Galactosidase Alpha (GLA) Antibody
195€ (20 µl)
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name
Galactosidase Alpha (GLA) Antibody
category
Primary Antibodies
provider
Abbexa
reference
abx001425
tested applications
ELISA, WB, IF/ICC
Description
GLA Antibody is a Rabbit Polyclonal antibody against 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.
Documents del producto
Instrucciones
Data sheet
Product specifications
| Category | Primary Antibodies |
| Immunogen Target | Galactosidase Alpha (GLA) |
| Host | Rabbit |
| Reactivity | Human, Mouse |
| Recommended Dilution | ELISA: 1 µg/ml, WB: 1/500 - 1/2000, IF/ICC: 1/50 - 1/100. Optimal dilutions/concentrations should be determined by the end user. |
| Clonality | Polyclonal |
| Conjugation | Unconjugated |
| Isotype | IgG |
| Purification | Purified by affinity chromatography. |
| Size 1 | 20 µl |
| Size 2 | 100 µl |
| Size 3 | 2 × 100 µl |
| Form | Liquid |
| Tested Applications | ELISA, WB, IF/ICC |
| Buffer | PBS, pH 7.3, containing 0.02% sodium azide, 50% glycerol. |
| Availability | Shipped within 5-10 working days. |
| Storage | Aliquot and store at -20°C. Avoid repeated freeze/thaw cycles. |
| Dry Ice | No |
| UniProt ID | P06280 |
| Gene ID | 2717 |
| NCBI Accession | NP_000160.1 |
| Background | Antibody anti-GLA |
| Status | RUO |
| Note | Concentration: > 0.2 mg/ml - |
Descripción
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GLA antibody
GLA, also named as Melibiase, Agalsidas and Alpha-galactosidase A, Belongs to the glycosyl hydrolase 27 family. It hydrolysis of terminal, non-reducing alpha-D-galactose residues in alpha-D-galactosides, including galactose oligosaccharides, galactomannans and galactolipids. Fabry disease is an X-linked lysosomal storage disorder resulting from the deficient activity of GLA. Enzyme replacement therapy(ERT) with GLA is currently the most effective therapeutic strategy for patients with Fabry disease, a lysosomal storage disease.
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