Repeat-Associated Non-ATG Translation in Neurological Diseases | Zendy

Tao Zu | Zendy; Amrutha Pattamatta | Zendy; Laura P.W. Ranum | Zendy

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Repeat-Associated Non-ATG Translation in Neurological Diseases

Author(s) -

Tao Zu,

Amrutha Pattamatta,

Laura P.W. Ranum

Publication year - 2018

Publication title -

cold spring harbor perspectives in biology

Language(s) - English

Resource type - Journals

SCImago Journal Rank - 6.011

H-Index - 173

ISSN - 1943-0264

DOI - 10.1101/cshperspect.a033019

Subject(s) - biology , myotonic dystrophy , trinucleotide repeat expansion , spinocerebellar ataxia , genetics , translation (biology) , open reading frame , untranslated region , start codon , gene , intron , ran , computational biology , stop codon , coding region , allele , microbiology and biotechnology , messenger rna , peptide sequence

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.

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