Dataset Information


SMA mouse tissue exon array analysis

ABSTRACT: We used Affymetrix exon arrays to identify potential changes in alternative splicing between mice affect with spinal muscular atropy (SMA) and their unaffected littermates. Keywords: disease state analysis Overall design: Total RNA was extracted from spinal cord, brain and kidney of three SMA and three control mice on postnatal day 11

INSTRUMENT(S): [MoEx-1_0-st] Affymetrix Mouse Exon 1.0 ST Array [probe set (exon) version]

SUBMITTER: Gideon Dreyfuss 

PROVIDER: GSE10599 | GEO | 2008-05-16



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SMN deficiency causes tissue-specific perturbations in the repertoire of snRNAs and widespread defects in splicing.

Zhang Zhenxi Z   Lotti Francesco F   Dittmar Kimberly K   Younis Ihab I   Wan Lili L   Kasim Mumtaz M   Dreyfuss Gideon G  

Cell 20080501 4

The survival of motor neurons (SMN) protein is essential for the biogenesis of small nuclear RNA (snRNA)-ribonucleoproteins (snRNPs), the major components of the pre-mRNA splicing machinery. Though it is ubiquitously expressed, SMN deficiency causes the motor neuron degenerative disease spinal muscular atrophy (SMA). We show here that SMN deficiency, similar to that which occurs in severe SMA, has unexpected cell type-specific effects on the repertoire of snRNAs and mRNAs. It alters the stoichio  ...[more]

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