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  Gene targeting of Gemin2 in mice reveals a correlation between defects in the biogenesis of U snRNPs and motoneuron cell death

Jablonka, S., Holtmann, B., Meister, G., Bandilla, M., Rossoll, W., Fischer, U., et al. (2002). Gene targeting of Gemin2 in mice reveals a correlation between defects in the biogenesis of U snRNPs and motoneuron cell death. Proceedings of the National Academy of Sciences of the United States of America, 99(15), 10126-10131.

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Item Permalink: https://hdl.handle.net/11858/00-001M-0000-0010-6EB2-D Version Permalink: https://hdl.handle.net/11858/00-001M-0000-0010-6EB3-B
Genre: Journal Article
Alternative Title : Proc. Natl. Acad. Sci. U. S. A.

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Jablonka, S., Author
Holtmann, B., Author
Meister, G.1, Author           
Bandilla, M.1, Author           
Rossoll, W., Author
Fischer, U.1, Author           
Sendtner, M., Author
Affiliations:
1Former Research Groups, Max Planck Institute of Biochemistry, Max Planck Society, ou_1565145              

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 Abstract: Neuronal degeneration in spinal muscular atrophy is caused by reduced expression of the survival motor neuron (SMN) protein. SMN and the tightly interacting Gemin2 form part of a macromolecular complex (SMN complex) that mediates assembly of spliceo-somal small nuclear ribonucleoproteins (U snRNPs). We used mouse genetics to investigate the function of this complex in motoneuron maintenance. Reduced Smn/Gemin2 protein levels lead to disturbed U snRNP assembly as indicated by reduced nuclear accumulation of Sm proteins. This finding correlates with enhanced motoneuron degeneration in Gemin2(+/-) / Smn(+/-) mice. Our data provide in vivo evidence that impaired production of U snRNPs contributes to motoneuron degeneration.

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Language(s): eng - English
 Dates: 2002-07-23
 Publication Status: Issued
 Pages: -
 Publishing info: -
 Table of Contents: -
 Rev. Type: Peer
 Identifiers: eDoc: 41637
ISI: 000177042400094
 Degree: -

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Title: Proceedings of the National Academy of Sciences of the United States of America
  Alternative Title : Proc. Natl. Acad. Sci. U. S. A.
Source Genre: Journal
 Creator(s):
Affiliations:
Publ. Info: -
Pages: - Volume / Issue: 99 (15) Sequence Number: - Start / End Page: 10126 - 10131 Identifier: ISSN: 0027-8424