Mouse anti-Human GLA/alpha-Galactosidase A Monoclonal Antibody | anti-GLA antibody

Anti-GLA/alpha-Galactosidase A Antibody, Mouse Monoclonal

Cat. Num. AAA8106151

• Gene Names: GLA; GALA
• Reactivity: Human
• Applications: ELISA
• Purity: Protein A
• Synonyms: GLA/alpha-Galactosidase A; Monoclonal Antibody; Anti-GLA/alpha-Galactosidase A Antibody; Mouse Monoclonal; alpha-Galactosidase A Antibody; Mouse MAb; galactosidase; alpha; Anti-GALA Antibody; anti-GLA antibody

• Host: Mouse
• Reactivity: Human
• Clonality: Monoclonal
• Clone Number: 05
• Specificity: Human alpha-Galactosidase A/GLA
• Purity/Purification: Protein A
• Form/Format: Liquid; 0.2um filtered solution in PBS

• Applicable Applications for anti-GLA antibody: ELISA (EIA) (Det)
• Application Notes: ELISA (Det): This antibody will detect Human alpha-Galactosidase A in ELISA pair set.; In a sandwich ELISA, it can be used as detection antibody when paired with.
• Immunogen: Recombinant Human alpha-Galactosidase A Protein
• Conjugation: Unconjugated
• Preparation: This 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). The IgG fraction of the cell culture supernatant was purified by Protein A affinity chromatography.
• Preparation and Storage: This antibody can be stored at 2-8 degree C for one month without detectable loss of activity. Antibody products are stable for twelve months from date of receipt when stored at -20 degree C to -80 degree C. Preservative-Free. Avoid repeated freeze-thaw cycles.

Related Product Information for anti-GLA antibody

Alpha-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 identification 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.

References

Koide T.et al., 1990, FEBS Lett. 259:353-356. Yang C.-C. et al., 2003, Clin. Genet. 63:205-209. Verovnik F. et al., 2004, Eur. J. Hum. Genet. 12:678-681. Nance C.S. et al., 2006, Arch. Neurol. 63:453-457.

NCBI and Uniprot Product Information

• NCBI GI #: 4504009
• NCBI GeneID: 2717
• NCBI Accession #: NP_000160.1
• NCBI GenBank Nucleotide #: NM_000169.2
• UniProt Accession #: P06280; Q6LER7
• Molecular Weight: 48,767 Da
• NCBI Official Full Name: alpha-galactosidase A
• NCBI Official Synonym Full Names: galactosidase, alpha
• NCBI Official Symbol: GLA
• NCBI Official Synonym Symbols: GALA
• NCBI Protein Information: alpha-galactosidase A; melibiase; alpha-gal A; agalsidase alfa; alpha-D-galactosidase A; alpha-D-galactoside galactohydrolase 1
• UniProt Protein Name: Alpha-galactosidase A
• Protein Family: Glycerol facilitator-aquaporin
• UniProt Gene Name: GLA
• UniProt Entry Name: AGAL_HUMAN

NCBI Description

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]

Uniprot Description

GLA: Defects in GLA are the cause of Fabry disease (FD). FD 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. Heterozygous females may exhibit the disorder in an attenuated form, they are more likely to show corneal opacities. Belongs to the glycosyl hydrolase 27 family.

Protein type: Carbohydrate Metabolism - galactose; Lipid Metabolism - sphingolipid; Glycan Metabolism - glycosphingolipid biosynthesis - globo series; Hydrolase; EC 3.2.1.22; Lipid Metabolism - glycerolipid

Chromosomal Location of Human Ortholog: Xq22

Cellular Component: Golgi apparatus; lysosomal lumen; lysosome; cytoplasm; extracellular region

Molecular Function: protein binding; protein homodimerization activity; hydrolase activity; alpha-galactosidase activity; galactoside binding; catalytic activity; receptor binding

Biological Process: sphingolipid metabolic process; negative regulation of nitric-oxide synthase activity; glycoside catabolic process; negative regulation of nitric oxide biosynthetic process; glycosphingolipid catabolic process; glycosylceramide catabolic process; glycosphingolipid metabolic process; oligosaccharide metabolic process

Disease: Fabry Disease