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Holocarboxylase synthetase

holocarboxylase synthetase, HLCS
This gene encodes an enzyme that catalyzes the binding of biotin to carboxylases and histones. The protein plays an important role in gluconeogenesis, fatty acid synthesis and branched chain amino acid catabolism. Defects in this gene are the cause of holocarboxylase synthetase deficiency. Multiple alternatively spliced variants, encoding the same protein, have been identified.[provided by RefSeq, Jun 2011] (from NCBI)
Top mentioned proteins: ACID, biotinidase, CAN, Histone, HAD
Papers on holocarboxylase synthetase
Antenatal and postnatal radiologic diagnosis of holocarboxylase synthetase deficiency: a systematic review.
Kothari et al., Gold Coast, Australia. In Pediatr Radiol, Feb 2016
BACKGROUND: Holocarboxylase synthetase deficiency results in impaired activation of enzymes implicated in glucose, fatty acid and amino acid metabolism.
Regulation of immunological and inflammatory functions by biotin.
Kuroishi, Sendai, Japan. In Can J Physiol Pharmacol, Dec 2015
Holocarboxylase synthetase (HLCS) catalyzes the biotinylation of carboxylases and other proteins, whereas biotinidase catalyzes the release of biotin from biotinylated peptides.
Biotin-dependent functions in adiposity: a study of monozygotic twin pairs.
Pietiläinen et al., Helsinki, Finland. In Int J Obes (lond), Dec 2015
In AT, HLCS and ACACB were hypermethylated and biotin cycle genes HLCS and BTD were downregulated (P<0.05).
Resveratrol compounds inhibit human holocarboxylase synthetase and cause a lean phenotype in Drosophila melanogaster.
Zempleni et al., Lincoln, United States. In J Nutr Biochem, Nov 2015
Holocarboxylase synthetase (HLCS) is the sole protein-biotin ligase in the human proteome.
Integration of Genome-Wide SNP Data and Gene-Expression Profiles Reveals Six Novel Loci and Regulatory Mechanisms for Amino Acids and Acylcarnitines in Whole Blood.
Scholz et al., Leipzig, Germany. In Plos Genet, Sep 2015
Based on an integrative analysis of expression quantitative trait loci in blood mononuclear cells and correlations between gene expressions and metabolite levels, we provide evidence for putative causative genes: SLC22A16 for total acylcarnitines, ARG1 for arginine, HLCS for 2-hydroxyisovalerylcarnitine, JAM3 for stearoylcarnitine via a trans-effect at chromosome 1, and PPP1R16A for aspartic acid traits.
β-Keto and β-hydroxyphosphonate analogs of biotin-5'-AMP are inhibitors of holocarboxylase synthetase.
Dussault et al., Lincoln, United States. In Bioorg Med Chem Lett, 2015
Holocarboxylase synthetase (HLCS) catalyzes the covalent attachment of biotin to cytoplasmic and mitochondrial carboxylases, nuclear histones, and over a hundred human proteins.
Epigenetic regulation of traf2- and Nck-interacting kinase (TNIK) in polycystic ovary syndrome.
Wang et al., Shenyang, China. In Am J Transl Res, 2014
Mechanistically, hypermethylated cg10180092 site-mediated loss of holocarboxylase synthetase (HLCS)-related H3K9me enrichment activated TNIK transcription in PCOS ovarian tissues.
Severe neonatal holocarboxylase synthetase deficiency in west african siblings.
Kirmse et al., Bethesda, United States. In Jimd Rep, 2014
In multiple carboxylase deficiency (MCD), the biotin-dependent carboxylases have decreased activity due to either biotinidase deficiency or holocarboxylase synthetase (HS) deficiency.
[Application of methylation-specific multiplex ligation-dependent probe amplification for the study of DNA methylation in placenta tissues].
Lin et al., Guangzhou, China. In Zhonghua Yi Xue Yi Chuan Xue Za Zhi, 2014
METHODS: For blood cells from 13 non-pregnant women and 9 euploid placenta, the ratios of DNA methylation were evaluated for 4 genes including CGI149, CGI113, HLCS and ACTB with MS-MLPA and bisulfite sequencing, respectively.
Novel roles of holocarboxylase synthetase in gene regulation and intermediary metabolism.
Cordonier et al., Lincoln, United States. In Nutr Rev, 2014
The role of holocarboxylase synthetase (HLCS) in catalyzing the covalent binding of biotin to the five biotin-dependent carboxylases in humans is well established, as are the essential roles of these carboxylases in the metabolism of fatty acids, the catabolism of leucine, and gluconeogenesis.
Vitamin-responsive disorders: cobalamin, folate, biotin, vitamins B1 and E.
Baumgartner, Zürich, Switzerland. In Handb Clin Neurol, 2012
Two inherited defects in biotin metabolism are known, holocarboxylase synthetase and biotinidase deficiency.
Biotin requirements for DNA damage prevention.
Baier et al., Lincoln, United States. In Mutat Res, 2012
Biotinylation of both carboxylases and histones is catalyzed by holocarboxylase synthetase.
Effects of single-nucleotide polymorphisms in the human holocarboxylase synthetase gene on enzyme catalysis.
Zempleni et al., Lincoln, United States. In Eur J Hum Genet, 2012
individuals with HLCS SNPs may benefit from supplemental biotin, yet to different extents depending on the genotype
Human holocarboxylase synthetase with a start site at methionine-58 is the predominant nuclear variant of this protein and has catalytic activity.
Zempleni et al., Lincoln, United States. In Biochem Biophys Res Commun, 2011
HLCS methionine-58 is a functional translation start site in human cells.
Holocarboxylase synthetase is a chromatin protein and interacts directly with histone H3 to mediate biotinylation of K9 and K18.
Zempleni et al., Lincoln, United States. In J Nutr Biochem, 2011
Holocarboxylase synthetase interacts directly with histone H3, causing biotinylation of lysine K9 and K18.
Biotin regulates the expression of holocarboxylase synthetase in the miR-539 pathway in HEK-293 cells.
Zempleni et al., Lincoln, United States. In J Nutr, 2010
The results of this study suggest that miR-539 is among the factors sensing biotin and regulating holocarboxylase synthetase expression.
Holocarboxylase synthetase: correlation of protein localisation with biological function.
Polyak et al., Adelaide, Australia. In Arch Biochem Biophys, 2010
the localisation of HCS and its isoforms
Hassan et al., Lincoln, United States. In Biofactors, 2009
Proteins such as holocarboxylase synthetase, biotinidase, and the biotin transporters SMVT and MCT1 play crucial roles in biotin homeostasis, and these roles are reviewed here.
A novel, enigmatic histone modification: biotinylation of histones by holocarboxylase synthetase.
Zempleni et al., Lincoln, United States. In Nutr Rev, 2008
Holocarboxylase synthetase catalyzes the covalent binding of biotin to histones in humans and other eukaryotes.
Isolation and characterization of mutations in the human holocarboxylase synthetase cDNA.
Narisawa et al., Sendai, Japan. In Nat Genet, 1994
Holocarboxylase synthetase (HCS) plays an essential role in biotin utilization in eukaryotic cells and its deficiency causes biotin-responsive multiple carboxylase deficiency in humans.
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