Is amyotrophic lateral sclerosis/frontotemporal dementia an autophagy disease?

Zhiqiang Deng, Patricia Sheehan, Shi Chen*, Zhenyu Yue*

*Corresponding author for this work

Research output: Contribution to journalReview articlepeer-review

39 Citations (Scopus)


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
Number of pages11
JournalMolecular Neurodegeneration
Publication statusPublished - 28 Dec 2017

User-Defined Keywords

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


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