Polyclonal Antibody to Galactosidase Alpha (GLa) | MyBioSource MBS2004427 product information

product summary

company name :

MyBioSource

product type :

antibody

product name :

Polyclonal Antibody to Galactosidase Alpha (GLa)

catalog :

MBS2004427

quantity :

0.01 mg

price :

105 USD

clonality :

polyclonal

host :

rabbit

conjugate :

nonconjugated

reactivity :

human

application :

western blot, immunohistochemistry, immunocytochemistry, immunoprecipitation, immunohistochemistry - paraffin section

more info or order :

MyBioSource product webpage

image

image 1 :

Western Blot: Sample: Recombinant GLa, Human.

image 2 :

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

image 3 :

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

product information

catalog number :

MBS2004427

products type :

Antibody

products full name :

Polyclonal Antibody to Galactosidase Alpha (GLa)

products short name :

[Galactosidase Alpha (GLa)]

other names :

[alpha-galactosidase A; Alpha-galactosidase A; alpha-galactosidase A; galactosidase alpha; Alpha-D-galactosidase A; Alpha-D-galactoside galactohydrolase; Melibiase; INN: Agalsidase]

products gene name :

[GLa]

other gene names :

[GLA; GLA; GALA]

clonality :

Polyclonal

host :

Rabbit

purity :

Antigen-specific affinity chromatography followed by Protein A affinity chromatography

form :

Supplied as solution form in 0.01M PBS, pH7.4, containing 0.05% Proclin-300, 50% glycerol.

concentration :

1mg/ml

storage stability :

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.

tested application :

Immunocytochemistry (ICC), Immunohistochemistry (IHC) - Formalin/Paraffin, Immunoprecipitation (IP), Western Blot (WB)

app 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.

image1 heading :

Western Blot (WB)

image2 heading :

Immunohistochemistry (IHC)

image3 heading :

Immunohistochemistry (IHC)

image4 heading :

Immunohistochemistry (IHC)

image4 description :

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

image5 heading :

Immunohistochemistry (IHC)

image5 description :

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

other info1 :

Organism Species: Homo sapiens (Human). Source: Polyclonal antibody preparation. Traits: Liquid

other info2 :

Immunogen: Recombinant GLa (Trp81~Leu429) expressed in E.coli

ncbi gi num :

4504009

ncbi acc num :

NP_000160.1

ncbi gb acc num :

NM_000169.2

uniprot acc num :

P06280

ncbi pathways :

Galactose Metabolism Pathway (82931); Galactose Metabolism Pathway (292); Glycerolipid Metabolism Pathway (82986); Glycerolipid Metabolism Pathway (361); Glycosphingolipid Biosynthesis - Globo Series Pathway (82996); Glycosphingolipid Biosynthesis - Globo Series Pathway (371); Glycosphingolipid Metabolism Pathway (530751); Lysosome Pathway (99052); Lysosome Pathway (96865); Metabolism Pathway (477135)

ncbi summary :

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 summary :

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

size1 :

0.01 mg

price1 :

105 USD

size2 :

0.02 mg

price2 :

125

size3 :

0.05 mg

price3 :

165

size4 :

0.1 mg

price4 :

210

size5 :

0.2 mg

price5 :

310

size6 :

1 mg

price6 :

725

more info or order :

MyBioSource product webpage