Primary coenzyme Q deficiency in Pdss2 mutant mice causes isolated renal disease.
Coenzyme Q (CoQ) is an essential electron carrier in the respiratory chain whose deficiency has been implicated in a wide variety of human mitochondrial disease manifestations. Its multi-step biosynthesis involves production of polyisoprenoid diphosphate in a reaction that requires the enzymes be en...
Main Authors: | , , , , , , , , , , , , |
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Format: | Article |
Language: | English |
Published: |
Public Library of Science (PLoS)
2008-04-01
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Series: | PLoS Genetics |
Online Access: | http://europepmc.org/articles/PMC2291193?pdf=render |