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.
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Authors | J E McGeoch, D N Palmer |
Journal | Molecular genetics and metabolism
(Mol Genet Metab)
Vol. 66
Issue 4
Pg. 387-92
(Apr 1999)
ISSN: 1096-7192 [Print] United States |
PMID | 10191134
(Publication Type: Journal Article, Research Support, Non-U.S. Gov't, Research Support, U.S. Gov't, Non-P.H.S.)
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Copyright | Copyright 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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