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Titolo:
EGR2 mutations in inherited neuropathies dominant-negatively inhibit myelin gene expression
Autore:
Nagarajan, R; Svaren, J; Le, N; Araki, T; Watson, M; Milbrandt, J;
Indirizzi:
Washington Univ, Sch Med, Dept Pathol, St Louis, MO 63110 USA Washington Univ St Louis MO USA 63110 Dept Pathol, St Louis, MO 63110 USA Washington Univ, Sch Med, Dept Internal Med, St Louis, MO 63110 USA Washington Univ St Louis MO USA 63110 nternal Med, St Louis, MO 63110 USA Univ Wisconsin, Sch Vet Med, Dept Comparat Biosci, Madison, WI 53706 USA Univ Wisconsin Madison WI USA 53706 omparat Biosci, Madison, WI 53706 USA
Titolo Testata:
NEURON
fascicolo: 2, volume: 30, anno: 2001,
pagine: 355 - 368
SICI:
0896-6273(200105)30:2<355:EMIIND>2.0.ZU;2-Z
Fonte:
ISI
Lingua:
ENG
Soggetto:
PERIPHERAL NERVOUS-SYSTEM; CELL-ADHESION MOLECULE; MARIE-TOOTH-DISEASE; SCHWANN-CELLS; NGFI-A; TRANSCRIPTIONAL ACTIVITY; OLIGONUCLEOTIDE ARRAYS; MICE DEFICIENT; GROWTH ARREST; PROTEIN ZERO;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Life Sciences
Citazioni:
68
Recensione:
Indirizzi per estratti:
Indirizzo: Milbrandt, J Washington Univ, Sch Med, Dept Pathol, 660 S Euclid Ave,Box 8118, St Louis, MO 63110 USA Washington Univ 660 S Euclid Ave,Box 8118 St Louis MO USA 63110
Citazione:
R. Nagarajan et al., "EGR2 mutations in inherited neuropathies dominant-negatively inhibit myelin gene expression", NEURON, 30(2), 2001, pp. 355-368

Abstract

The identification of EGR2 mutations in patients with neuropathies and thephenotype Egr2/Krox20(-/-) have demonstrated that the Egr2 transcription factor is critical for peripheral nerve myelination. However, the mechanism by which these mutations cause disease remains unclear, as most patients present with disease in the heterozygous state, whereas Egr2(+/-) mice are phenotypically normal. To understand the effect of aberrant Egr2 activity on Schwann cell gene expression, we performed microarray expression profiling to identify genes regulated by Egr2 in Schwann cells. These include genes encoding myelin proteins and enzymes required for synthesis of normal myelinlipids. Using these newly identified targets, we have shown that neuropathy-associated EGR2 mutants dominant-negatively inhibit wild-type Egr2-mediated expression of essential myelin genes to levels sufficiently low to result in the abnormal myelination observed in these patients.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 03/07/20 alle ore 00:31:18