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Chk X Galactosidase, alpha

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货号：AB3533

品牌：Millipore 密理博

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重要规格表

Species Reactivity Key Applications Host Format Antibody Type
H ELISA, WB Ch Affinity Purified Polyclonal Antibody
Description
Catalogue Number AB3533
Brand Family Chemicon®
Trade Name
  • Chemicon
Description Anti-α Galactosidase Antibody
References
Product Information
Format Affinity Purified
Presentation Affinity purified immunoglobulin. Liquid in PBS containing 0.02% sodium azide.
Applications
Application Anti-α Galactosidase Antibody detects level of α Galactosidase & has been published & validated for use in ELISA & WB.
Key Applications
  • ELISA
  • Western Blotting
Application Notes Immunoblotting: 1:1,000-1:5,000

ELISA: 1:1,000-1:5,000

Optimal working dilutions must be determined by the end user.
Biological Information
Immunogen Synthetic peptides from human galactosidase, alpha.
Concentration Please refer to the Certificate of Analysis for the lot-specific concentration.
Host Chicken
Specificity Galactosidase, alpha.
Species Reactivity
  • Human
Antibody Type Polyclonal Antibody
Entrez Gene Number
Entrez Gene 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.
Gene Symbol
  • GLA
  • GALA
  • melibiase
  • Melibiase
  • EC 3.2.1.22
UniProt Number
UniProt Summary SIZE: 429 amino acids; 48767 Da
SUBUNIT: Homodimer.
SUBCELLULAR LOCATION: Lysosome.
DISEASE: SwissProt: P06280 # Defects in GLA are the cause of Fabry disease (FD) [MIM:301500]. 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.
SIMILARITY: SwissProt: P06280 ## Belongs to the glycosyl hydrolase 27 family.
Physicochemical Information
Dimensions
Materials Information
Toxicological Information
Safety Information according to GHS
Safety Information
Product Usage Statements
Usage Statement
  • Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
Storage and Shipping Information
Storage Conditions Maintain at -20°C in undiluted aliquots for up to 6 months after date of receipt. Avoid repeated freeze/thaw cycles.
Packaging Information
Material Size 100 µg
Transport Information
Supplemental Information
Specifications

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