Results 51 to 60 of about 4,972 (128)

A motor function for the DEAD-box RNA helicase, Gemin3, in Drosophila.

PLoS Genetics, 2008
The survival motor neuron (SMN) protein, the determining factor for spinal muscular atrophy (SMA), is complexed with a group of proteins in human cells. Gemin3 is the only RNA helicase in the SMN complex.
Ruben J Cauchi, Kay E Davies, Ji-Long Liu   +2 more
doaj   +1 more source

Diabetic polyneuropathy, sensory neurons, nuclear structure and spliceosome alterations: a role for CWC22

Disease Models & Mechanisms, 2017
Unique deficits in the function of adult sensory neurons as part of their early neurodegeneration might account for progressive polyneuropathy during chronic diabetes mellitus.
Masaki Kobayashi, Ambika Chandrasekhar, Chu Cheng, Jose A. Martinez, Hilarie Ng, Cristiane de la Hoz, Douglas W. Zochodne   +6 more
doaj   +1 more source

Oligomerization regulates the interaction of Gemin5 with members of the SMN complex and the translation machinery

Cell Death Discovery
RNA-binding proteins are multifunctional molecules impacting on multiple steps of gene regulation. Gemin5 was initially identified as a member of the survival of motor neurons (SMN) complex.
Rosario Francisco-Velilla, Salvador Abellan, Azman Embarc-Buh, Encarnacion Martinez-Salas   +3 more
doaj   +1 more source

Proteomic analysis of the SMN complex reveals conserved and etiologic connections to the proteostasis network

Frontiers in RNA Research
IntroductionMolecular chaperones and co-chaperones are highly conserved cellular components that perform a variety of duties related to the proper three-dimensional folding of the proteome.
A. Gregory Matera, A. Gregory Matera, A. Gregory Matera, Rebecca E. Steiner, C. Allie Mills, Benjamin D. McMichael, Laura E. Herring, Eric L. Garcia, Eric L. Garcia   +8 more
doaj   +1 more source

hnRNP R regulates mitochondrial movement and membrane potential in axons of motoneurons

Neurobiology of Disease
Axonal mitochondria defects are early events in the pathogenesis of motoneuron disorders such as spinal muscular atrophy and amyotrophic lateral sclerosis.
Sophia Dithmar, Abdolhossein Zare, Saeede Salehi, Michael Briese, Michael Sendtner   +4 more
doaj   +1 more source

Alternative splicing events driven by altered levels of GEMIN5 undergo translation

RNA Biology
GEMIN5 is a multifunctional protein involved in various aspects of RNA biology, including biogenesis of snRNPs and translation control. Reduced levels of GEMIN5 confer a differential translation to selective groups of mRNAs, and biallelic variants ...
Rosario Francisco-Velilla, Salvador Abellan, Juan Antonio Garcia-Martin, Juan Carlos Oliveros, Encarnacion Martinez-Salas   +4 more
doaj   +1 more source

Spliceosomal Sm core assembly: AlphaFold 3 predicted structure and phosphorylation-dependent regulation of the human 6S complex. [PDF]

Comput Struct Biotechnol J
Grimmler M, Reinhart M, Alers S, Peter C.   +3 more
europepmc   +1 more source

Novel Translational Concept: Axon-to-Muscle Exosomal Signaling as an Emerging Therapeutic Target in Spinal Muscular Atrophy. [PDF]

Biomedicines
Fajkić A, Belančić A, Lam YW, Rački V, Pilipović K, Janković T, Mežnarić S, Mršić-Pelčić J, Vitezić D.   +8 more
europepmc   +1 more source

Spinal motor neuron degeneration after brachial plexus avulsion: mechanisms and therapeutic targets. [PDF]

Front Neurosci
Yang J, Qin B, Yang Y, Fang J, He W.
europepmc   +1 more source

SMN deficiency inhibits endochondral ossification via promoting TRAF6-induced ubiquitination degradation of YBX1 in spinal muscular atrophy. [PDF]

Bone Res
Zhou Z, Fan X, Xiang T, Suo Y, Shi X, Li Y, Hua Y, Sheng L, Zhou X.   +8 more
europepmc   +1 more source