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Details on Person UniProt:P23434 GCSH

Class:IdReferenceGeneProduct:55510
_chainChangeLogtransit peptide:1-48 added on Sat February 7 2015;chain:49-173 added on Sat February 7 2015
_displayNameUniProt:P23434 GCSH
_timestamp2026-02-20 21:39:48
chaintransit peptide:1-48
chain:49-173
checksum3CC5D09D59C1243D
commentFUNCTION The glycine cleavage system catalyzes the degradation of glycine. The H protein (GCSH) shuttles the methylamine group of glycine from the P protein (GLDC) to the T protein (GCST). Has a pivotal role in the lipoylation of enzymes involved in cellular energetics such as the mitochondrial dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex (DLAT), and the mitochondrial dihydrolipoyllysine-residue succinyltransferase component of 2-oxoglutarate dehydrogenase complex (DLST) (PubMed:36190515).COFACTOR Binds 1 lipoyl cofactor covalently.SUBUNIT Interacts with GLDC (By similarity). The glycine cleavage system is composed of four proteins: P (GLDC), T (GCST), L (DLD) and H (GCSH).INTERACTION The disease is caused by variants affecting the gene represented in this entry.SIMILARITY Belongs to the GcvH family.
descriptionrecommendedName: fullName evidence="9"Glycine cleavage system H protein, mitochondrial alternativeName: Lipoic acid-containing protein
geneNameGCSH
identifierP23434
isSequenceChangedFALSE
keyword3D-structure
Disease variant
Lipoyl
Mitochondrion
Neurodegeneration
Proteomics identification
Reference proteome
Transit peptide
modified[InstanceEdit:9836292] Weiser, Joel, 2023-05-25
[InstanceEdit:9852000] Weiser, Joel, 2023-11-03
[InstanceEdit:9862192] Weiser, Joel, 2024-02-26
[InstanceEdit:9926675] Weiser, Joel, 2024-11-03
[InstanceEdit:9983091] Weiser, Joel, 2026-02-20
nameGCSH
referenceDatabase[ReferenceDatabase:2] UniProt
referenceGene[ReferenceDNASequence:8999503] ENSEMBL:ENSG00000140905 GCSH [Homo sapiens]
secondaryIdentifierGCSH_HUMAN
Q9H1E9
sequenceLength173
species[Species:48887] Homo sapiens
(referenceEntity)[EntityWithAccessionedSequence:5693951] GCSH [mitochondrial matrix] [Homo sapiens]
[EntityWithAccessionedSequence:5694601] lipoyl-K107-GCSH [mitochondrial matrix] [Homo sapiens]
[EntityWithAccessionedSequence:6792571] lipo-107-GCSH [mitochondrial matrix] [Homo sapiens]
[EntityWithAccessionedSequence:6793586] octanoyl-K107-GCSH [mitochondrial matrix] [Homo sapiens]
[EntityWithAccessionedSequence:6793611] Lipo-K107-GCSH [mitochondrial matrix] [Homo sapiens]
[EntityWithAccessionedSequence:9930732] AmMeLipo-K107-GCSH [mitochondrial matrix] [Homo sapiens]
(referenceSequence)[ModifiedResidue:5694589] N6-lipoyl-L-lysine at 107
[ModifiedResidue:6792576] N6-lipoyl-L-lysine at 107
[ModifiedResidue:6793588] N6-octanoyl-L-lysine at 107
[GroupModifiedResidue:9930735] N6-lipoyl-L-lysine (N(6)-((R)-S(8)-ammoniomethyldihydrolipoyl)-L-lysine(1+) residue) at 173
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No pathways have been reviewed or authored by UniProt:P23434 GCSH (55510)