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.
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
Gary J
GJ
Bassell
Sofija
S
Kelic
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