Better understanding the neurobiology of primary lateral sclerosis

P. Hande Ozdinler, Mukesh Gautam, Oge Gozutok, Csaba Konrad, Giovanni Manfredi, Estela Area Gomez, Hiroshi Mitsumoto, Marcella L. Erb, Zheng Tian, Georg Haase*

*Corresponding author for this work

Research output: Contribution to journalReview articlepeer-review

2 Scopus citations

Abstract

Primary lateral sclerosis (PLS) is a rare neurodegenerative disease characterized by progressive degeneration of upper motor neurons (UMNs). Recent studies shed new light onto the cellular events that are particularly important for UMN maintenance including intracellular trafficking, mitochondrial energy homeostasis and lipid metabolism. This review summarizes these advances including the role of Alsin as a gene linked to atypical forms of juvenile PLS, and discusses wider aspects of cellular pathology that have been observed in adult forms of PLS. The review further discusses the prospects of new transgenic upper motor neuron reporter mice, human stem cell-derived UMN cultures, cerebral organoids and non-human primates as future model systems to better understand and ultimately treat PLS.

Original languageEnglish (US)
Pages (from-to)35-46
Number of pages12
JournalAmyotrophic Lateral Sclerosis and Frontotemporal Degeneration
Volume21
Issue numberS1
DOIs
StatePublished - 2020

Keywords

  • ALS2
  • Alsin
  • Betz cell
  • Golgi apparatus
  • bioenergetics
  • corticospinal motor neuron
  • endosomes
  • membrane lipids
  • mitochondria
  • primary lateral sclerosis
  • upper motor neuron

ASJC Scopus subject areas

  • Neurology
  • Clinical Neurology

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