Mouse Gla ELISA Kit (V2LY-0626-LY2014)

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Tested Data
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Basic Information

Sensitivity
9.23 μ U/mL
Detection Range
20-4500 μ U/mL
Sample Type
Serum, Plasma, cell culture supernates
Specificity
Mouse
Assay Type
Sandwich
Reactivity
Mouse
Assay Time
1.5 h
Molecule Mass
47.6 kDa
Components
  • Pre-coated ELISA plate: 12 wells * 8 detachable strips
  • Standard solution: 0.5ml x1
  • Standard diluent: 3ml x1
  • Streptavidin-HRP: 6ml x1
  • Stop solution: 6ml x1
  • Substrate solution A: 6ml x1
  • Substrate solution B: 6ml x1
  • Wash buffer concentrate (25x): 20ml x1
  • Biotinylated antibody: 1ml x1

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

Storage
Store at 2-8°C
More Infomation

Target

Full Name
Galactosidase Alpha
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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