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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.

Anti-GLA antibodies

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Target: GLA
Host: Mouse
Antibody Isotype: IgG2a
Specificity: Human
Clone: CF184
Application*: ELISA, WB, IHC, IF
Target: GLA
Host: Rabbit
Antibody Isotype: IgG
Specificity: Mouse, Rat, Human
Clone: CBLG1-2776
Application*: WB
Target: GLA
Host: Mouse
Antibody Isotype: IgG2a
Specificity: Human
Clone: 407
Application*: ELISA, IF, IHC, WB
Target: GLA
Host: Mouse
Antibody Isotype: IgG2b, κ
Specificity: Human
Clone: C2
Application*: ICC, IHC, IP, WB
Target: GLA
Host: Mouse
Antibody Isotype: IgG2b, κ
Specificity: Human
Clone: C6
Application*: ICC, IHC, IP, WB
Target: GLA
Host: Mouse
Antibody Isotype: IgG2a
Specificity: Human
Clone: CBFYH-2974
Application*: E, IF, IH, WB, IR
Target: GLA
Host: Rabbit
Antibody Isotype: IgG
Specificity: Human
Clone: 201
Application*: WB
Target: GLA
Host: Mouse
Antibody Isotype: IgG
Specificity: Human
Clone: CBLG1-1256
Application*: E, P, WB
Target: GLA
Host: Mouse
Antibody Isotype: IgG
Specificity: Human
Clone: CBLG1-1253
Application*: E, WB
Target: GLA
Host: Mouse
Antibody Isotype: IgG
Specificity: Human
Clone: CBLG1-1251
Application*: E
Target: GLA
Host: Mouse
Antibody Isotype: IgG
Specificity: Human
Clone: CBLG1-1250
Application*: IC, C/P, E (IA), WB
Target: GLA
Host: Mouse
Antibody Isotype: IgG
Specificity: Human
Clone: CBLG1-1247
Application*: WB
Target: GLA
Host: Mouse
Antibody Isotype: IgG
Specificity: Human
Clone: 2H7G10
Application*: E
Target: GLA
Host: Mouse
Antibody Isotype: IgG
Specificity: Human
Clone: 13j37
Application*: E, IC, IH, WB
Target: GLA
Host: Rabbit
Antibody Isotype: IgG
Specificity: Human
Clone: 1
Application*: E, WB
Target: GLA
Host: Mouse
Antibody Isotype: IgG
Specificity: Human
Clone: CBLG1-120
Application*: E, IC, WB
Target: GLA
Host: Mouse
Antibody Isotype: IgG
Specificity: Human
Clone: CBLG1-119
Application*: WB
Target: GLA
Host: Mouse
Antibody Isotype: IgG2b
Specificity: Human
Clone: M3B
Application*: IP
Target: GLA
Host: Mouse
Antibody Isotype: IgG2b
Specificity: Human
Clone: CBFYH-0371
Application*: E
More Infomation
For Research Use Only. Not For Clinical Use.
(P): Predicted
* Abbreviations
IFImmunofluorescence
IHImmunohistochemistry
IPImmunoprecipitation
WBWestern Blot
EELISA
MMicroarray
CIChromatin Immunoprecipitation
FFlow Cytometry
FNFunction Assay
IDImmunodiffusion
RRadioimmunoassay
TCTissue Culture
GSGel Supershift
NNeutralization
BBlocking
AActivation
IInhibition
DDepletion
ESELISpot
DBDot Blot
MCMass Cytometry/CyTOF
CTCytotoxicity
SStimulation
AGAgonist
APApoptosis
IMImmunomicroscopy
BABioassay
CSCostimulation
EMElectron Microscopy
IEImmunoelectrophoresis
PAPeptide Array
ICImmunocytochemistry
PEPeptide ELISA
MDMeDIP
SHIn situ hybridization
IAEnzyme Immunoassay
SEsandwich ELISA
PLProximity Ligation Assay
ECELISA(Cap)
EDELISA(Det)
BIBioimaging
IOImmunoassay
LFLateral Flow Immunoassay
LALuminex Assay
CImmunohistochemistry-Frozen Sections
PImmunohistologyp-Paraffin Sections
ISIntracellular Staining for Flow Cytometry
MSElectrophoretic Mobility Shift Assay
RIRNA Binding Protein Immunoprecipitation (RIP)
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