RNA metabolism in neurodegenerative disease

被引：102

作者：

Liu, Elaine Y. ^{[1
]}

Cali, Christopher P. ^{[1
]}

Lee, Edward B. ^{[1
]}

机构：

[1] Univ Penn, Perelman Sch Med, Translat Neuropathol Res Labs, 613A Stellar Chance Labs, Philadelphia, PA 19104 USA

来源：

DISEASE MODELS & MECHANISMS | 2017年 / 10卷 / 05期

基金：

美国国家卫生研究院;

关键词：

Disease; RNA binding proteins; Microsatellite repeats; miRNA; tRNA; lncRNA; RNA; AMYOTROPHIC-LATERAL-SCLEROSIS; DNA-BINDING PROTEIN; FRONTOTEMPORAL LOBAR DEGENERATION; AMYLOID PRECURSOR PROTEIN; LOSS-OF-FUNCTION; ALZHEIMERS-DISEASE; MYOTONIC-DYSTROPHY; NONCODING RNA; HEXANUCLEOTIDE REPEAT; HUNTINGTONS-DISEASE;

D O I：

10.1242/dmm.028613

中图分类号：

Q2 [细胞生物学];

学科分类号：

071009 ; 090102 ;

摘要：

Aging-related neurodegenerative diseases are progressive and fatal neurological diseases that are characterized by irreversible neuron loss and gliosis. With a growing population of aging individuals, there is a pressing need to better understand the basic biology underlying these diseases. Although diverse disease mechanisms have been implicated in neurodegeneration, a common theme of altered RNA processing has emerged as a unifying contributing factor to neurodegenerative disease. RNA processing includes a series of distinct processes, including RNA splicing, transport and stability, as well as the biogenesis of non-coding RNAs. Here, we highlight how some of these mechanisms are altered in neurodegenerative disease, including the mislocalization of RNA-binding proteins and their sequestration induced by microsatellite repeats, microRNA biogenesis alterations and defective tRNA biogenesis, as well as changes to long-intergenic non-coding RNAs. We also highlight potential therapeutic interventions for each of these mechanisms.

引用

页码：508 / 517

页数：10

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