Literature DB >> 18485864

Is good housekeeping the key to motor neuron survival?

Kevin Talbot1, Kay E Davies.   

Abstract

Spinal muscular atrophy (SMA) is caused by a drastic reduction in the ubiquitously expressed SMN protein, which is critical for the correct assembly of the snRNP complexes required for RNA splicing. However, it is unclear why loss of SMN and altered snRNP assembly only seem to affect motor neurons. Reporting in this issue, Zhang et al. (2008) challenge prior assumptions about the housekeeping function of SMN and demonstrate that loss of SMN leads to highly tissue-specific effects on splicing.

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Year:  2008        PMID: 18485864     DOI: 10.1016/j.cell.2008.05.002

Source DB:  PubMed          Journal:  Cell        ISSN: 0092-8674            Impact factor:   41.582


  7 in total

Review 1.  From phenologs to silent suppressors: Identifying potential therapeutic targets for human disease.

Authors:  Andy Golden
Journal:  Mol Reprod Dev       Date:  2017-10-03       Impact factor: 2.609

2.  Triptolide increases transcript and protein levels of survival motor neurons in human SMA fibroblasts and improves survival in SMA-like mice.

Authors:  Ya-Yun Hsu; Yuh-Jyh Jong; Hsin-Hung Tsai; Yu-Ting Tseng; Li-Mei An; Yi-Ching Lo
Journal:  Br J Pharmacol       Date:  2012-06       Impact factor: 8.739

3.  A feedback loop regulates splicing of the spinal muscular atrophy-modifying gene, SMN2.

Authors:  Francine M Jodelka; Allison D Ebert; Dominik M Duelli; Michelle L Hastings
Journal:  Hum Mol Genet       Date:  2010-09-30       Impact factor: 6.150

4.  The loss of the snoRNP chaperone Nopp140 from Cajal bodies of patient fibroblasts correlates with the severity of spinal muscular atrophy.

Authors:  Benoît Renvoisé; Sabrina Colasse; Philippe Burlet; Louis Viollet; U Thomas Meier; Suzie Lefebvre
Journal:  Hum Mol Genet       Date:  2009-01-07       Impact factor: 6.150

5.  A new cis-acting motif is required for the axonal SMN-dependent Anxa2 mRNA localization.

Authors:  Khalil Rihan; Etienne Antoine; Thomas Maurin; Barbara Bardoni; Rémy Bordonné; Johann Soret; Florence Rage
Journal:  RNA       Date:  2017-03-03       Impact factor: 4.942

6.  Sporadic ALS has compartment-specific aberrant exon splicing and altered cell-matrix adhesion biology.

Authors:  Stuart J Rabin; Jae Mun Hugo Kim; Michael Baughn; Ryan T Libby; Young Joo Kim; Yuxin Fan; Randell T Libby; Albert La Spada; Brad Stone; John Ravits
Journal:  Hum Mol Genet       Date:  2009-10-28       Impact factor: 6.150

7.  Spliceosome integrity is defective in the motor neuron diseases ALS and SMA.

Authors:  Hitomi Tsuiji; Yohei Iguchi; Asako Furuya; Ayane Kataoka; Hiroyuki Hatsuta; Naoki Atsuta; Fumiaki Tanaka; Yoshio Hashizume; Hiroyasu Akatsu; Shigeo Murayama; Gen Sobue; Koji Yamanaka
Journal:  EMBO Mol Med       Date:  2013-01-25       Impact factor: 12.137

  7 in total

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