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AB3533 Anti-α Galactosidase Antibody

AB3533
100 µg  
Purchase on Sigma-Aldrich

特卖

概述

Replacement Information

重要规格表

Species ReactivityKey ApplicationsHostFormatAntibody Type
HELISA, WBChAffinity PurifiedPolyclonal Antibody
Description
Catalogue NumberAB3533
Brand Family Chemicon®
Trade Name
  • Chemicon
DescriptionAnti-α Galactosidase Antibody
References
Product Information
FormatAffinity Purified
PresentationAffinity purified immunoglobulin. Liquid in PBS containing 0.02% sodium azide.
Quality LevelMQ100
Applications
ApplicationAnti-α Galactosidase Antibody detects level of α Galactosidase & has been published & validated for use in ELISA & WB.
Key Applications
  • ELISA
  • Western Blotting
Application NotesImmunoblotting: 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
ImmunogenSynthetic peptides from human galactosidase, alpha.
ConcentrationPlease refer to the Certificate of Analysis for the lot-specific concentration.
HostChicken
SpecificityGalactosidase, alpha.
Species Reactivity
  • Human
Antibody TypePolyclonal Antibody
Entrez Gene Number
Entrez Gene SummaryThis 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 SummarySIZE: 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 ConditionsMaintain at -20°C in undiluted aliquots for up to 6 months after date of receipt. Avoid repeated freeze/thaw cycles.
Packaging Information
Material Size100 µg
Transport Information
Supplemental Information
Specifications
Global Trade Item Number
产品目录编号 GTIN
AB3533 04053252281266