Functional and structural basis of carnitine palmitoyltransferase 1A deficiency.

scientific article published on 29 September 2003

Functional and structural basis of carnitine palmitoyltransferase 1A deficiency. is …
instance of (P31):
scholarly articleQ13442814

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P356DOI10.1074/JBC.M310130200
P698PubMed publication ID14517221

P50authorLiang TongQ41845659
Jean-Paul BonnefontQ56241280
Carina Prip-BuusQ88092873
P2093author name stringGerwald Jogl
Jean Girard
Laure Thuillier
Stéphanie Gobin
Audrey Faye
Mihaiti Chi
P2860cites workMolecular and enzymatic characterization of a unique carnitine palmitoyltransferase 1A mutation in the Hutterite communityQ24291222
Human liver mitochondrial carnitine palmitoyltransferase I: characterization of its cDNA and chromosomal localization and partial analysis of the geneQ24317500
Cleavage of Structural Proteins during the Assembly of the Head of Bacteriophage T4Q25938983
Crystal structure of carnitine acetyltransferase and implications for the catalytic mechanism and fatty acid transportQ27640305
Structure of human carnitine acetyltransferase. Molecular basis for fatty acyl transferQ27640412
Comparative protein modelling by satisfaction of spatial restraintsQ27860866
Carnitine palmitoyltransferase deficienciesQ28140940
A novel brain-expressed protein related to carnitine palmitoyltransferase IQ28206878
Expression analysis of two mutations in carnitine palmitoyltransferase IA deficiencyQ28214222
Structural model of a malonyl-CoA-binding site of carnitine octanoyltransferase and carnitine palmitoyltransferase I: mutational analysis of a malonyl-CoA affinity domainQ28564300
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 domainQ28567275
The extreme C terminus of rat liver carnitine palmitoyltransferase I is not involved in malonyl-CoA sensitivity but in initial protein foldingQ28577659
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 sensitivityQ28578477
Identification of conserved amino acid residues in rat liver carnitine palmitoyltransferase I critical for malonyl-CoA inhibition. Mutation of methionine 593 abolishes malonyl-CoA inhibitionQ28579126
Topology of carnitine palmitoyltransferase I in the mitochondrial outer membraneQ28579497
The mitochondrial carnitine palmitoyltransferase system. From concept to molecular analysisQ29618429
The malonyl-CoA-long-chain acyl-CoA axis in the maintenance of mammalian cell functionQ33755073
Molecular enzymology of carnitine transfer and transportQ33938860
Diseases of liporegulation: new perspective on obesity and related disordersQ34130773
Molecular basis of hepatic carnitine palmitoyltransferase I deficiencyQ37384234
Organization of the human liver carnitine palmitoyltransferase 1 gene ( CPT1A) and identification of novel mutations in hypoketotic hypoglycaemiaQ38363595
Are the beta-cell signaling molecules malonyl-CoA and cystolic long-chain acyl-CoA implicated in multiple tissue defects of obesity and NIDDM?Q40965216
Characterisation of carnitine palmitoyltransferases in patients with a carnitine palmitoyltransferase deficiency: implications for diagnosis and therapyQ41356691
Oxidation of fatty acids in cultured fibroblasts: a model system for the detection and study of defects in oxidationQ41626822
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 activityQ43735145
Banting lecture 2001: dysregulation of fatty acid metabolism in the etiology of type 2 diabetesQ43833528
Topological and functional analysis of the rat liver carnitine palmitoyltransferase 1 expressed in Saccharomyces cerevisiaeQ47895898
Isolation and characterization of cDNA and genomic clones encoding human muscle type carnitine palmitoyltransferase I.Q48063300
Conformations of the nucleotide and polypeptide binding domains of a cytosolic Hsp70 molecular chaperone are coupledQ71246498
Use of six chimeric proteins to investigate the role of intramolecular interactions in determining the kinetics of carnitine palmitoyltransferase I isoformsQ73680421
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 bindingQ74638102
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 sensitivityQ77478142
P433issue50
P407language of work or nameEnglishQ1860
P304page(s)50428-50434
P577publication date2003-09-29
P1433published inJournal of Biological ChemistryQ867727
P1476titleFunctional and structural basis of carnitine palmitoyltransferase 1A deficiency
P478volume278

Reverse relations

cites work (P2860)
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