Carnitine palmitoyltransferase 1C: From cognition to cancer
نویسندگان
چکیده
منابع مشابه
The soluble carnitine palmitoyltransferase from bovine liver
The properties of two carnitine acyltransferases (CPT) purified from bovine liver are compared to confirm that they are different proteins. The soluble CPT and the inner CPT from mitochondria differ in subunit Mr, native Mr, pI and reactivity with thiol reagents. All eight free thiol groups in soluble CPT react with 5,5'dithiobis-(2-nitrobenzoate) in the absence of any unfolding reagent, and ac...
متن کاملCarnitine palmitoyltransferase II deficiency
The authors investigated 32 patients with the muscle form of CPT II deficiency. Total carnitine palmitoyltransferase enzyme system (CPT) activity was normal but abnormally inhibited by malonyl-CoA, palmitoyl-CoA, and the detergents Triton X and Tween 20. Mutation analysis identified three described mutations (S113L, P50H, and F448L) and two novel mutations (M214T and Y479F). Using modeling tech...
متن کاملCarnitine palmitoyltransferase 1C promotes cell survival and tumor growth under conditions of metabolic stress.
Tumor cells gain a survival/growth advantage by adapting their metabolism to respond to environmental stress, a process known as metabolic transformation. The best-known aspect of metabolic transformation is the Warburg effect, whereby cancer cells up-regulate glycolysis under aerobic conditions. However, other mechanisms mediating metabolic transformation remain undefined. Here we report that ...
متن کاملFunctional and Structural Basis of Carnitine Palmitoyltransferase
Carnitine palmitoyltransferase 1A (CPT1A) is the key regulatory enzyme of hepatic long-chain fatty acid -oxidation. Human CPT1A deficiency is characterized by recurrent attacks of hypoketotic hypoglycemia. We presently analyzed at both the functional and structural levels five missense mutations identified in three CPT1A-deficient patients, namely A275T, A414V, Y498C, G709E, and G710E. Heterolo...
متن کاملTravels with carnitine palmitoyltransferase I: from liver to germ cell with stops in between.
Although malonyl-CoA was first recognized as a central component in the regulation of hepatic ketogenesis, its inhibitory effect on mitochondrial carnitine palmitoyltransferase I (CPT I) has come to be viewed as a key element in fuel sensing in a variety of body tissues. This broadening role of the malonyl-CoA/CPT I partnership in the control of glucose and fatty acid metabolism, as well as cur...
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ژورنال
عنوان ژورنال: Progress in Lipid Research
سال: 2016
ISSN: 0163-7827
DOI: 10.1016/j.plipres.2015.11.004