The Survival of Motor Neuron (SMN) Protein Interacts with the mRNA-Binding Protein HuD and Regulates Localization of Poly(A) mRNA in Primary Motor Neuron Axons
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چکیده
منابع مشابه
The survival of motor neuron (SMN) protein interacts with the mRNA-binding protein HuD and regulates localization of poly(A) mRNA in primary motor neuron axons.
Spinal muscular atrophy (SMA) results from reduced levels of the survival of motor neuron (SMN) protein, which has a well characterized function in spliceosomal small nuclear ribonucleoprotein assembly. Currently, it is not understood how deficiency of a housekeeping protein leads to the selective degeneration of spinal cord motor neurons. Numerous studies have shown that SMN is present in neur...
متن کاملNeurobiology of Disease The Survival of Motor Neuron (SMN) Protein Interacts with the mRNA-Binding Protein HuD and Regulates Localization of Poly(A) mRNA in Primary Motor Neuron Axons
Claudia Fallini,1* Honglai Zhang,3* Yuehang Su,1 Vincenzo Silani,4 Robert H. Singer,3 Wilfried Rossoll,1 and Gary J. Bassell1,2 1Department of Cell Biology and 2Department of Neurology and Center for Neurodegenerative Diseases, Emory University School of Medicine, Atlanta, Georgia 30322, 3Department of Anatomy and Structural Biology, Albert Einstein College of Medicine, Bronx, New York 10461, a...
متن کاملDynamics of survival of motor neuron (SMN) protein interaction with the mRNA-binding protein IMP1 facilitates its trafficking into motor neuron axons.
Spinal muscular atrophy (SMA) is a lethal neurodegenerative disease specifically affecting spinal motor neurons. SMA is caused by the homozygous deletion or mutation of the survival of motor neuron 1 (SMN1) gene. The SMN protein plays an essential role in the assembly of spliceosomal ribonucleoproteins. However, it is still unclear how low levels of the ubiquitously expressed SMN protein lead t...
متن کاملInvolvement of survival motor neuron (SMN) protein in cell death.
Infantile spinal muscular atrophy (SMA) is caused by mutations in the survival motor neuron (SMN)1 gene. We investigated the role of human (h) SMN protein on cell death in PC12 and Rat-1 cells. hSMN prolonged cell survival in PC12 cells deprived of trophic support and in Rat-1 cells induced to die by activation of the proto-oncogene c-Myc, to similar magnitude as Bcl-2 or IAP-2. While hSMN was ...
متن کاملHuD interacts with survival motor neuron protein and can rescue spinal muscular atrophy-like neuronal defects.
Spinal muscular atrophy is an autosomal-recessive neuromuscular disease caused by disruption of the survival of motor neuron (SMN) gene, which promotes cytoplasmic assembly of the splicing core machinery. It remains unclear how a deficiency in SMN results in a disorder leading to selective degeneration of lower motor neurons. We report here that SMN interacts with RNA-binding protein HuD in neu...
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ژورنال
عنوان ژورنال: Journal of Neuroscience
سال: 2011
ISSN: 0270-6474,1529-2401
DOI: 10.1523/jneurosci.3631-10.2011