title: A novel brain-expressed protein related to carnitine palmitoyltransferase I.
abstract: Malonyl-CoenzymeA acts as a fuel sensor, being both an intermediate of fatty acid synthesis and an inhibitor of the two known isoforms of carnitine palmitoyltransferase I (CPT I), which control mitochondrial fatty acid oxidation. We describe here a novel CPT1 family member whose mRNA is present predominantly in brain and testis. Chromosomal locations and genome organization are reported for the mouse and human genes. The protein sequence contains all the residues known to be important for both carnitine acyltransferase activity and malonyl-CoA binding in other family members. Yeast expressed protein has no detectable catalytic activity with several different acyl-CoA esters that are good substrates for other carnitine acyltransferases, including the liver isoform of CPT I, which is also expressed in brain; however, it displays high-affinity malonyl-CoA binding. Thus this new CPT I related protein may be specialized for the metabolism of a distinct class of fatty acids involved in brain function.
authors.affiliation: Hannah Research Institute, Ayr, KA6 5HL, UK. pricen@hri.sari.ac.uk
authors.forename: Nigel
authors.initials: N
authors.lastname: Price
authors.forename: Feike
authors.initials: F
authors.lastname: van der Leij
authors.forename: Vicky
authors.initials: V
authors.lastname: Jackson
authors.forename: Clark
authors.initials: C
authors.lastname: Corstorphine
authors.forename: Ross
authors.initials: R
authors.lastname: Thomson
authors.forename: Annette
authors.initials: A
authors.lastname: Sorensen
authors.forename: Victor
authors.initials: V
authors.lastname: Zammit
mesh_terms: D000595:Amino Acid Sequence; D000818:Animals; D001921:Brain / Q000276:immunology / Q000378:metabolism*; D002334:Carnitine O-Palmitoyltransferase / Q000096:biosynthesis / Q000235:genetics* / Q000276:immunology / Q000378:metabolism; D002874:Chromosome Mapping; D017124:Conserved Sequence; D018076:DNA, Complementary; D005786:Gene Expression Regulation; D006801:Humans; D007438:Introns; D051379:Mice; D008969:Molecular Sequence Data; D009928:Organ Specificity; D010802:Phylogeny; D016415:Sequence Alignment
chemical_list: D018076:DNA, Complementary; D002334:Carnitine O-Palmitoyltransferase