Ketogenic flux from lipids and leucine, assessment in 3-hydroxy-3-methylglutaryl CoA lyase deficiency.
Holmes
H C
HC
Medical Unit (Cellular and Molecular Mechanisms Research Group), London Hospital Medical College, UK.
Burns
S P
SP
Chalmers
R A
RA
Bain
M S
MS
Iles
R A
RA
D002331:Carnitine / Q000008:administration & dosage; D002648:Child; D006801:Humans; D007657:Ketone Bodies / Q000378:metabolism*; D007930:Leucine / Q000378:metabolism*; D050356:Lipid Metabolism; D009682:Magnetic Resonance Spectroscopy; D008297:Male; D007652:Oxo-Acid-Lyases / Q000172:deficiency*; D014631:Valerates / Q000652:urine
D007657:Ketone Bodies; D014631:Valerates; C004961:beta-hydroxyisovaleric acid; D007652:Oxo-Acid-Lyases; C021410:3-hydroxy-3-methylglutaryl-coenzyme A lyase; D007930:Leucine; D002331:Carnitine