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Anti-GLA/alpha-Galactosidase A Antibody (8C241)

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Catalog No. TMAY-02223
Alias GLA/α-Galactosidase A Protein, galactosidase, α, galactosidase, alpha, Ags

Anti-GLA/alpha-Galactosidase A Antibody (8C241) is a Mouse antibody targeting GLA/alpha-Galactosidase A. Anti-GLA/alpha-Galactosidase A Antibody (8C241) can be used in ELISA(Det).

Anti-GLA/alpha-Galactosidase A Antibody (8C241)

Anti-GLA/alpha-Galactosidase A Antibody (8C241)

😃Good
Catalog No. TMAY-02223Alias GLA/α-Galactosidase A Protein, galactosidase, α, galactosidase, alpha, Ags
Anti-GLA/alpha-Galactosidase A Antibody (8C241) is a Mouse antibody targeting GLA/alpha-Galactosidase A. Anti-GLA/alpha-Galactosidase A Antibody (8C241) can be used in ELISA(Det).
Pack SizePriceAvailabilityQuantity
20 μL$92 7-10 days
100 μL$199 7-10 days
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Product Introduction

Bioactivity
Description
Anti-GLA/alpha-Galactosidase A Antibody (8C241) is a Mouse antibody targeting GLA/alpha-Galactosidase A. Anti-GLA/alpha-Galactosidase A Antibody (8C241) can be used in ELISA(Det).
AliasGLA/α-Galactosidase A Protein, galactosidase, α, galactosidase, alpha, Ags
Ig Type
Monoclonal Mouse IgG2b
Clone
8C241
Reactivity
Human
Application
ELISA(Det)
Antibody Type
Monoclonal
Host SpeciesMouse
ConstructionThis antibody was produced from a hybridoma resulting from the fusion of a mouse myeloma with B cells obtained from a mouse immunized with purified, recombinant Human alpha-Galactosidase A (rh alpha-Galactosidase A; TMPY-01727; NP_000160.1; Met1-Leu429). The IgG fraction of the cell culture supernatant was purified by Protein A affinity chromatography.
PurificationProtein A
AppearanceLiquid
Formulation0.2 μm filtered solution in PBS
Research BackgroundAlpha-galactosidase A, also known as Alpha-D-galactoside galactohydrolase, Alpha-D-galactosidase A, Melibiase and GLA, is a member of the glycosyl hydrolase 27 family. GLA is used as a long-term enzyme replacement therapy in patients with a confirmed diagnosis of Fabry disease. Defects in GLA are the cause of Fabry disease (FD) which is a 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 globotriaoslyceramide (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. Deficiency of GLA leads to the accumulation of glycosphingolipids in the vasculature leading to multiorgan pathology. In addition to well-described microvascular disease, deficiency of GLA is also characterized by premature macrovascular events such as stroke and possibly myocardial infarction.
Related Conjugates and Formulations
Conjucates
Unconjugated
Antigen Details
Immunogen
Recombinant Human alpha-Galactosidase A Protein (TMPY-01727)
Antigen Species
Human
Chemical Properties
Stability & Storage
Stability & StorageStore at 2°C-8°C for 1 month. Store at -20°C or -80°C for 12 months. Avoid repeated freeze-thaw cycles. Preservative-Free.
TransportShipping with blue ice.

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Please see Inhibitor Handling Instructions for more frequently ask questions. Topics include: how to prepare stock solutions, how to store products, and cautions on cell-based assays & animal experiments, etc
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