Thelen, Maximilian Paul and Kye, Min Jeong ORCID: 0000-0002-1323-7256 (2020). The Role of RNA Binding Proteins for Local mRNA Translation: Implications in Neurological Disorders. Front. Mol. Biosci., 6. LAUSANNE: FRONTIERS MEDIA SA. ISSN 2296-889X

Full text not available from this repository.

Abstract

As neurons are one of the most highly polarized cells in our body, they require sophisticated cellular mechanisms to maintain protein homeostasis in their subcellular compartments such as axons and dendrites. When neuronal protein homeostasis is disturbed due to genetic mutations or deletions, this often results in degeneration of neurons leading to devastating outcome such as spinal muscular atrophy (SMA), amyotrophic lateral sclerosis (ALS), and fragile X syndrome (FXS). Ribonucleoprotein (RNP) complexes are macromolecular complexes composed of RNA binding proteins (RBPs) and their target RNAs. RBPs contain RNA binding domains and bind to RNA molecules via specific sequence motifs. RNP complexes have various functions in gene expression including messenger RNA (mRNA) trafficking, RNA processing and silencing. In neurons, RBPs deliver specific sets of mRNAs to subcellular compartments such as axons and dendrites to be locally translated. Mutations or deletions in genes coding for RNPs have been reported as causes for neurological disorders such as SMA, ALS, and FXS. As RBPs determine axonal or dendritic mRNA repertoires as well as proteomes by trafficking selective mRNAs and regulating local protein synthesis, they play a crucial role for neuronal function. In this review, we summarize the role of well-known RBPs, SMN, TDP-43, FUS, and FMRP, and review their function for local protein synthesis in neurons. Furthermore, we discuss their pathological contribution to the neurological disorders.

Item Type: Journal Article
Creators:
CreatorsEmailORCIDORCID Put Code
Thelen, Maximilian PaulUNSPECIFIEDUNSPECIFIEDUNSPECIFIED
Kye, Min JeongUNSPECIFIEDorcid.org/0000-0002-1323-7256UNSPECIFIED
URN: urn:nbn:de:hbz:38-348779
DOI: 10.3389/fmolb.2019.00161
Journal or Publication Title: Front. Mol. Biosci.
Volume: 6
Date: 2020
Publisher: FRONTIERS MEDIA SA
Place of Publication: LAUSANNE
ISSN: 2296-889X
Language: English
Faculty: Unspecified
Divisions: Unspecified
Subjects: no entry
Uncontrolled Keywords:
KeywordsLanguage
SPINAL MUSCULAR-ATROPHY; MOTOR-NEURON SMN; MENTAL-RETARDATION PROTEIN; ALS-ASSOCIATED MUTATIONS; STRESS GRANULE DYNAMICS; FRAGILE-X-SYNDROME; AXONAL DISTRIBUTION; TDP-43; SURVIVAL; GENEMultiple languages
Biochemistry & Molecular BiologyMultiple languages
URI: http://kups.ub.uni-koeln.de/id/eprint/34877

Downloads

Downloads per month over past year

Altmetric

Export

Actions (login required)

View Item View Item