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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...
Autores principales: | , , , , , , , , , , , , |
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Formato: | Texto |
Lenguaje: | English |
Publicado: |
Public Library of Science
2008
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2291193/ https://www.ncbi.nlm.nih.gov/pubmed/18437205 http://dx.doi.org/10.1371/journal.pgen.1000061 |