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Titolo:
Ablation of NF1 function in neurons induces abnormal development of cerebral cortex and reactive gliosis in the brain
Autore:
Zhu, Y; Romero, MI; Ghosh, P; Ye, ZY; Charnay, P; Rushing, EJ; Marth, JD; Parada, LF;
Indirizzi:
Univ Texas, SW Med Ctr, Ctr Dev Biol, Dallas, TX 75390 USA Univ Texas Dallas TX USA 75390 ed Ctr, Ctr Dev Biol, Dallas, TX 75390 USA Univ Texas, SW Med Ctr, Kent Waldrep Fdn Ctr, Basic Res Nerve Growth & Regenerat, Dallas, TX 75390 USA Univ Texas Dallas TX USA 75390 e Growth & Regenerat, Dallas, TX 75390 USA Univ Calif San Diego, Howard Hughes Med Inst, La Jolla, CA 92093 USA Univ Calif San Diego La Jolla CA USA 92093 d Inst, La Jolla, CA 92093 USA Univ Texas, SW Med Ctr, Dept Pathol, Dallas, TX 75390 USA Univ Texas Dallas TX USA 75390 Med Ctr, Dept Pathol, Dallas, TX 75390 USA
Titolo Testata:
GENES & DEVELOPMENT
fascicolo: 7, volume: 15, anno: 2001,
pagine: 859 - 876
SICI:
0890-9369(20010401)15:7<859:AONFIN>2.0.ZU;2-4
Fonte:
ISI
Lingua:
ENG
Soggetto:
NEUROFIBROMATOSIS TYPE-1 GENE; GTPASE-ACTIVATING PROTEIN; TUMOR-SUPPRESSOR; NERVOUS-SYSTEM; DROSOPHILA NF1; SCHWANN-CELLS; RAS P21; EXPRESSION; MICE; MUTATIONS;
Keywords:
neurofibromatosis; tumor suppressor; animal models; knockouts; astrogliosis;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Life Sciences
Citazioni:
79
Recensione:
Indirizzi per estratti:
Indirizzo: Parada, LF Univ Texas, SW Med Ctr, Ctr Dev Biol, Dallas, TX 75390 USA UnivTexas Dallas TX USA 75390 Dev Biol, Dallas, TX 75390 USA
Citazione:
Y. Zhu et al., "Ablation of NF1 function in neurons induces abnormal development of cerebral cortex and reactive gliosis in the brain", GENE DEV, 15(7), 2001, pp. 859-876

Abstract

Neurofibromatosis type 1 (NF1) is a prevalent genetic disorder that affects growth properties of neural-crest-derived cell populations. In addition, approximately one-half of NF1 patients exhibit learning disabilities. To characterize NF1 function both in vitro and in vivo, we circumvent the embryonic lethality of NF1 null mouse embryos by generating a conditional mutation in the NF1 gene using Cre/loxP technology. Introduction of a Synapsin I promoter driven Cre transgenic mouse strain into the conditional NF1 background has ablated NF1 function in most differentiated neuronal populations. These mice have abnormal development of the cerebral cortex, which suggests that NF1 has an indispensable role in this aspect of CNS development. Furthermore, although they are tumor free, these mice display extensive astrogliosis in the absence of conspicuous neurodegeneration or microgliosis. Theseresults indicate that NF1-deficient neurons are capable of inducing reactive astrogliosis via a non-cell autonomous mechanism.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 27/09/20 alle ore 13:26:25