نتایج جستجو برای: carnitine palmitoyltransferase 1a

تعداد نتایج: 38168  

Journal: :Molecular genetics and metabolism 2010
Sorcha A Collins Graham Sinclair Sarah McIntosh Fiona Bamforth Robert Thompson Isaac Sobol Geraldine Osborne Andre Corriveau Maria Santos Brendan Hanley Cheryl R Greenberg Hilary Vallance Laura Arbour

Carnitine palmitoyltransferase 1A (CPT1A), encoded by the gene CPT1A, is the hepatic isoform of CPT1 and is a major regulatory point in long-chain fatty acid oxidation. CPT1A deficiency confers risk for hypoketotic hypoglycaemia, hepatic encephalopathy, seizures, and sudden unexpected death in infancy (SUDI). It remains controversial whether the CPT1A gene variant, c.1436C>T (p.P479L), identifi...

Journal: :Biochemical Society transactions 2001
N M Broadway R J Pease G Birdsey N A Turner M Shayeghi E D Saggerson

Liver microsomes contain two carnitine acyltransferase activities. One of these has properties closely corresponding to those of 88 kDa mitochondrial carnitine palmitoyltransferase-1 (CPT-1). Antisera against CPT-1 cross-react with an 88 kDa microsomal protein, suggesting that CPT-1 may be targeted to both microsomal and mitochondrial membranes. However, no experiments using cDNAs corresponding...

Journal: :Biochemical and biophysical research communications 2004
Carine Nicot Laura Napal Joana Relat Silvia González Amadeu Llebaria Gebre Woldegiorgis Pedro F Marrero Diego Haro

Carnitine palmitoyltransferase I (CPT-I) and II (CPT-II) enzymes are components of the carnitine palmitoyltransferase shuttle system which allows entry of long-chain fatty acids into the mitochondrial matrix for subsequent oxidation. This system is tightly regulated by malonyl-CoA levels since this metabolite is a strong reversible inhibitor of the CPT-I enzyme. There are two distinct CPT-I iso...

Journal: :American journal of physiology. Endocrinology and metabolism 2013
Su Gao Dolors Serra Wendy Keung Fausto G Hegardt Gary D Lopaschuk

Carnitine palmitoyltransferase-1 (CPT-1) liver isoform, or CPT-1a, is implicated in CNS control of food intake. However, the exact brain nucleus site(s) in mediating this action of CPT-1a has not been identified. In this report, we assess the role of CPT-1a in hypothalamic ventromedial nucleus (VMN). We stereotaxically injected an adenoviral vector containing CPT-1a coding sequence into the VMN...

2017
Marlen Knobloch Gregor-Alexander Pilz Bart Ghesquière Werner J. Kovacs Thomas Wegleiter Darcie L. Moore Martina Hruzova Nicola Zamboni Peter Carmeliet Sebastian Jessberger

Hippocampal neurogenesis is important for certain forms of cognition, and failing neurogenesis has been implicated in neuropsychiatric diseases. The neurogenic capacity of hippocampal neural stem/progenitor cells (NSPCs) depends on a balance between quiescent and proliferative states. Here, we show that the rate of fatty acid oxidation (FAO) regulates the activity of NSPCs. Quiescent NSPCs show...

Journal: :The Journal of biological chemistry 2011
Kwangwon Lee Janos Kerner Charles L Hoppel

CPT1a (carnitine palmitoyltransferase 1a) in the liver mitochondrial outer membrane (MOM) catalyzes the primary regulated step in overall mitochondrial fatty acid oxidation. It has been suggested that the fundamental unit of CPT1a exists as a trimer, which, under native conditions, could form a dimer of the trimers, creating a hexamer channel for acylcarnitine translocation. To examine the stat...

Journal: :The Biochemical journal 1972
J F Chase P K Tubbs

1. The CoA and carnitine esters of 2-bromopalmitate are extremely powerful and specific inhibitors of mitochondrial fatty acid oxidation. 2. 2-Bromopalmitoyl-CoA, added as such or formed from 2-bromopalmitate, inhibits the carnitine-dependent oxidation of palmitate or palmitoyl-CoA, but not the oxidation of palmitoylcarnitine, by intact liver mitochondria. 3. 2-Bromopalmitoylcarnitine inhibits ...

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