GLA

This gene encodes a homodimeric glycoprotein that hydrolyses the terminal alpha-galactosyl moieties from glycolipids and glycoproteins. This enzyme predominantly hydrolyzes ceramide trihexoside, and it can catalyze the hydrolysis of melibiose into galactose and glucose. A variety of mutations in this gene affect the synthesis, processing, and stability of this enzyme, which causes Fabry disease, a rare lysosomal storage disorder that results from a failure to catabolize alpha-D-galactosyl glycolipid moieties. [provided by RefSeq, Jul 2008]
Full Name
Galactosidase Alpha
Function
Catalyzes the hydrolysis of glycosphingolipids and participates in their degradation in the lysosome.
Biological Process
Glycoside catabolic process Source: GO_Central
Glycosphingolipid catabolic process Source: UniProtKB
Glycosylceramide catabolic process Source: UniProtKB
Negative regulation of nitric oxide biosynthetic process Source: UniProtKB
Negative regulation of nitric-oxide synthase activity Source: UniProtKB
Oligosaccharide metabolic process Source: UniProtKB
Cellular Location
Lysosome
Involvement in disease
Fabry disease (FD):
Rare X-linked sphingolipidosis disease where glycolipid accumulates in many tissues. The disease consists of an inborn error of glycosphingolipid catabolism. FD patients show systemic accumulation of globotriaosylceramide (Gb3) and related glycosphingolipids in the plasma and cellular lysosomes throughout the body. Clinical recognition in males results from characteristic skin lesions (angiokeratomas) over the lower trunk. Patients may show ocular deposits, febrile episodes, and burning pain in the extremities. Death results from renal failure, cardiac or cerebral complications of hypertension or other vascular disease. Heterozygous females may exhibit the disorder in an attenuated form, they are more likely to show corneal opacities.
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Anti-GLA antibodies

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Mouse Anti-GLA Recombinant Antibody (C6) (CBMAB-AP2538LY)

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Target: GLA
Host: Mouse
Antibody Isotype: IgG2b, κ
Specificity: Human
Clone: C6
Application*: ICC, IHC, IP, WB
Datasheet SDS

Mouse Anti-GLA Recombinant Antibody (3B) (CBMAB-G0505-LY)

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Target: GLA
Host: Mouse
Antibody Isotype: IgG1
Specificity: Human
Clone: 3B
Application*: IH
Datasheet SDS

Mouse Anti-GLA Recombinant Antibody (CF184) (CBMAB-FT452LY)

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Target: GLA
Host: Mouse
Antibody Isotype: IgG2a
Specificity: Human
Clone: CF184
Application*: ELISA, WB, IHC, IF
Datasheet SDS

Rabbit Anti-GLA Recombinant Antibody (CBLG1-2776) (CBMAB-G3648-LY)

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Target: GLA
Host: Rabbit
Antibody Isotype: IgG
Specificity: Mouse, Rat, Human
Clone: CBLG1-2776
Application*: WB
Datasheet SDS

Rabbit Anti-GLA Recombinant Antibody (CBLG1-2776) (V2LY-1225-LY1426)

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Target: GLA
Host: Rabbit
Antibody Isotype: IgG
Specificity: Human
Clone: CBLG1-2776
Application*: E, IP
Datasheet SDS

Mouse Anti-GLA Recombinant Antibody (CBLG1-1251) (V2LY-1225-LY1425)

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Target: GLA
Host: Mouse
Antibody Isotype: IgG1
Specificity: Human
Clone: CBLG1-1251
Application*: E
Datasheet SDS

Mouse Anti-GLA Recombinant Antibody (CBLG1-1247) (V2LY-1225-LY1423)

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Target: GLA
Host: Mouse
Antibody Isotype: IgG2b
Specificity: Human
Clone: CBLG1-1247
Application*: E
Datasheet SDS

Rabbit Anti-GLA Recombinant Antibody (1) (V2LY-1225-LY1421)

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Target: GLA
Host: Rabbit
Antibody Isotype: IgG
Specificity: Human
Clone: 1
Application*: WB
Datasheet SDS

Rabbit Anti-GLA Recombinant Antibody (
30H11) (V2LY-0725-LY1519)

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Target: GLA
Host: Rabbit
Antibody Isotype: IgG
Specificity: Human
Clone: 30H11
Application*: E, IH, F
Datasheet SDS

Mouse Anti-GLA Recombinant Antibody (407) (CBMAB-AP2540LY)

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Target: GLA
Host: Mouse
Antibody Isotype: IgG2a
Specificity: Human
Clone: 407
Application*: ELISA, IF, IHC, WB
Datasheet SDS

Mouse Anti-GLA Recombinant Antibody (C2) (CBMAB-AP2539LY)

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Target: GLA
Host: Mouse
Antibody Isotype: IgG2b, κ
Specificity: Human
Clone: C2
Application*: ICC, IHC, IP, WB
Datasheet SDS

Mouse Anti-GLA Monoclonal Antibody (CBFYH-2974) (CBMAB-H1241-FY)

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Target: GLA
Host: Mouse
Antibody Isotype: IgG2a
Specificity: Human
Clone: CBFYH-2974
Application*: E, IF, IH, WB, IR
Datasheet SDS

Rabbit Anti-GLA Recombinant Antibody (201) (CBMAB-A4425-YC)

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Target: GLA
Host: Rabbit
Antibody Isotype: IgG
Specificity: Human
Clone: 201
Application*: WB
Datasheet SDS

Mouse Anti-GLA Recombinant Antibody (CBLG1-1256) (CBMAB-G3658-LY)

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Target: GLA
Host: Mouse
Antibody Isotype: IgG
Specificity: Human
Clone: CBLG1-1256
Application*: E, P, WB
Datasheet SDS

Mouse Anti-GLA Recombinant Antibody (CBLG1-1253) (CBMAB-G3655-LY)

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Target: GLA
Host: Mouse
Antibody Isotype: IgG
Specificity: Human
Clone: CBLG1-1253
Application*: E, WB
Datasheet SDS

Mouse Anti-GLA Recombinant Antibody (CBLG1-1251) (CBMAB-G3653-LY)

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Target: GLA
Host: Mouse
Antibody Isotype: IgG
Specificity: Human
Clone: CBLG1-1251
Application*: E
Datasheet SDS

Mouse Anti-GLA Recombinant Antibody (CBLG1-1250) (CBMAB-G3652-LY)

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Target: GLA
Host: Mouse
Antibody Isotype: IgG2b, κ
Specificity: Human
Clone: CBLG1-1250
Application*: WB, IP, IF, E, P
Datasheet SDS

Mouse Anti-GLA Recombinant Antibody (CBLG1-1247) (CBMAB-G3649-LY)

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Target: GLA
Host: Mouse
Antibody Isotype: IgG
Specificity: Human
Clone: CBLG1-1247
Application*: WB
Datasheet SDS

Mouse Anti-GLA Recombinant Antibody (10) (CBMAB-G3646-LY)

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Target: GLA
Host: Mouse
Antibody Isotype: IgG
Specificity: Human
Clone: 10
Application*: IF
Datasheet SDS

Mouse Anti-GLA Recombinant Antibody (13j37) (CBMAB-G3644-LY)

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Target: GLA
Host: Mouse
Antibody Isotype: IgG
Specificity: Human
Clone: 13j37
Application*: E, IC, IH, WB
Datasheet SDS

Rabbit Anti-GLA Recombinant Antibody (1) (CBMAB-G3643-LY)

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Target: GLA
Host: Rabbit
Antibody Isotype: IgG
Specificity: Human
Clone: 1
Application*: E, WB
Datasheet SDS

Mouse Anti-GLA Recombinant Antibody (CBLG1-120) (CBMAB-G0508-LY)

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Target: GLA
Host: Mouse
Antibody Isotype: IgG
Specificity: Human
Clone: CBLG1-120
Application*: E, IC, WB
Datasheet SDS

Mouse Anti-GLA Recombinant Antibody (CBLG1-119) (CBMAB-G0507-LY)

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Target: GLA
Host: Mouse
Antibody Isotype: IgG
Specificity: Human
Clone: CBLG1-119
Application*: WB
Datasheet SDS

Mouse Anti-GLA (AA 1-429) Recombinant Antibody (CBFYH-0371) (CBMAB-H1242-FY)

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Target: GLA
Host: Mouse
Antibody Isotype: IgG2b
Specificity: Human
Clone: CBFYH-0371
Application*: E
Datasheet SDS
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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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