Abstract
Electron transfer flavoprotein (composed of alpha and beta subunits) is an obligatory electron acceptor for several dehydrogenases and is located in the mitochondrial matrix. Electrons accepted by electron transfer flavoprotein (ETF) are transferred to the main mitochondrial respiratory chain by way of ETF dehydrogenase (ETFDH). In humans, deficiency of ETF or ETFDH leads to glutaric acidemia type II, an inherited metabolic disorder that can be fatal in its neonatal form and is characterized by severe hypoketotic hypoglycemia and acidosis. We used cDNA probes for the Etfdh, Etfb, and Etfa genes to determine localization of these mouse genes to chromosomes 3, 7, and 13.
Publication types
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Comparative Study
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Research Support, Non-U.S. Gov't
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Research Support, U.S. Gov't, P.H.S.
MeSH terms
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Amino Acid Metabolism, Inborn Errors / genetics*
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Animals
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Carbohydrate Metabolism, Inborn Errors / genetics
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Chromosome Mapping
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Electron-Transferring Flavoproteins
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Fatty Acid Desaturases / genetics*
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Flavoproteins / genetics*
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Genes
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Genetic Linkage
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Glutarates / blood*
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Humans
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Iron-Sulfur Proteins*
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Mice
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Multienzyme Complexes / genetics*
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Oxidoreductases Acting on CH-NH Group Donors*
Substances
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Electron-Transferring Flavoproteins
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Flavoproteins
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Glutarates
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Iron-Sulfur Proteins
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Multienzyme Complexes
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Fatty Acid Desaturases
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Oxidoreductases Acting on CH-NH Group Donors
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electron-transferring-flavoprotein dehydrogenase
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glutaric acid