RNA quality control and protein aggregates in amyotrophic lateral sclerosis: A review

Ashok Verma, Rup Tandan

Research output: Contribution to journalArticle

16 Citations (Scopus)

Abstract

Amyotrophic lateral sclerosis (ALS) is the most common motor neuron disease in adults. The biologic basis of ALS remains unknown. However, ALS research has taken a dramatic turn over the past 4 years. Ground breaking discoveries of mutations of genes that encode RNA processing proteins, and demonstration that abnormal aggregates of these and other proteins precede motor neuron loss in familial and sporadic ALS, have initiated a paradigm shift in understanding the pathogenic mechanisms of ALS. Curiously, some of these RNA binding proteins have prion-like domains, with a propensity to self-aggregation. The emerging hypothesis that a focal cascade of toxic protein aggregates, and their consequent non-cell-autonomous spread to neighborhood groups of neurons, fits the classical temporo-spatial progression of ALS. This article reviews the current research efforts toward understanding the role of RNA-processing regulation and protein aggregates in ALS.

Original languageEnglish
Pages (from-to)330-338
Number of pages9
JournalMuscle and Nerve
Volume47
Issue number3
DOIs
StatePublished - Mar 1 2013

Fingerprint

Amyotrophic Lateral Sclerosis
Quality Control
RNA
Motor Neuron Disease
RNA-Binding Proteins
Prions
Poisons
Genetic Association Studies
Motor Neurons
Research
Protein Aggregates
Neurons
Mutation
Proteins

Keywords

  • ALS
  • FUS/TLS
  • Protein aggregates
  • RNA metabolism
  • TDP-43

ASJC Scopus subject areas

  • Clinical Neurology
  • Cellular and Molecular Neuroscience
  • Physiology (medical)
  • Physiology

Cite this

RNA quality control and protein aggregates in amyotrophic lateral sclerosis : A review. / Verma, Ashok; Tandan, Rup.

In: Muscle and Nerve, Vol. 47, No. 3, 01.03.2013, p. 330-338.

Research output: Contribution to journalArticle

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