Is amyotrophic lateral sclerosis/frontotemporal dementia an autophagy disease?

Zhiqiang Deng, Patricia Sheehan, Shi Chen, Zhenyu Yue

Research output: Contribution to journalReview articlepeer-review

39 Scopus citations

Abstract

Amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) are neurodegenerative disorders that share genetic risk factors and pathological hallmarks. Intriguingly, these shared factors result in a high rate of comorbidity of these diseases in patients. Intracellular protein aggregates are a common pathological hallmark of both diseases. Emerging evidence suggests that impaired RNA processing and disrupted protein homeostasis are two major pathogenic pathways for these diseases. Indeed, recent evidence from genetic and cellular studies of the etiology and pathogenesis of ALS-FTD has suggested that defects in autophagy may underlie various aspects of these diseases. In this review, we discuss the link between genetic mutations, autophagy dysfunction, and the pathogenesis of ALS-FTD. Although dysfunction in a variety of cellular pathways can lead to these diseases, we provide evidence that ALS-FTD is, in many cases, an autophagy disease.

Original languageEnglish
Article number90
JournalMolecular Neurodegeneration
Volume12
Issue number1
DOIs
StatePublished - 28 Dec 2017

Keywords

  • Amyotrophic lateral sclerosis
  • Autophagy
  • Autophagy-related genes
  • Disease-associated genes
  • Frontotemporal dementia

Fingerprint

Dive into the research topics of 'Is amyotrophic lateral sclerosis/frontotemporal dementia an autophagy disease?'. Together they form a unique fingerprint.

Cite this