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Rabbit Anti-GLA Recombinant Antibody (CBLG1-2776) (CBMAB-G3648-LY)

This product is antibody recognizes GLA. The antibody CBLG1-2776 immunoassay techniques such as: WB.
See all GLA antibodies

Summary

Host Animal
Rabbit
Specificity
Mouse, Rat, Human
Clone
CBLG1-2776
Antibody Isotype
IgG
Application
WB

Basic Information

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

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
Human2717
Mouse11605
Rat363494
UniProt ID
HumanP06280
MouseP51569
RatD3ZJF9
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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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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