Binding proteins for mRNA localization and local translation, and their dysfunction in genetic neurological disease.
Neurons utilize mRNA transport and local translation as a means to influence development and plasticity. The molecular mechanisms for this mRNA sorting involve the recognition of cis-acting sequences by distinct mRNA binding proteins that have a dual role, acting in both mRNA transport and translational regulation. Other proteins play a part in the assembly of messenger ribonucleoprotein complexes into transport granules. mRNA binding proteins are crucial targets of phosphorylation signals that regulate local translation. Fragile X syndrome and spinal muscular atrophy have emerged as two genetic neurological diseases that could result, in part, from impaired assembly, localization, and translational regulation of these messenger ribonucleoproteins.
Bassell
Gary J
GJ
Department of Neuroscience, Rose Kennedy Center for Mental Retardation, Albert Einstein College of Medicine, 1410 Pelham Parkway, Bronx, New York 10461, New York, USA. bassell@aecom.yu.edu
Kelic
Sofija
S
D021581:Active Transport, Cell Nucleus / Q000235:genetics; D000818:Animals; D002490:Central Nervous System / Q000378:metabolism* / Q000503:physiopathology; D005600:Fragile X Syndrome / Q000235:genetics / Q000378:metabolism; D020271:Heredodegenerative Disorders, Nervous System / Q000235:genetics* / Q000378:metabolism / Q000503:physiopathology; D006801:Humans; D009134:Muscular Atrophy, Spinal / Q000235:genetics / Q000378:metabolism; D014176:Protein Biosynthesis / Q000235:genetics; D012333:RNA, Messenger / Q000378:metabolism*; D016601:RNA-Binding Proteins / Q000235:genetics / Q000378:metabolism*
D012333:RNA, Messenger; D016601:RNA-Binding Proteins