TY - JOUR
T1 - Tissue distribution of carnitine biosynthetic enzymes in man
AU - Rebouche, Charles J.
AU - Engel, Andrew G.
N1 - Funding Information:
This work was supported by a Research Center Grant from the Muscular Dystrophy Association and NIH grant NS 6277.
PY - 1980/6/5
Y1 - 1980/6/5
N2 - The distribution in human tissues of enzymes which convert ε{lunate}-N-trimethyl-L-lysine to L-carnitine was studied. Existing methodology was modified and new procedures were developed to measure enzyme activities. ε{lunate}-N-Trimethyl-L-lysine was converted to γ-butyrobetaine in three enzymatic steps (hydroxylation at carbon 3, aldol cleavage between carbons 2 and 3 to yield glycine and γ-trimethlaminobutyraldehyde, and subsequent oxidation of the aldehyde) in all tissues studied (liver, brain, kidney, heart and skeletal muscle), but γ-butyrobetaine was hydroxylated to form L-carnitine only in liver, kidney and brain. γ-Butyrobetaine hydroxylase (4-trimethylaminobutyrate, 2-oxoglutarate : oxygen oxidoreductase (3-hydroxylating), EC 1.14.11.1) activity in liver was dependent on the age of the subject. The activity rose from 12% in infants to 100% of the adult mean by age 15 years. No age dependence could be demonstrated for the other three enzymes studied.
AB - The distribution in human tissues of enzymes which convert ε{lunate}-N-trimethyl-L-lysine to L-carnitine was studied. Existing methodology was modified and new procedures were developed to measure enzyme activities. ε{lunate}-N-Trimethyl-L-lysine was converted to γ-butyrobetaine in three enzymatic steps (hydroxylation at carbon 3, aldol cleavage between carbons 2 and 3 to yield glycine and γ-trimethlaminobutyraldehyde, and subsequent oxidation of the aldehyde) in all tissues studied (liver, brain, kidney, heart and skeletal muscle), but γ-butyrobetaine was hydroxylated to form L-carnitine only in liver, kidney and brain. γ-Butyrobetaine hydroxylase (4-trimethylaminobutyrate, 2-oxoglutarate : oxygen oxidoreductase (3-hydroxylating), EC 1.14.11.1) activity in liver was dependent on the age of the subject. The activity rose from 12% in infants to 100% of the adult mean by age 15 years. No age dependence could be demonstrated for the other three enzymes studied.
KW - Carnitine biosynthesis
KW - Enzyme distribution
KW - Trimethyllysine
KW - γ-Butyrobetaine (Man)
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U2 - 10.1016/0304-4165(80)90133-6
DO - 10.1016/0304-4165(80)90133-6
M3 - Article
C2 - 6770910
AN - SCOPUS:0019310364
SN - 0304-4165
VL - 630
SP - 22
EP - 29
JO - BBA - General Subjects
JF - BBA - General Subjects
IS - 1
ER -