127334-Biotin
Clone Type
PolyclonalHost
RabbitSource
HumanConjugate
BiotinIsotype
IgGGrade
Affinity PurifiedApplications
WBCrossreactivity
HuAccession #
NM_000169.1, NP_000160.1Shipping Temp
Blue IceStorage Temp
-20°CNotes
Preservative Free
BSA Free
Rabbit Anti-GLA (Alpha-galactosidase A, Alpha-D-galactoside Galactohydrolase, Alpha-D-galactosidase A, Agalsidase, Melibiase) (Biotin)
GLA is 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.
Applications
Suitable for use in Western Blot. Other applications not tested.
Recommended Dilutions
Optimal dilutions to be determined by the researcher.
Amino Acid Sequence
MQLRNPELHLGCALALRFLALVSWDIPGARALDNGLARTPTMGWLHWERFMCNLDCQEEPDSCISEKLFMEMAELMVSEGWKDAGYEYLCIDDCWMAPQRDSEGRLQADPQRFPHGIRQLANYVHSKGLKLGIYADVGNKTCAGFPGSFGYYDIDAQTFADWGVDLLKFDGCYCDSLENLADGYKHMSLALNRTGRSIVYSCEWPLYMWPFQKPNYTEIRQYCNHWRNFADIDDSWKSIKSILDWTSFNQERIVDVAGPGGWNDPDMLVIGNFGLSWNQQVTQMALWAIMAAPLFMSNDLRHISPQAKALLQDKDVIAINQDPLGKQGYQLRQGDNFEVWERPLSGLAWAVAMINRQEIGGPRSYTIAVASLGKGVACNPACFITQLLPVKRKLGFYEWTSRLRSHINPTGTVLLQLENTMQMSLKDLL
Storage and Stability
Store product at 4°C if to be used immediately within two weeks. For long-term storage, aliquot to avoid repeated freezing and thawing and store at -20°C. Aliquots are stable at -20°C for 12 months after receipt. Dilute required amount only prior to immediate use. Further dilutions can be made in assay buffer. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
Note: Applications are based on unconjugated antibody.
Immunogen
Full length protein corresponding to aa1-429 from human GLA.
Form
Supplied as a liquid in PBS, pH 7.4. No preservative added. Labeled with Biotin.
Purity
Purified by Protein A affinity chromatography.
Specificity
Recognizes human GLA.
