LMPD Database

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LMP001519

Record overview

LMPD IDLMP001519
Gene ID3141
SpeciesHomo sapiens (Human)
Gene Nameholocarboxylase synthetase (biotin-(proprionyl-CoA-carboxylase (ATP-hydrolysing)) ligase)
Gene SymbolHLCS
SynonymsHCS
Alternate namesbiotin--protein ligase; biotin apo-protein ligase; biotin--[acetyl-CoA-carboxylase] ligase; biotin--[methylcrotonoyl-CoA-carboxylase] ligase; biotin--[methylmalonyl-CoA-carboxytransferase] ligase; holocarboxylase synthetase (biotin-(proprionyl-Coenzyme A-carboxylase (ATP-hydrolysing)) ligase)
Chromosome21
Map Location21q22.13
EC Number6.3.4.-
SummaryThis 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]
OrthologsView orthologs and multiple alignments for HLCS

Proteins

biotin--protein ligase
Refseq ID:NP_001229713
Protein GI:338753397
UniProt ID:P50747
mRNA ID:NM_001242784
Length:726
RefSeq Status:REVIEWED
MEDRLHMDNGLVPQKIVSVHLQDSTLKEVKDQVSNKQAQILEPKPEPSLEIKPEQDGMEHVGRDDPKALGEEPKQRRGSASGSEPAGDSDRGGGPVEHYH
LHLSSCHECLELENSTIESVKFASAENIPDLPYDYSSSLESVADETSPEREGRRVNLTGKAPNILLYVGSDSQEALGRFHEVRSVLADCVDIDSYILYHL
LEDSALRDPWTDNCLLLVIATRESIPEDLYQKFMAYLSQGGKVLGLSSSFTFGGFQVTSKGALHKTVQNLVFSKADQSEVKLSVLSSGCRYQEGPVRLSP
GRLQGHLENEDKDRMIVHVPFGTRGGEAVLCQVHLELPPSSNIVQTPEDFNLLKSSNFRRYEVLREILTTLGLSCDMKQVPALTPLYLLSAAEEIRDPLM
QWLGKHVDSEGEIKSGQLSLRFVSSYVSEVEITPSCIPVVTNMEAFSSEHFNLEIYRQNLQTKQLGKVILFAEVTPTTMRLLDGLMFQTPQEMGLIVIAA
RQTEGKGRGGNVWLSPVGCALSTLLISIPLRSQLGQRIPFVQHLMSVAVVEAVRSIPEYQDINLRVKWPNDIYYSDLMKIGGVLVNSTLMGETFYILIGC
GFNVTNSNPTICINDLITEYNKQHKAELKPLRADYLIARVVTVLEKLIKEFQDKGPNSVLPLYYRYWVHSGQQVHLGSAEGPKVSIVGLDDSGFLQVHQE
GGEVVTVHPDGNSFDMLRNLILPKRR
 
biotin--protein ligase
Refseq ID:NP_000402
Protein GI:46255045
UniProt ID:P50747
mRNA ID:NM_000411
Length:726
RefSeq Status:REVIEWED
Protein sequence is identical to GI:338753397 (mRNA isoform)
 
biotin--protein ligase
Refseq ID:NP_001229714
Protein GI:338753400
UniProt ID:P50747
mRNA ID:NM_001242785
Length:726
RefSeq Status:REVIEWED
Protein sequence is identical to GI:338753397 (mRNA isoform)