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Ion pores made of mitochondrial ATP synthase subunit c in the neuronal plasma membrane and Batten disease.

Abstract
A hypothesis is outlined that the neurodegeneration of the Batten disease syndromes that involve an overaccumulation of subunit c is caused by a newly characterized function of the protein, its ability to assemble in the plasma membrane into ion pores (J. E. M. McGeoch and G. Guidotti, Brain Res 766: 188-194, 1997), rendering the cell liable to constant electrical excitability to a degree that causes cell death.
AuthorsJ E McGeoch, D N Palmer
JournalMolecular genetics and metabolism (Mol Genet Metab) Vol. 66 Issue 4 Pg. 387-92 (Apr 1999) ISSN: 1096-7192 [Print] United States
PMID10191134 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't, Research Support, U.S. Gov't, Non-P.H.S.)
CopyrightCopyright 1999 Academic Press.
Chemical References
  • Ion Channels
  • Proton-Translocating ATPases
Topics
  • Cell Membrane (metabolism)
  • Electrophysiology
  • Humans
  • Ion Channels (metabolism)
  • Mitochondria (enzymology)
  • Models, Biological
  • Neuronal Ceroid-Lipofuscinoses (metabolism)
  • Patch-Clamp Techniques
  • Proton-Translocating ATPases (metabolism)

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