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3-hydroxy-3-methylglutaryl-CoA synthase 2

Hydroxymethylglutaryl-CoA Synthase, HMG-CoA synthase, 3-hydroxy-3-methylglutaryl coenzyme A synthase
The protein encoded by this gene belongs to the HMG-CoA synthase family. It is a mitochondrial enzyme that catalyzes the first reaction of ketogenesis, a metabolic pathway that provides lipid-derived energy for various organs during times of carbohydrate deprivation, such as fasting. Mutations in this gene are associated with HMG-CoA synthase deficiency. Alternatively spliced transcript variants encoding different isoforms have been found for this gene.[provided by RefSeq, Oct 2009] (from NCBI)
Top mentioned proteins: ACID, CAN, HAD, fibrillin-1, STEP
Papers on Hydroxymethylglutaryl-CoA Synthase
mTOR inhibition activates overall protein degradation by the ubiquitin proteasome system as well as by autophagy.
Goldberg et al., Boston, United States. In Proc Natl Acad Sci U S A, Jan 2016
Several growth-related proteins were identified that were ubiquitinated and degraded more rapidly after mTOR inhibition, including HMG-CoA synthase, whose enhanced degradation probably limits cholesterol biosynthesis upon insulin deficiency.
Spatial and temporal regulation of sterol biosynthesis in Nicotiana benthamiana.
Chappell et al., Ames, United States. In Physiol Plant, Jan 2016
Consistent with the precursor incorporation data, analysis of gene transcript and measurements of putative rate-limiting enzyme activities for HMG-CoA synthase (EC and reductase (EC
A novel MVA-mediated pathway for isoprene production in engineered E. coli.
Liu et al., Qingdao, China. In Bmc Biotechnol, Dec 2015
A novel biosynthetic pathway of isoprene in E. coli was established by co-expressing the heterologous mvaE gene encoding acetyl-CoA acetyltransferase/HMG-CoA reductase and mvaS gene encoding HMG-CoA synthase from Enterococcus faecalis, fatty acid decarboxylase (OleTJE) and oleate hydratase (OhyAEM).
Mammalian autophagy is essential for hepatic and renal ketogenesis during starvation.
Uzu et al., Ōtsu, Japan. In Sci Rep, Dec 2015
Interestingly, renal as well as hepatic expression of HMG-CoA synthase 2 increased with prolonged starvation.
Absence of keratin 8 or 18 promotes antimitochondrial autoantibody formation in aging male mice.
Omary et al., Turku, Finland. In Faseb J, Dec 2015
Mass spectrometry and purified protein analysis identified, mitochondrial HMG-CoA synthase, aldehyde dehydrogenase, and catalase as the primary autoantigens, and glutamate dehydrogenase and epoxide hydrolase-2 as additional autoantigens.
Implications of a peroxisome proliferator-activated receptor alpha (PPARα) ligand clofibrate in breast cancer.
Sharma-Walia et al., North Chicago, United States. In Oncotarget, Dec 2015
Surprisingly, the expression of lipogenic pathway genes including SREBP-1c (sterol regulatory element-binding protein-1c), HMG-CoA synthase, SPTLC1 (serine palmitoyltransferase long-chain), and Acyl-CoA oxidase (ACO) decreased with a concurrent increase in fatty acid oxidation genes such as CPT-1a (carnitine palmitoyltransferase 1a) and SREBP-2 (Sterol regulatory element-binding protein-2).
De novo assembly and comparative analysis of root transcriptomes from different varieties of Panax ginseng C. A. Meyer grown in different environments.
He et al., Beijing, China. In Sci China Life Sci, Nov 2015
In particular, some key ginsenoside biosynthesis-related genes, including HMG-CoA synthase (HMGS), mevalonate kinase (MVK), and squalene epoxidase (SE), were upregulated in wild-grown ginseng.
Genome-wide RNAi analysis reveals that simultaneous inhibition of specific mevalonate pathway genes potentiates tumor cell death.
Penn et al., Toronto, Canada. In Oncotarget, Oct 2015
Genes of the MVA pathway were amongst the top-scoring targets, including sterol regulatory element binding transcription factor 2 (SREBP2), 3-hydroxy-3-methylglutaryl-coenzyme A synthase 1 (HMGCS1) and geranylgeranyl diphosphate synthase 1 (GGPS1).
Proteomic analysis of conidia germination in Fusarium oxysporum f. sp. cubense tropical race 4 reveals new targets in ergosterol biosynthesis pathway for controlling Fusarium wilt of banana.
Yi et al., Guangzhou, China. In Appl Microbiol Biotechnol, Sep 2015
Four enzymes, C-24 sterol methyltransferase (ERG6), cytochrome P450 lanosterol C-14α-demethylase (EGR11), hydroxymethylglutaryl-CoA synthase (ERG13), and C-4 sterol methyl oxidase (ERG25), in the ergosterol biosynthesis pathway were identified and verified, and they hold great promise as new targets for effective inhibition of Foc TR4 early growth in controlling Fusarium wilt of banana.
The phenotype of a knockout mouse identifies flavin-containing monooxygenase 5 (FMO5) as a regulator of metabolic ageing.
Shephard et al., London, United Kingdom. In Biochem Pharmacol, Sep 2015
Five proteins were down regulated in the liver of Fmo5(-/-) mice: aldolase B, ketohexokinase and cytosolic glycerol 3-phosphate dehydrogenase (GPD1) are involved in glucose or fructose metabolism and GPD1 also in production of glycerol 3-phosphate, a precursor of triglyceride biosynthesis; HMG-CoA synthase 1 is involved in cholesterol biosynthesis; and malic enzyme 1 catalyzes the oxidative decarboxylation of malate to pyruvate, in the process producing NADPH for use in lipid and cholesterol biosynthesis.
Ketone body metabolism and its defects.
Aoyama et al., Gifu, Japan. In J Inherit Metab Dis, 2014
Defects in ketogenesis include mitochondrial HMG-CoA synthase (mHS) deficiency and HMG-CoA lyase (HL) deficiency.
Characterization of splice variants of the genes encoding human mitochondrial HMG-CoA lyase and HMG-CoA synthase, the main enzymes of the ketogenesis pathway.
Pié et al., Zaragoza, Spain. In Mol Biol Rep, 2012
An alternative transcript of HMGCS2 carrying a deletion of exon 4, and two alternative transcripts of HMGCL with deletions of exons 5 and 6, and exons 5, 6 and 7, respectively, were detected.
Human HMGCS2 regulates mitochondrial fatty acid oxidation and FGF21 expression in HepG2 cell line.
Marrero et al., Barcelona, Spain. In J Biol Chem, 2011
Human HMGCS2 regulates mitochondrial fatty acid oxidation and FGF21 expression
Mupirocin: biosynthesis, special features and applications of an antibiotic from a gram-negative bacterium.
Thomas et al., Birmingham, United Kingdom. In Appl Microbiol Biotechnol, 2011
Special features of this group are in-cis methyltransferase domains and a trans-acting HMG-CoA synthase-cassette which insert α- and β- methyl groups respectively while enoyl reductase domains are absent from the condensing modules.
Enzymes of the mevalonate pathway of isoprenoid biosynthesis.
Miziorko, Kansas City, United States. In Arch Biochem Biophys, 2011
The biosynthetic acetoacetyl-CoA thiolase and HMG-CoA synthase reactions rely on key amino acids that are different but are situated in active sites that are similar throughout the family of initial condensation enzymes.
Crystal structures of human HMG-CoA synthase isoforms provide insights into inherited ketogenesis disorders and inhibitor design.
Yue et al., Oxford, United Kingdom. In J Mol Biol, 2010
The authors report high-resolution crystal structures of the human cytosolic (hHMGCS1) and mitochondrial (hHMGCS2) isoforms in binary product complexes.
Ketogenic HMGCS2 Is a c-Myc target gene expressed in differentiated cells of human colonic epithelium and down-regulated in colon cancer.
Marrero et al., Barcelona, Spain. In Mol Cancer Res, 2006
Ketogenesis is an undesirable metabolic characteristic of the proliferating cell, which is down-regulated through c-Myc-mediated repression of the key metabolic gene HMGCS2.
Studies on biosynthetic genes and enzymes of isoprenoids produced by actinomycetes.
Dairi, Kosugi, Japan. In J Antibiot (tokyo), 2005
All these clusters contained genes coding for mevalonate kinase, mevalonate diphosphate decarboxylase, phosphomevalonate kinase, type 2 IPP isomerase, HMG-CoA reductase, and HMG-CoA synthase.
New insights in the transcriptional activity and coregulator molecules in the arterial wall.
Napoli et al., Napoli, Italy. In Int J Cardiol, 2002
For example, statins modulate activation of the class-I transcription factor sterol responsive element-binding protein (SREBP), whose target genes including hydroxyl-methyl-glutaryl acetyl Coenzyme-A (HMG-CoA) reductase, HMG-CoA synthase, and the low-density lipoprotein receptor, all of which are involved in cholesterol and fatty acid metabolism.
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