Lipid Dysfunction and Pathogenesis of Multiple System Atrophy

Lipid Dysfunction and Pathogenesis of Multiple System Atrophy Multiple system atrophy MSA is a progressive neurodegenerative disease characterized by the accumulation of alpha synuclein protein in the cytoplasm of oligodendrocytes the myelin producing support c

  • Title: Lipid Dysfunction and Pathogenesis of Multiple System Atrophy
  • Author: Applied Research Press
  • ISBN: 9781516835409
  • Page: 354
  • Format: Paperback
  • Multiple system atrophy MSA is a progressive neurodegenerative disease characterized by the accumulation of alpha synuclein protein in the cytoplasm of oligodendrocytes, the myelin producing support cells of the central nervous system CNS The brain is the most lipid rich organ in the body and disordered metabolism of various lipid constituents is increasingly recognizMultiple system atrophy MSA is a progressive neurodegenerative disease characterized by the accumulation of alpha synuclein protein in the cytoplasm of oligodendrocytes, the myelin producing support cells of the central nervous system CNS The brain is the most lipid rich organ in the body and disordered metabolism of various lipid constituents is increasingly recognized as an important factor in the pathogenesis of several neurodegenerative diseases alpha Synuclein is a 17 kDa protein with a close association to lipid membranes and biosynthetic processes in the CNS, yet its precise function is a matter of speculation, particularly in oligodendrocytes alpha Synuclein aggregation in neurons is a well characterized feature of Parkinson s disease and dementia with Lewy bodies Epidemiological evidence and in vitro studies of alpha synuclein molecular dynamics suggest that disordered lipid homeostasis may play a role in the pathogenesis of alpha synuclein aggregation However, MSA is distinct from other alpha synucleinopathies in a number of respects, not least the disparate cellular focus of alpha synuclein pathology The recent identification of causal mutations and polymorphisms in COQ2, a gene encoding a biosynthetic enzyme for the production of the lipid soluble electron carrier coenzyme Q 10 ubiquinone , puts membrane transporters as central to MSA pathogenesis, although how such transporters are involved in the early myelin degeneration observed in MSA remains unclear The purpose of this review is to bring together available evidence to explore the potential role of membrane transporters and lipid dyshomeostasis in the pathogenesis of alpha synuclein aggregation in MSA We hypothesize that dysregulation of the specialized lipid metabolism involved in myelin synthesis and maintenance by oligodendrocytes underlies the unique neuropathology of MSA Proceeds from the sale of this book go to the support of an elderly disabled person.

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      Posted by:Applied Research Press
      Published :2019-07-10T01:50:41+00:00

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