Research Topics
Genomes and Genes
| Mary C HuntSummaryAffiliation: Karolinska Institutet Country: Sweden Publications
| Collaborators
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Detail Information
Publications
A revised nomenclature for mammalian acyl-CoA thioesterases/hydrolasesMary C Hunt
Karolinska Institutet, Department of Laboratory Medicine, Division of Clinical Chemistry C1 74, Karolinska University Hospital at Huddinge, Stockholm, Sweden
J Lipid Res 46:2029-32. 2005..The family root symbol is ACOT, with human genes named ACOT1-ACOT12, and rat and mouse genes named Acot1-Acot12. Several of the ACOT genes are the result of splicing events, and these splice variants are cataloged...- Identification of fatty acid oxidation disorder patients with lowered acyl-CoA thioesterase activity in human skin fibroblastsM C Hunt
Karolinska University Hospital at Huddinge, Stockholm, Sweden
Eur J Clin Invest 35:38-46. 2005..However, to date no patients deficient in acyl-CoA thioesterases have been identified... - The identification of a succinyl-CoA thioesterase suggests a novel pathway for succinate production in peroxisomesMaria A K Westin
Department of Laboratory Medicine, Division of Clinical Chemistry, C1 74, Karolinska University Hospital at Huddinge, SE 141 86 Stockholm, Sweden
J Biol Chem 280:38125-32. 2005.... - Molecular cloning and characterization of two mouse peroxisome proliferator-activated receptor alpha (PPARalpha)-regulated peroxisomal acyl-CoA thioesterasesMaria A K Westin
Department of Laboratory Medicine, Karolinska Institutet, C1 74, Karolinska University Hospital at Huddinge, SE 141 86 Stockholm, Sweden
J Biol Chem 279:21841-8. 2004..These data show that PTE-Ia and PTE-Ic have different functions based on different substrate specificities and tissue expression... - Analysis of the mouse and human acyl-CoA thioesterase (ACOT) gene clusters shows that convergent, functional evolution results in a reduced number of human peroxisomal ACOTsMary C Hunt
Karolinska Institutet, Department of Laboratory Medicine, Division of Clinical Chemistry C1 74, Karolinska University Hospital at Huddinge, Stockhold SE 141 86, Sweden
FASEB J 20:1855-64. 2006..These data strongly suggest that the human ACOT4 gene has acquired the functions of three mouse genes by a functional convergent evolution that also provides an explanation for the unexpectedly low number of human genes... - Differential regulation of cytosolic and peroxisomal bile acid amidation by PPAR alpha activation favors the formation of unconjugated bile acidsKarianne Solaas
Division of Clinical Chemistry, Karolinska Institutet, Karolinska University Hospital at Huddinge, SE 141 86 Stockholm, Sweden
J Lipid Res 45:1051-60. 2004..These effects caused by WY-14,643 treatment were abolished in PPARalpha-null mice. The results from this study suggest that an increased formation of unconjugated bile acids occurs during PPARalpha activation... - A peroxisomal acyltransferase in mouse identifies a novel pathway for taurine conjugation of fatty acidsSarah Jayne Reilly
Karolinska Institutet, Department of Laboratory Medicine, Division of Clinical Chemistry C1 74, Karolinska University Hospital at Huddinge, SE 141 86 Stockholm, Sweden
FASEB J 21:99-107. 2007.... - Peroxisomes contain a specific phytanoyl-CoA/pristanoyl-CoA thioesterase acting as a novel auxiliary enzyme in alpha- and beta-oxidation of methyl-branched fatty acids in mouseMaria A K Westin
Department of Laboratory Medicine, Division of Clinical Chemistry, C1 74, Karolinska University Hospital at Huddinge, Karolinska Institutet, SE 141 86 Stockholm, Sweden
J Biol Chem 282:26707-16. 2007..Furthermore, Acot6 was identified as a target gene of the peroxisome proliferator-activated receptor alpha (PPARalpha) and is up-regulated in mouse liver in a PPARalpha-dependent manner... - The nudix hydrolase 7 is an Acyl-CoA diphosphatase involved in regulating peroxisomal coenzyme A homeostasisSarah Jayne Reilly
Division of Clinical Chemistry C1 74, Department of Laboratory Medicine, Karolinska Institutet, Karolinska University Hospital at Huddinge, SE 141 86 Stockholm, Sweden
J Biochem 144:655-63. 2008..Taken together these data suggest that NUDT7alpha function is tightly linked to peroxisomal CoASH/acyl-CoA homeostasis... - The role Acyl-CoA thioesterases play in mediating intracellular lipid metabolismMary C Hunt
Department of Medical Laboratory Sciences and Technology, Division of Clinical Chemistry, Karolinska Institutet, Huddinge University Hospital, S 141 86, Stockholm, Sweden
Prog Lipid Res 41:99-130. 2002.... - The acyl-CoA thioesterase I is regulated by PPARalpha and HNF4alpha via a distal response element in the promoterBikesh Dongol
Karolinska Institutet, Department of Laboratory Medicine, Division of Clinical Chemistry, Karolinska University Hospital at Huddinge, Stockholm, Sweden
J Lipid Res 48:1781-91. 2007..However, transfection with a plasmid containing HNF4alpha also resulted in an increase in promoter activity. Together, these data show that Acot1 is under regulation by an interplay between HNF4alpha and PPARalpha... - The human bile acid-CoA:amino acid N-acyltransferase functions in the conjugation of fatty acids to glycineJames O'Byrne
Department of Laboratory Medicine, Division of Clinical Chemistry, Karolinska Institutet, Huddinge University Hospital, SE 141 86 Stockholm, Sweden
J Biol Chem 278:34237-44. 2003..Therefore, the cytosolic BACAT enzyme may play important roles in protection against toxicity by accumulation of unconjugated bile acids and non-esterified very long-chain fatty acids... - Localization of peroxisomal matrix proteins by photobleachingCharlotta Buch
Department of Biosciences and Nutrition, Karolinska Institutet, SE 141 57 Huddinge, Sweden
Biochem Biophys Res Commun 388:355-9. 2009..We conclude that the cytosolic localization was due to its carboxyterminal non-consensus peroxisomal targeting signal (-SQL) since mutation of the -SQL to -SKL resulted in BAAT being efficiently imported into peroxisomes... - PPARalpha is a key regulator of hepatic FGF21Thomas Lundåsen
Center for Endocrinology, Metabolism, and Diabetes, Department of Medicine, M63, Karolinska Institutet, Karolinska University Hospital, Huddinge, SE 141 86 Stockholm, Sweden
Biochem Biophys Res Commun 360:437-40. 2007..Further studies on the mechanisms of regulation of FGF21 by PPARalpha in humans will be of great interest... - Identification of glycine N-acyltransferase-like 2 (GLYATL2) as a transferase that produces N-acyl glycines in humansDominik P Waluk
Stockholm University, Department of Genetics, Microbiology and Toxicology, Stockholm, Sweden
FASEB J 24:2795-803. 2010..The expression pattern and the identification of high levels of N-acyl glycines in skin and lung may indicate a role for N-acyl glycines in barrier function/immune response and the potential role of hGLYATL2 in this regard is discussed... - Characterization of an acyl-coA thioesterase that functions as a major regulator of peroxisomal lipid metabolismMary C Hunt
Department of Medical Laboratory Sciences and Technology, Division of Clinical Chemistry, Karolinska Institutet, Huddinge University Hospital, SE 141 86 Stockholm, Sweden
J Biol Chem 277:1128-38. 2002..We propose that PTE-2 functions as a key regulator of peroxisomal lipid metabolism... - Short- and medium-chain carnitine acyltransferases and acyl-CoA thioesterases in mouse provide complementary systems for transport of beta-oxidation products out of peroxisomesM A K Westin
Karolinska Institutet, Department of Laboratory Medicine, Division of Clinical Chemistry, C1 74, Karolinska University Hospital at Huddinge, Stockholm, Sweden
Cell Mol Life Sci 65:982-90. 2008..These data also explain earlier observed tissue differences in peroxisomal production of acetyl-CoA/acetyl-carnitine/acetate and underscores the differences in peroxisome function in various organs... - Alternative exon usage selectively determines both tissue distribution and subcellular localization of the acyl-CoA thioesterase 7 gene productsM C Hunt
Department of Laboratory Medicine, Division of Clinical Chemistry C1 74, Karolinska Institutet, Karolinska University Hospital at Huddinge, Stockholm, Sweden
Cell Mol Life Sci 64:1558-70. 2007..Taken together, these data show that the Acot7 gene is expressed as multiple isoforms in a tissue-specific manner, and that expression in tissues other than brain and testis is likely to play important roles in fatty acid metabolism...