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  Specific interaction of Smn, the spinal muscular atrophy determining gene product, with hnRNP-R and gry-rbp/hnRNP-Q: a role for Smn in RNA processing in motor axons?

Rossoll, W., Kröning, A. K., Ohndorf, U. M., Steegborn, C., Jablonka, S., & Sendtner, M. (2002). Specific interaction of Smn, the spinal muscular atrophy determining gene product, with hnRNP-R and gry-rbp/hnRNP-Q: a role for Smn in RNA processing in motor axons? Human Molecular Genetics, 11(1), 93-105.

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Genre: Zeitschriftenartikel
Alternativer Titel : Hum. Mol. Genet.

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 Urheber:
Rossoll, W., Autor
Kröning, A. K., Autor
Ohndorf, U. M., Autor
Steegborn, C.1, Autor           
Jablonka, S., Autor
Sendtner, M., Autor
Affiliations:
1External Organizations, ou_persistent22              

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 Zusammenfassung: Spinal muscular atrophy (SMA), the most common hereditary motor neuron disease in children and young adults is caused by mutations in the telomeric survival motor neuron (SMN1) gene. The human genome, in contrast to mouse, contains a second SMN gene (SMN2) which codes for a gene product which is alternatively spliced at the C-terminus, but also gives rise to low levels of full-length SMN protein. The reason why reduced levels of the ubiquitously expressed SMN protein lead to specific motor neuron degeneration without affecting other cell types is still not understood. Using yeast two-hybrid techniques, we identified hnRNP-R and the highly related gry- rbp/hnRNP-Q as novel SMN interaction partners. These proteins have previously been identified in the context of RNA processing, in particular mRNA editing, transport and splicing. hnRNP-R and gry-rbp/hnRNP-Q interact with wild-type Smn but not with truncated or mutant Smn forms identified in SMA. Both proteins are widely expressed and developmentally regulated with expression peaking at E19 in mouse spinal cord. hnRNP-R binds RNA through its RNA recognition motif domains. Interestingly, hnRNP-R is predominantly located in axons of motor neurons and co-localizes with Smn in this cellular compartment. Thus, this finding could provide a key to understand a motor neuron-specific Smn function in SMA.

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Sprache(n): eng - English
 Datum: 2002-01-01
 Publikationsstatus: Erschienen
 Seiten: -
 Ort, Verlag, Ausgabe: -
 Inhaltsverzeichnis: -
 Art der Begutachtung: Expertenbegutachtung
 Identifikatoren: eDoc: 34934
ISI: 000173544300010
 Art des Abschluß: -

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Titel: Human Molecular Genetics
  Alternativer Titel : Hum. Mol. Genet.
Genre der Quelle: Zeitschrift
 Urheber:
Affiliations:
Ort, Verlag, Ausgabe: -
Seiten: - Band / Heft: 11 (1) Artikelnummer: - Start- / Endseite: 93 - 105 Identifikator: ISSN: 0964-6906