HLCS

Gene Name		holocarboxylase synthetase (biotin-(proprionyl-CoA-carboxylase (ATP-hydrolysing)) ligase)
Image		No pdb structure
Gene Ontology Annotations	Cellular Component	Chromatin Nuclear Lamina Cytoplasm Mitochondrion Cytosol Nuclear Matrix
	Molecular Function	Biotin-[acetyl-CoA-carboxylase] Ligase Activity Biotin-[methylcrotonoyl-CoA-carboxylase] Ligase Activity Biotin-[methylmalonyl-CoA-carboxytransferase] Ligase Activity Biotin-[propionyl-CoA-carboxylase (ATP-hydrolyzing)] Ligase Activity Protein Binding ATP Binding Biotin Binding Biotin-protein Ligase Activity Enzyme Binding Protein Homodimerization Activity
	Biological Process	Vitamin Metabolic Process Water-soluble Vitamin Metabolic Process Biotin Metabolic Process Cell Proliferation Protein Biotinylation Histone Modification Small Molecule Metabolic Process Response To Biotin Histone Biotinylation
Pathways		Metabolism of vitamins and cofactors Defective GIF causes intrinsic factor deficiency Defective CD320 causes methylmalonic aciduria Defective LMBRD1 causes methylmalonic aciduria and homocystinuria type cblF Defective HLCS causes multiple carboxylase deficiency Defective MMAB causes methylmalonic aciduria type cblB Defective BTD causes biotidinase deficiency Defective MMACHC causes methylmalonic aciduria and homocystinuria type cblC Defective MTR causes methylmalonic aciduria and homocystinuria type cblG Defective MMADHC causes methylmalonic aciduria and homocystinuria type cblD Defective MMAA causes methylmalonic aciduria type cblA Defective MTRR causes methylmalonic aciduria and homocystinuria type cblE Defective CUBN causes hereditary megaloblastic anemia 1 Defective AMN causes hereditary megaloblastic anemia 1 Defective MUT causes methylmalonic aciduria mut type Metabolism of water-soluble vitamins and cofactors Defects in biotin (Btn) metabolism Defective TCN2 causes hereditary megaloblastic anemia Biotin transport and metabolism Defects in cobalamin (B12) metabolism Defects in vitamin and cofactor metabolism
Drugs		Biotin
Diseases
GWAS
Protein-protein Interactions		4 interactors: ACACB DDX52 POT1 SNX27
Entrez ID		3141
HPRD ID		08360
Ensembl ID		ENSG00000159267
Uniprot IDs		P50747
PDB IDs
Enriched GO Terms of Interacting Partners?		Establishment Of Natural Killer Cell Polarity Positive Regulation Of DNA Strand Elongation Malonyl-CoA Biosynthetic Process Telomere Formation Via Telomerase Positive Regulation Of Helicase Activity Telomere Capping Negative Regulation Of Telomere Maintenance Via Telomerase Positive Regulation Of Telomerase Activity Telomere Assembly Positive Regulation Of Telomere Maintenance Via Telomerase Negative Regulation Of Fatty Acid Oxidation Carnitine Shuttle Negative Regulation Of Telomerase Activity Telomere Maintenance Via Telomerase Negative Regulation Of Telomere Maintenance Retrograde Transport, Endosome To Plasma Membrane Positive Regulation Of Telomere Maintenance Regulation Of Telomere Maintenance Via Telomerase Carnitine Transmembrane Transport Carnitine Transport Biotin Metabolic Process Regulation Of Telomerase Activity Fatty-acyl-CoA Biosynthetic Process Coenzyme A Biosynthetic Process Quaternary Ammonium Group Transport Regulation Of Telomere Maintenance Positive Regulation Of DNA Biosynthetic Process Acetyl-CoA Metabolic Process Negative Regulation Of Fatty Acid Metabolic Process RNA-dependent DNA Replication Negative Regulation Of DNA Biosynthetic Process Coenzyme A Metabolic Process Endosome To Lysosome Transport Regulation Of Fatty Acid Oxidation Telomere Maintenance Via Telomere Lengthening Positive Regulation Of Lipid Storage Positive Regulation Of Heart Growth Long-chain Fatty Acid Transport Ammonium Transmembrane Transport Lysosomal Transport Regulation Of DNA Biosynthetic Process Positive Regulation Of Organ Growth Negative Regulation Of DNA Replication Regulation Of Lipid Storage Regulation Of Heart Growth Natural Killer Cell Activation Fatty Acid Transport Acyl-CoA Metabolic Process Positive Regulation Of DNA Replication Telomere Maintenance
Tagcloud ?		accumulated cholesterol copyright cyclodextrin derived effectively glycolipids hepatocyte hpgcd hpgcds hydroxypropyl impaired ipsc lysosomal niemann npc phenocopy pick pluripotent powerful progenitors prolonged protected reserved restore rights signature storage tools