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DraconiticBiochemistryBiomolecules · Catabolism · Biosynthesis · MetabolismMap

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Human metabolism

Reactome's human metabolism drawn as one network. Each molecule has one home node; widely shared molecules also appear as dashed copies in other territories, linked from their details. .

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Metabolite; its structure appears as you zoom in
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Dashed ring: a copy of a widely shared molecule or cofactor in another territory or pathway
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Binding, release or complex event; its proteins are named in its details
Outline: reactions mostly in the mitochondrion

Arrows follow Reactome's curated reaction direction.

HLCS biotinylates ACACB

ACACB:2Mn2+ + Btn + ATP → Btn-ACACB:2Mn2+ + AMP + PPi

Reactome: R-HSA-4167511
Compartment: cytosol · mitochondrial outer membrane
Rhea: None recorded

Inputs

ACACB:2Mn2+R-HSA-2993859, mitochondrial outer membrane
BtnCHEBI:15956, C10H16N2O3S, cytosol
ATPCHEBI:30616, C10H12N5O13P3, cytosol

Outputs

Btn-ACACB:2Mn2+R-HSA-2993829, mitochondrial outer membrane
AMPCHEBI:456215, C10H12N5O7P, cytosol
PPiCHEBI:33019, HO7P2, cytosol

Catalysed by

biotin--[biotin carboxyl-carrier protein] ligase activity of HLCS [cytosol]EC 6.3.4.10; UniProt P50747

Literature

Mutations in the holocarboxylase synthetase gene HLCS Hum. Mutat., 2005
Holocarboxylase synthetase: correlation of protein localisation with biological function Arch. Biochem. Biophys., 2010
Identification of holocarboxylase synthetase (HCS) proteins in human placenta Biochim. Biophys. Acta, 1998
Selectivity in post-translational biotin addition to five human carboxylases J. Biol. Chem., 2012
The subcellular localization of acetyl-CoA carboxylase 2 Proc Natl Acad Sci U S A, 2000
Human acetyl-CoA carboxylase: characterization, molecular cloning, and evidence for two isoforms Proc Natl Acad Sci U S A, 1995