Functional and structural basis of carnitine palmitoyltransferase 1A deficiency. (Q50335986)

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19 December 2019

title

Functional and structural basis of carnitine palmitoyltransferase 1A deficiency (English)

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19 December 2019

5

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19 December 2019

Jean-Paul Bonnefont

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19 December 2019

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Carina Prip-Buus

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19 December 2019

author name string

Stéphanie Gobin

1

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19 December 2019

Laure Thuillier

2

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19 December 2019

Gerwald Jogl

3

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19 December 2019

Audrey Faye

4

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19 December 2019

Mihaiti Chi

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19 December 2019

Jean Girard

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19 December 2019

language of work or name

English

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publication date

29 September 2003

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Journal of Biological Chemistry

19 December 2019

published in

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19 December 2019

volume

278

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19 December 2019

issue

50

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19 December 2019

page(s)

50428-50434

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The mitochondrial carnitine palmitoyltransferase system. From concept to molecular analysis

19 December 2019

cites work

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Molecular enzymology of carnitine transfer and transport

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Are the beta-cell signaling molecules malonyl-CoA and cystolic long-chain acyl-CoA implicated in multiple tissue defects of obesity and NIDDM?

21 January 2018

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The malonyl-CoA-long-chain acyl-CoA axis in the maintenance of mammalian cell function

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Diseases of liporegulation: new perspective on obesity and related disorders

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Banting lecture 2001: dysregulation of fatty acid metabolism in the etiology of type 2 diabetes

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Human liver mitochondrial carnitine palmitoyltransferase I: characterization of its cDNA and chromosomal localization and partial analysis of the gene

21 January 2018

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Isolation and characterization of cDNA and genomic clones encoding human muscle type carnitine palmitoyltransferase I.

21 January 2018

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A novel brain-expressed protein related to carnitine palmitoyltransferase I

21 January 2018

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The N-terminal domain of rat liver carnitine palmitoyltransferase 1 contains an internal mitochondrial import signal and residues essential for folding of its C-terminal catalytic domain

21 January 2018

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Molecular basis of hepatic carnitine palmitoyltransferase I deficiency

21 January 2018

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Structural model of the catalytic core of carnitine palmitoyltransferase I and carnitine octanoyltransferase (COT): mutation of CPT I histidine 473 and alanine 381 and COT alanine 238 impairs the catalytic activity

21 January 2018

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Structural model of a malonyl-CoA-binding site of carnitine octanoyltransferase and carnitine palmitoyltransferase I: mutational analysis of a malonyl-CoA affinity domain

21 January 2018

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Identification of conserved amino acid residues in rat liver carnitine palmitoyltransferase I critical for malonyl-CoA inhibition. Mutation of methionine 593 abolishes malonyl-CoA inhibition

21 January 2018

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The extreme C terminus of rat liver carnitine palmitoyltransferase I is not involved in malonyl-CoA sensitivity but in initial protein folding

21 January 2018

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Identification by mutagenesis of conserved arginine and glutamate residues in the C-terminal domain of rat liver carnitine palmitoyltransferase I that are important for catalytic activity and malonyl-CoA sensitivity

21 January 2018

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Crystal structure of carnitine acetyltransferase and implications for the catalytic mechanism and fatty acid transport

21 January 2018

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Structure of human carnitine acetyltransferase. Molecular basis for fatty acyl transfer

21 January 2018

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Carnitine palmitoyltransferase deficiencies

21 January 2018

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Molecular and enzymatic characterization of a unique carnitine palmitoyltransferase 1A mutation in the Hutterite community

21 January 2018

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Organization of the human liver carnitine palmitoyltransferase 1 gene ( CPT1A) and identification of novel mutations in hypoketotic hypoglycaemia

21 January 2018

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Expression analysis of two mutations in carnitine palmitoyltransferase IA deficiency

21 January 2018

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Characterisation of carnitine palmitoyltransferases in patients with a carnitine palmitoyltransferase deficiency: implications for diagnosis and therapy

21 January 2018

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Oxidation of fatty acids in cultured fibroblasts: a model system for the detection and study of defects in oxidation

21 January 2018

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Topological and functional analysis of the rat liver carnitine palmitoyltransferase 1 expressed in Saccharomyces cerevisiae

21 January 2018

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Cleavage of Structural Proteins during the Assembly of the Head of Bacteriophage T4

21 January 2018

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Comparative protein modelling by satisfaction of spatial restraints

21 January 2018

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Topology of carnitine palmitoyltransferase I in the mitochondrial outer membrane

21 January 2018

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The N-terminal domain of rat liver carnitine palmitoyltransferase 1 mediates import into the outer mitochondrial membrane and is essential for activity and malonyl-CoA sensitivity

21 January 2018

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A single amino acid change (substitution of glutamate 3 with alanine) in the N-terminal region of rat liver carnitine palmitoyltransferase I abolishes malonyl-CoA inhibition and high affinity binding

21 January 2018

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Conformations of the nucleotide and polypeptide binding domains of a cytosolic Hsp70 molecular chaperone are coupled

21 January 2018

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Use of six chimeric proteins to investigate the role of intramolecular interactions in determining the kinetics of carnitine palmitoyltransferase I isoforms

21 January 2018

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21 January 2018

Identifiers

DOI

10.1074/JBC.M310130200

1 reference

https://www.ebi.ac.uk/europepmc/webservices/rest/search?query=EXT_ID:14517221%20AND%20SRC:MED&resulttype=core&format=json

19 December 2019

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