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Galactosidase alpha Polyclonal Antibody, AbBy Fluor™ 647 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

AbBy Fluor™ 647

Source

KLH conjugated synthetic peptide derived from human Galactosidase alpha

Applications

WB, FCM, IF (IHC-P), IF (IHC-F), IF (ICC)

Purification

Purified by Protein A.

Excitation Emission

650nm/665nm

Concentration

1µg/µl

Dilution

WB (1:300-5000), FCM (1:20-100), IF (IHC-P) (1:50-200), IF (IHC-F) (1:50-200), IF (ICC) (1:50-200)

Buffer

Aqueous buffered solution containing 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.

Modification

Unmodified

Storage Conditions

Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.

Gene ID URL

2717

Predicted Cross Reactivity

Mouse, Dog, Cow, Pig, Rabbit

Curated Selection

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