Article
Insertion of proteolipid protein into oligodendrocyte mitochondria regulates extracellular pH and adenosine triphosphate.
Glia - 1 Mar 2014
Appikatla Sunita, Bessert Denise, Lee Icksoo, Hüttemann Maik, Mullins Chadwick, Somayajulu-Nitu Mallika, Yao Fayi, Skoff Robert P
Abstract excerpt
Proteolipid protein (PLP) and DM20, the most abundant myelin proteins, are coded by the human PLP1 and non-human Plp1 PLP gene. Mutations in the PLP1 gene cause Pelizaeus-Merzbacher disease (PMD) with duplications of the native PLP1 gene accounting for 70% of PLP1 mutations. Humans with PLP1 duplications and mice with extra Plp1 copies have extensive neuronal degeneration. The mechanism that causes neuronal...
Topics
- Adenosine Triphosphate
- Animals
- Animals, Newborn
- Brain
- Cells, Cultured
- Chlorocebus aethiops
- Electron Transport Complex IV
- Extracellular Fluid
- Female
- Gene Expression Regulation
- Humans
- Hydrogen-Ion Concentration
- Lactic Acid