Galactosidase alpha Polyclonal Antibody, Biotin Conjugated
Product Specifications
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.
Synonyms
GALA; Alpha-galactosidase A; Alpha-D-galactosidase A; Alpha-D-galactoside galactohydrolase; Melibiase; GLA
Gene ID
2717
Swiss Prot
P06280
Cellular Locus
Cytoplasm
Host
Rabbit
Cross Reactivity
Human, Rat
Immunogen
101-200/429
Target
Galactosidase alpha
Clonality
Polyclonal
Isotype
IgG
Conjugation
Biotin
Source
KLH conjugated synthetic peptide derived from human Galactosidase alpha
Applications
WB, ELISA, IHC-P, IHC-F
Purification
Purified by Protein A.
Concentration
1µg/µl
Dilution
WB (1:300-5000), ELISA (1:500-1000), IHC-P (1:200-400), IHC-F (1:100-500)
Buffer
Aqueous buffered solution containing 0.01M TBS (pH 7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
Modification
Unmodified
Storage Conditions
Store at -20°C for 12 months.
Gene ID URL
2717
Predicted Cross Reactivity
Mouse, Dog, Cow, Pig, Rabbit
Curated Selection
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