Holocarboxylase synthetase

Holocarboxylase synthetase
Identifiers
AliasesBiotin-(propionyl-CoA-carboxylase (ATP-hydrolyzing)) ligasebiotin-propionyl-CoA-carboxylase (ATP-hydrolysing) ligasebiotin-[propionyl-CoA-carboxylase (ATP-hydrolyzing)] synthetasebiotin:apo-propanoyl-CoA:carbon-dioxide ligase (ADP-forming) ligase (AMP-forming)biotin-propionyl-CoA-carboxylase (ATP-hydrolysing) synthetasepropionyl coenzyme A holocarboxylase synthetasepropionyl-CoA holocarboxylase synthetasebiotin-propionyl coenzyme A carboxylase synthetase
External IDsGeneCards: [1]; OMA:- orthologs
Orthologs
SpeciesHumanMouse
Entrez
Ensembl
UniProt
RefSeq (mRNA)

n/a

n/a

RefSeq (protein)

n/a

n/a

Location (UCSC)n/an/a
PubMed searchn/an/a
Wikidata
View/Edit Human
Biotin—[propionyl-CoA-carboxylase (ATP-hydrolyzing)] ligase
Identifiers
EC no.6.3.4.10
CAS no.37318-67-5
Databases
IntEnzIntEnz view
BRENDABRENDA entry
ExPASyNiceZyme view
KEGGKEGG entry
MetaCycmetabolic pathway
PRIAMprofile
PDB structuresRCSB PDB PDBe PDBsum
Gene OntologyAmiGO / QuickGO
Search
PMCarticles
PubMedarticles
NCBIproteins
Holocarboxylase synthetase (biotin—(propionyl-Coenzyme A-carboxylase (ATP-hydrolysing)) ligase)
Identifiers
SymbolHLCS
NCBI gene3141
HGNC4976
OMIM609018
RefSeqNM_000411
UniProtP50747
Other data
EC number6.3.4.10
LocusChr. 21 q22.1
Search for
StructuresSwiss-model
DomainsInterPro

Holocarboxylase synthetase (biotin—(propionyl-Coenzyme A-carboxylase (ATP-hydrolysing)) ligase)), also known as protein—biotin ligase, is a family of enzymes (EC 6.3.4.10). This enzyme is important for the effective use of biotin, a B vitamin found in foods such as liver, egg yolks, and milk. In many of the body's tissues, holocarboxylase synthetase activates other specific enzymes (called biotin-dependent carboxylases) by attaching biotin to them. These carboxylases are involved in many critical cellular functions, including the production and breakdown of proteins, fats, and carbohydrates.

The catalyzed reaction:

ATP + biotin + apo-propionyl-CoA:carbon-dioxide ligase (ADP-forming) AMP + diphosphate + propionyl-CoA:carbon-dioxide ligase (ADP-forming)

The 3 substrates of this enzyme are ATP, biotin, and [[apo-[propionyl-CoA:carbon-dioxide ligase (ADP-forming)]], whereas its 3 products are AMP, diphosphate, and propionyl-CoA:carbon-dioxide ligase (ADP-forming).

Holocarboxylase synthetase may also play a role in regulating the activity of genes. In the nucleus, the enzyme likely attaches biotin molecules to histones, which are structural proteins that bind to DNA and give chromosomes their shape. Changing the shape of histones may help determine whether certain genes are turned on or off; however, it is not known how adding biotin affects gene regulation.

The HLCS gene is located on the long (q) arm of chromosome 21 at position 22.1, from base pair 37,045,059 to base pair 37,284,372.


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