GCSH
The enzyme system for cleavage of glycine (glycine cleavage system; EC 2.1.2.10), which is confined to the mitochondria, is composed of 4 protein components: P protein (a pyridoxal phosphate-dependent glycine decarboxylase; MIM 238300), H protein (a lipoic acid-containing protein), T protein (a tetrahydrofolate-requiring enzyme; MIM 238310), and L protein (a lipoamide dehydrogenase; MIM 238331). Glycine encephalopathy (GCE; MIM 605899), also called nonketotic hyperglycinemia (NKH), may be due to a defect in any one of these enzymes.[supplied by OMIM
Full Name
glycine cleavage system protein H (aminomethyl carrier)
Function
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).
Biological Process
Glycine catabolic process Source: ProtInc
Glycine decarboxylation via glycine cleavage system Source: GO_Central
Protein lipoylation Source: UniProtKB
Glycine decarboxylation via glycine cleavage system Source: GO_Central
Protein lipoylation Source: UniProtKB
Cellular Location
Mitochondrion
Involvement in disease
Non-ketotic hyperglycinemia (NKH):
Autosomal recessive disease characterized by accumulation of a large amount of glycine in body fluid and by severe neurological symptoms.
Autosomal recessive disease characterized by accumulation of a large amount of glycine in body fluid and by severe neurological symptoms.
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Anti-GCSH antibodies
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Target: GCSH
Host: Mouse
Antibody Isotype: IgG1, κ
Specificity: Human
Clone: CBLG1-819
Application*: SE, E, WB
Target: GCSH
Host: Mouse
Antibody Isotype: IgG1, κ
Specificity: Human
Clone: CBLG1-818
Application*: E, EC, WB
Target: GCSH
Host: Rabbit
Antibody Isotype: IgG
Specificity: Human
Clone: 105
Application*: E
Target: GCSH
Host: Mouse
Antibody Isotype: IgG1, κ
Specificity: Human
Clone: CBFYH-0297
Application*: E, WB
Target: GCSH
Host: Mouse
Antibody Isotype: IgG1, κ
Specificity: Human
Clone: CBLG1-819
Application*: WB, E
Target: GCSH
Host: Mouse
Antibody Isotype: IgG1, κ
Specificity: Human
Clone: CBLG1-818
Application*: WB, E
Target: GCSH
Host: Rabbit
Antibody Isotype: IgG
Specificity: Human
Clone: 105
Application*: E
Target: GCSH
Host: Rabbit
Antibody Isotype: IgG
Specificity: Human
Clone: CBFYH-0297
Application*: P
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(P): Predicted
* Abbreviations
- AActivation
- AGAgonist
- APApoptosis
- BBlocking
- BABioassay
- BIBioimaging
- CImmunohistochemistry-Frozen Sections
- CIChromatin Immunoprecipitation
- CTCytotoxicity
- CSCostimulation
- DDepletion
- DBDot Blot
- EELISA
- ECELISA(Cap)
- EDELISA(Det)
- ESELISpot
- EMElectron Microscopy
- FFlow Cytometry
- FNFunction Assay
- GSGel Supershift
- IInhibition
- IAEnzyme Immunoassay
- ICImmunocytochemistry
- IDImmunodiffusion
- IEImmunoelectrophoresis
- IFImmunofluorescence
- IGImmunochromatography
- IHImmunohistochemistry
- IMImmunomicroscopy
- IOImmunoassay
- IPImmunoprecipitation
- ISIntracellular Staining for Flow Cytometry
- LALuminex Assay
- LFLateral Flow Immunoassay
- MMicroarray
- MCMass Cytometry/CyTOF
- MDMeDIP
- MSElectrophoretic Mobility Shift Assay
- NNeutralization
- PImmunohistologyp-Paraffin Sections
- PAPeptide Array
- PEPeptide ELISA
- PLProximity Ligation Assay
- RRadioimmunoassay
- SStimulation
- SESandwich ELISA
- SHIn situ hybridization
- TCTissue Culture
- WBWestern Blot
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