Electron transfer flavoprotein defect

Glutaric acidurias Type I Primary defect of glutarate oxidation Type II Defect of electron transfer flavoprotein Type I Severe basal ganglia/cerebellar disease with macrocephaly. Onset 1-2 years Type II Fulminant neurological syndrome of the neonate. Often with renal/hepatic cysts. Usually fatal Diet low in lysine and tryptophan Supplementation with coenzyme Q, riboflavin, carnitine... 

Christensen E, Kolvraa S, and Gregersen N (1984) Glutaric aciduria type II evidence for a defect related to the electron transfer flavoprotein or its dehydrogenase. Pediatric Research 18, 663-7. 

Frerman FE, Goodman SI. Defects of the electron transfer flavoprotein and electron transfer flavopro-tein-ubiquinone oxidoreductase glutaric acidemia type II. In Scriver CR, Beaudet AL, Valle D, Sly WS, Childs B, Kinzler KW, et al, eds. The metabofic molecular bases of inherited disease, 8th ed. New York McGraw-Hill, 2001 2357-65. 

B (electron transfer flavoprotein-u) Multiple acyl-CoA dehydrogenation defect ETF-/ FB 19ql3 3 130410... 

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