Proteasome inhibitors to alleviate aberrant IKBKAP mRNA splicing and low IKAP/hELP1 synthesis in familial dysautonomia

Proteasome inhibitors to alleviate aberrant IKBKAP mRNA splicing and low IKAP/hELP1 synthesis in familial dysautonomia

FD is a rare neurodegenerative disorder caused by a mutation of the IKBKAP gene, which induces low expression levels of the Elongator subunit IKAP/hELP1 protein. A rational strategy for FD treatment could be to identify drugs increasing IKAP/hELP1 expression levels by blocking protein degradation pa...

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Bibliographic Details
Main Authors: Mylène Hervé, El Chérif Ibrahim
Format: Article
Language:English
Published: Elsevier 2017-07-01
Series:Neurobiology of Disease
Subjects:
Familial dysautonomia
Proteasome
Carfilzomib
IKBKAP
mRNA splicing
MicroRNA
Online Access:http://www.sciencedirect.com/science/article/pii/S0969996117300839

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http://www.sciencedirect.com/science/article/pii/S0969996117300839

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