c) gene transfer as a therapy for SCIDX1, A retrovirus harboring the murine ye chain was introduced into gamma(c)-deficient bone marrow cells, which were then transplanted into alymphoid RAG2/gamma(c) double-deficient recipient mice. Circulating lymphocytes appeared 4 weeks postgraft and achieved steady-state levels by 8 weeks. The mature lymphocytes present in the grafted mice had integrated the gamma(c) transgene, expressed gamma(c) transcripts, and were able to proliferate in response to y, dependent cytokines. The gamma(c)-transduced animals demonstrated (1) normal levels of immunoglobulin subclasses, including immunoglobulin G1 (IgG1) and IgG2a (which are severely decreased in gamma(c)(-) mice); (a) the ability to mount an antigen-specific, T-dependent antibody response showing effective in vivo T-B cell cooperation, and (3) the presence of gut-associated cryptopatches and intraepithelial lymphocytes, Importantly, peripheral B and T cells were still present 47 weeks after a primary graft, and animals receiving a secondary graft of gamma(c)-transduced bone marrow cells demonstrated peripheral lymphoid reconstitution. That gamma(c) gene transfer to hematopoietic precursor cells can correct the immune system abnormalities In gamma(c)(-) mice supports the feasibility of In vivo retroviral gene transfer as a treatment for human SCIDX1, (C) 2000 by The American Society of Hematology.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 06/07/20 alle ore 08:56:05