Definition by Functional and Structural Analysis of Two Malonyl-CoA Sites in Carnitine Palmitoyltransferase 1A
نویسندگان
چکیده
منابع مشابه
Malonyl-CoA and carnitine palmitoyltransferase I: an expanding partnership.
Inhibition of mitochondrial carnitine palmitoyltransferase I (CPT I) by malonyl-CoA, the product of the acetyl-CoA carboxylase reaction, was first recognized in 1977 during the course of studies on hepatic ketogenesis and its regulation [ l ] . What emerged from that work was that with carbohydrate feeding (high insulin/low glucagon) the liver is actively engaged in fatty acid biosynthesis, the...
متن کاملSequencing and functional expression of the malonyl-CoA-sensitive carnitine palmitoyltransferase from Drosophila melanogaster.
Using expressed sequence tag data, we obtained a cDNA for a carnitine palmitoyltransferase I (CPT I)-like molecule from Drosophila melanogaster. The cDNA encodes a 782-residue protein that shows 49% and 48% sequence identity with the rat liver and skeletal-muscle isoforms of CPT I respectively. The sequence has two predicted membrane-spanning regions, suggesting that it adopts the same topology...
متن کاملInteracting effects of L-carnitine and malonyl-CoA on rat liver carnitine palmitoyltransferase.
Malonyl-CoA significantly increased the Km for L-carnitine of overt carnitine palmitoyltransferase in liver mitochondria from fed rats. This effect was observed when the molar palmitoyl-CoA/albumin concentration ratio was low (0.125-1.0), but not when it was higher (2.0). In the absence of malonyl-CoA, the Km for L-carnitine increased with increasing palmitoyl-CoA/albumin ratios. Malonyl-CoA di...
متن کاملAltered sensitivity of carnitine palmitoyltransferase to inhibition by malonyl-CoA in ketotic diabetic rats.
Carnitine palmitoyltransferase of liver mitochondria prepared from ketotic diabetic rats has a diminished sensitivity to inhibition by malonyl-CoA compared with carnitine palmitoyltransferase of mitochondria prepared from normal fed rats.
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 کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
ژورنال
عنوان ژورنال: Journal of Biological Chemistry
سال: 2007
ISSN: 0021-9258
DOI: 10.1074/jbc.m700885200