Repeat-Associated Non-ATG Translation in Neurological Diseases.
Cold Spring Harb Perspect Biol
; 10(12)2018 12 03.
Article
in En
| MEDLINE
| ID: mdl-29891563
ABSTRACT
More than 40 different neurological diseases are caused by microsatellite repeat expansions that locate within translated or untranslated gene regions, including 5' and 3' untranslated regions (UTRs), introns, and protein-coding regions. Expansion mutations are transcribed bidirectionally and have been shown to give rise to proteins, which are synthesized from three reading frames in the absence of an AUG initiation codon through a novel process called repeat-associated non-ATG (RAN) translation. RAN proteins, which were first described in spinocerebellar ataxia type 8 (SCA8) and myotonic dystrophy type 1 (DM1), have now been reported in a growing list of microsatellite expansion diseases. This article reviews what is currently known about RAN proteins in microsatellite expansion diseases and experiments that provide clues on how RAN translation is regulated.
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Main subject:
Protein Biosynthesis
/
Central Nervous System Diseases
Type of study:
Risk_factors_studies
Limits:
Humans
Language:
En
Journal:
Cold Spring Harb Perspect Biol
Journal subject:
BIOLOGIA
Year:
2018
Document type:
Article