carnitine palmitoyltransferase

car·ni·tine palmi·to·yl·trans·fer·ase

1. an enzyme that reversibly forms acylcarnitines and coenzyme A from carnitine and acylcoenzyme A (often, palmitoyl-CoA); important in fatty acid oxidation. Deficiency of isozyme I results in ketogenesis with hypoglycemia; deficiency of isozyme II affects primarily skeletal muscle.

carnitine palmitoyltransferase

[kär′nitēn päl′mitō′əltrans′fərās]
an enzyme that catalyzes the transfer between coenzyme A and carnitine of long-chain fatty acids. Deficiency is a cause of defective fatty acid oxidation. Also called carnitine palmityltransferase.
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The expression levels of 10 genes: retinoid X receptor alpha (RXRA), peroxisome proliferator-activated receptor gamma (PPARG), phospholipid transfer protein (PLTP), stearoylCoA desaturase (SCD), nuclear receptor subfamily 1 group H member 3 (NR1H3), fatty acid binding protein 3 (FABP3), carnitine palmitoyltransferase II (CPT2), acyl-Coenzyme A dehydrogenase long chain (ACADL), acyl-Coenzyme A oxidase 2 branched chain (ACOX2), and fatty acid binding protein 4 (FABP4), showed significant differences in gene expression between the low- and high-marbled groups (p < 0.
In neonates, carnitine palmitoyltransferase deficiency is diagnosed in blood using mass spectrometry.
A catalyzed by carnitine palmitoyltransferase (CPT).
We investigated whether the age-related impairment of cardiac fatty acid catabolism occurs, at least partially, through diminished levels of L-carnitine, which would adversely affect carnitine palmitoyltransferase 1 (CPT1), the rate-limiting enzyme for fatty acyl-CoA uptake into mitochondria for beta]-oxidation.
For example, preliminary evidence suggests that carnitine palmitoyltransferase type 1A deficiency, a fatty acid disorder highly prevalent in AN and other indigenous circumpolar populations, might contribute to infant mortality (8).
Background & Purpose: Carnitine Palmitoyltransferase II (CPT2) deficiency (myopathic form) is a rare, progressive adult metabolic myopathy caused by a genetic insufficiency in CPT2 enzyme production.
Carnitine is supplied into the cell by a plasma membrane carnitine transporter commonly referred to as OCTN2, located in the cellular membrane, and three enzymes: carnitine palmitoyltransferase I (CPT I) in the outer mitochondrial membrane; carnitine/acylcarnitine translocase (CACT) within the inner mitochondrial membrane, and carnitine palmitoyltransferase (CPT II) in the inner mitochondrial membrane (carnitine O-palmitoyltransferase EC 2.
The team also observed an increase in these cells of carnitine palmitoyltransferase 1C (CPT1C), a protein usually found only in the brains of healthy individuals.
Fasting during first days of life can trigger disorder in [beta] oxidation of fatty acids--deficiency of carnitine palmitoyltransferase II.
Anaesthetic management of a parturient with carnitine palmitoyltransferase 2 deficiency.
However, there have been reports of the activity of carnitine palmitoyltransferase as the essential enzyme for the physiological expression of deacylation-reacylation process, within the phospholipid fatty acid membrane of human erythrocytes (14, 15).
It is important to note than increased C14 and C14:1 may be produced in other disorders as well, such as carnitine palmitoyltransferase II (CPT II), carnitine/acylcarnitine translocase (CACT), MADD, and long-chain 3-hydroxyacyl-CoA dehydrogenase (LCHAD) deficiency.
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