Mouse Anti-GLA Recombinant Antibody (CBLG1-1253) (CBMAB-G3655-LY)

Name: Mouse Anti-GLA Recombinant Antibody (CBLG1-1253)
SKU: CBMAB-G3655-LY
Rating: 5 (1 reviews)

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Datasheet

SDS

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Datasheet Target Q & As Review & reward Protocols Associated Products

Basic Information

Host Animal

Mouse

Clone

CBLG1-1253

Application

ELISA, WB

Specificity

Human

Antibody Isotype

IgG

Clonality

Monoclonal

Application Notes

The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Format

Liquid

Buffer

50% glycerol

Preservative

0.02% NaN3

Concentration

0.5 mg/mL

Purity

> 95% Purity determined by SDS-PAGE.

Storage

Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freezethaw cycles.

More Infomation

Target

Full Name

Galactosidase Alpha

Introduction

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]

Entrez Gene ID

2717

UniProt ID

P06280

Alternative Names

Galactosidase Alpha; Alpha-D-Galactosidase A; EC 3.2.1.22; Melibiase; Alpha-D-Galactoside Galactohydrolase 1; Alpha-D-Galactoside Galactohydrolase; Alpha-Galactosidase A;

Function

Catalyzes the hydrolysis of glycosphingolipids and participates in their degradation in the lysosome.

Biological Process

Glycoside catabolic process Source: GO_Central
Glycosphingolipid catabolic process Source: UniProtKB
Glycosylceramide catabolic process Source: UniProtKB
Negative regulation of nitric oxide biosynthetic process Source: UniProtKB
Negative regulation of nitric-oxide synthase activity Source: UniProtKB
Oligosaccharide metabolic process Source: UniProtKB

Cellular Location

Lysosome

Involvement in disease

Fabry disease (FD):
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 globotriaosylceramide (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.

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Please try the standard protocols which include: protocols, troubleshooting and guide.

Enzyme-linked Immunosorbent Assay (ELISA)
Flow Cytometry
Immunofluorescence (IF)
Immunohistochemistry (IHC)
Immunoprecipitation (IP)
Western Blot (WB)
Enzyme Linked Immunospot (ELISpot)
Proteogenomic
Other Protocols

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Isotype control

For research use only. Not intended for any clinical use.

Custom Antibody Labeling

We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

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