Disruption of snRNP biogenesis factors Tgs1 and pICln induces phenotypes that mirror aspects of SMN-Gemins complex perturbation in Drosophila, providing new insights into spinal muscular atrophy

The neuromuscular disorder, spinal muscular atrophy (SMA), results from insufficient levels of the survival motor neuron (SMN) protein. Together with Gemins 2–8 and Unrip, SMN forms the large macromolecular SMN-Gemins complex, which is known to be indispensable for chaperoning the assembly of splice...

Ausführliche Beschreibung

Bibliografische Detailangaben

Hauptverfasser:	Rebecca M. Borg, Benji Fenech Salerno, Neville Vassallo, Rémy Bordonne, Ruben J. Cauchi
Format:	Artigo
Sprache:	Inglês
Veröffentlicht:	Elsevier 2016-10-01
Schriftenreihe:	Neurobiology of Disease
Schlagworte:	SMN complex snRNP assembly Unrip Strap wmd pICln
Online-Zugang:	http://www.sciencedirect.com/science/article/pii/S0969996116301553
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