Rabbit anti-Human Galactosidase Alpha (GLa) Polyclonal Antibody | anti-GLa antibody

Polyclonal Antibody to Galactosidase Alpha (GLa)

Cat. Num. AAA2004427

• Gene Names: GLA; GALA
• Reactivity: Human
• Applications: Immunocytochemistry, Immunohistochemistry, Immunoprecipitation, Western Blot
• Purity: Antigen-specific affinity chromatography followed by Protein A affinity chromatography
• Synonyms: Galactosidase Alpha (GLa); Polyclonal Antibody; Polyclonal Antibody to Galactosidase Alpha (GLa); anti-GLa antibody

• Host: Rabbit
• Reactivity: Human
• Clonality: Polyclonal
• Purity/Purification: Antigen-specific affinity chromatography followed by Protein A affinity chromatography
• Form/Format: Supplied as solution form in 0.01M PBS, pH7.4, containing 0.05% Proclin-300, 50% glycerol.
• Concentration: 500ug/mL (varies by lot)

• Applicable Applications for anti-GLa antibody: Immunocytochemistry (ICC), Immunohistochemistry (IHC) - Formalin/Paraffin, Immunoprecipitation (IP), Western Blot (WB)
• Application Notes: Western blotting: 0.5-2ug/mL;1:500-2000; Immunohistochemistry: 5-20ug/mL;1:50-200; Immunocytochemistry: 5-20ug/mL;1:50-200; Optimal working dilutions must be determined by end user.
• Organism Species: Homo sapiens (Human)
• Source: Polyclonal antibody preparation
• Traits: Liquid
• Cross Reactivity: Human
• Immunogen: Recombinant GLa (Trp81~Leu429) expressed in E.coli
• Preparation and Storage: Storage: Avoid repeated freeze/thaw cycles.; Store at 4ºC for frequent use.; Aliquot and store at -20ºC for 12 months.; ; Stability Test: The thermal stability is described by the loss rate. The loss rate was determined by accelerated thermal degradation test, that is, incubate the protein at 37°C for 48h, and no obvious degradation and precipitation were observed. The loss rate is less than 5% within the expiration date under appropriate storage condition.

Western Blot (WB)

(Western Blot: Sample: Recombinant GLa, Human.)

Immunohistochemistry (IHC)

(DAB staining on IHC-P; Samples: Human Breast Cancer Tissue)

Immunohistochemistry (IHC)

(HE staining on IHC-P; Samples: Human Adrenal Gland Tissue.)

Immunohistochemistry (IHC)

(HE staining on IHC-P; Samples: Human Intestine Tissue)

Immunohistochemistry (IHC)

(DAB staining on IHC-P; Samples: Human Liver Tissue)

Immunohistochemistry (IHC)

(DAB staining on IHC-P; Samples: Human Stomach Cancer Tissue.)

Immunohistochemistry (IHC)

(DAB staining on IHC-P; Samples: Human Kidney Tissue.)

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
• 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
• UniProt Protein Name: Alpha-galactosidase A
• Protein Family: Glycerol facilitator-aquaporin
• UniProt Gene Name: GLA

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; EC 3.2.1.22; Glycan Metabolism - glycosphingolipid biosynthesis - globo series; Hydrolase; Lipid Metabolism - glycerolipid; Lipid Metabolism - sphingolipid

Chromosomal Location of Human Ortholog: Xq22.1

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

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

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

Disease: Fabry Disease