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AB3533 Anti-α Galactosidase Antibody

Anti-α Galactosidase Antibody detects level of α Galactosidase & has been published & validated for use in ELISA & WB.

Anti-α Galactosidase Antibody: SDB (Sicherheitsdatenblätter), Analysenzertifikate und Qualitätszertifikate, Dossiers, Broschüren und andere verfügbare Dokumente.
Research Category: Metabolism Research Sub-Category: Enzymes & Biochemistry Gene Symbol: GLA, GALA, melibiase, Melibiase, EC 3.2.1.22 UniProt Number: P06280
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AB3533
100 µg  
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      Key Spec Table

      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
      Bestellnummer GTIN
      AB3533 04053252281266