Galactosidase alpha Polyclonal Antibody, ALEXA FLUOR® 350 Conjugated

Applications

  • IF(IHC-P)

Reactivity

  • Human
  • Mouse
  • Rat
Overview
Catalog # bs-7593R-A350
Product Name Galactosidase alpha Polyclonal Antibody, ALEXA FLUOR® 350 Conjugated
Applications IF(IHC-P)
Reactivity Human, Mouse, Rat
Specifications
Conjugation ALEXA FLUOR® 350
Host Rabbit
Source KLH conjugated synthetic peptide derived from human Galactosidase alpha
Immunogen Range 127-148/429
Clonality Polyclonal
Isotype IgG
Concentration 1ug/ul
Purification Purified by Protein A.
Storage Aqueous buffered solution containing 1% BSA, 50% glycerol and 0.09% sodium azide. Store at 4°C for 12 months.
Target
Gene ID 2717
Swiss Prot P06280
Subcellular location Lysosome
Synonyms GALA; Alpha-galactosidase A; Alpha-D-galactosidase A; Alpha-D-galactoside galactohydrolase; Melibiase; GLA
Background Galactosidase alpha is involved in the hydrolysis of terminal, non reducing alpha D galactose residues in alpha D galactosides, including galactose oligosaccharides, galactomannans and galactohydrolase. Defects in GLA are the cause of Fabry's disease (FD). FD is a rare X-linked sphingolipidosis disease where glycolipid accumulates in many tissues. 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.
Application Dilution
IF(IHC-P) 1:50-200