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
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.
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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
More Infomation
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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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