Results 231 to 240 of about 21,879 (258)
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Features of carnitine palmitoyltransferase type I deficiency
Journal of Inherited Metabolic Disease, 2001AbstractCarnitine palmitoyltransferase type I (CPT I) is unique among long‐chain fatty acid oxidation enzymes in that there are two tissue‐specific isoforms, ‘hepatic’ and ‘muscle’, which are encoded by two separate genes. The ‘hepatic’ isoform is expressed in liver, kidney and fibroblasts and at low levels in the heart, while the other isoform occurs ...
S E, Olpin +12 more
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Journal of the Neurological Sciences, 1979
Deficiency of carnitine palmitoyltransferase II (CPT II), was found to be the cause of the syndrome of muscle pain and myoglobinuria following strenuous exercise in an otherwise healthy young man. During fasting, serum creatine kinase remained low and ketogenesis was normal.
H R, Scholte, F G, Jennekens, J J, Bouvy
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Deficiency of carnitine palmitoyltransferase II (CPT II), was found to be the cause of the syndrome of muscle pain and myoglobinuria following strenuous exercise in an otherwise healthy young man. During fasting, serum creatine kinase remained low and ketogenesis was normal.
H R, Scholte, F G, Jennekens, J J, Bouvy
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Lipids, 1998
AbstractSalmon farmers are currently using high‐energy feeds containing up to 35% fat; the fish's capability of fully utilizing these high‐energy feeds has received little attention. Carnitine is an essential component in the process of mitochondrial fatty acid oxidation and, with the cooperation of two carnitine palmitoyltransferases (CPT‐I and CPT‐II)
L, Frøyland +4 more
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AbstractSalmon farmers are currently using high‐energy feeds containing up to 35% fat; the fish's capability of fully utilizing these high‐energy feeds has received little attention. Carnitine is an essential component in the process of mitochondrial fatty acid oxidation and, with the cooperation of two carnitine palmitoyltransferases (CPT‐I and CPT‐II)
L, Frøyland +4 more
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Measurement of carnitine palmitoyltransferase I in hepatocyte monolayers
Biochemical Society Transactions, 1994Long-chain fatty acyl-groups are translocated across the mitochondrial inner membrane as acyl-carnitine esters. Camitine palmitoyltransferase I (CPT I) on the outer mitochondrial membrane, catalyzes the formation of palmitoylcarnitine from palmitoyl-CoA and camitine.
T D, Spurway +4 more
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Lethal neonatal presentation of carnitine palmitoyltransferase I deficiency
Journal of Inherited Metabolic Disease, 2001AbstractA neonate subsequently diagnosed with carnitine palmitoyl‐transferase I deficiency died at 34 h of untreatable bradycardia. There was fatty infiltration of the liver and increased free carnitine and reduced acylcarnitines in the blood.
F, Invernizzi +5 more
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Topology of Hepatic Mitochondrial Carnitine Palmitoyltransferase I
2006Our earlier work using intact mitochondria and isolated mitochondrial outer membranes confirms the observations of Murthy and Pande that CPT-I is located on the mitochondrial outer membranes and supports the notion that this enzyme has a malonyl-CoA binding domain facing the cytosol and an acyl-CoA binding domain facing the inter membrane space.
K, Kashfi, G A, Cook
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Bioorganic & Medicinal Chemistry Letters, 1999
Acylamidomorpholinium carnitine analogues, 6-(tetradecanamidomethyl- and -hexadecanamidomethyl)-4,4-dimethylmorpholin-4-ium-2-a cetate, 1, synthesized as complete sets of stereoisomers, were assayed as inhibitors for isozymes of carnitine palmitoyltransferase (CPT).
P S, Savle +3 more
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Acylamidomorpholinium carnitine analogues, 6-(tetradecanamidomethyl- and -hexadecanamidomethyl)-4,4-dimethylmorpholin-4-ium-2-a cetate, 1, synthesized as complete sets of stereoisomers, were assayed as inhibitors for isozymes of carnitine palmitoyltransferase (CPT).
P S, Savle +3 more
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High intensity exercise inhibits carnitine palmitoyltransferase-I sensitivity to
Abstract The decline in fat oxidation at higher power outputs of exercise is a complex interaction between several mechanisms; however, the influence of mitochondrial bioenergetics in this process remains elusive. Therefore, using permeabilized muscle fibers from mouse skeletal muscle, we aimed to determine if acute exercise altered ...
Heather L. Petrick, Graham P. Holloway
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Rat Liver Mitochondrial Contact Sites and Carnitine Palmitoyltransferase-I
Archives of Biochemistry and Biophysics, 2001In hepatic mitochondria, the outer membrane enzyme, carnitine palmitoyltransferase-I (CPT-I), appears to colocalize with contact sites. We have prepared contact sites that are essentially devoid of noncontact site membranes. The contact site fraction has a high specific activity for CPT-I and contains a protein at 88 kDa that is recognized by ...
C, Hoppel +3 more
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Effects of carnitine palmitoyltransferase I inhibitors on hepatic hypertrophy
European Journal of Pharmacology, 2000We investigated the effect of two types of carnitine palmitoyltransferase I inhibitors, ethyl 2-(6-(4-chlorophenoxy)hexyl)oxirane-2-carboxylate (etomoxir) and (R)-3-carboxy-N,N, N-trimethyl-2-¿[hydroxy(tetradecyloxy)phosphinyl]oxy¿-1-propana minium hydroxide (SDZ CPI 975), on cardiac and hepatic hypertrophy in ddY mice.
T, Yotsumoto +3 more
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