Galactosidase alpha Antibody, Biotin Conjugated
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Catalog number
bs-7593R-Biotin
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Price
Please ask
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Size
0.1ml
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Long name
Galactosidase alpha Polyclonal Antibody, Biotin Conjugated
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Category
Conjugated Primary Antibodies
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Conjugation
Biotin
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Host Organism
Rabbit (Oryctolagus cuniculus)
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Target Antigen
Galactosidase alpha
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Specificity
This is a highly specific antibody against Galactosidase alpha.
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Modification
Unmodified
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Modification site
None
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Clonality
Polyclonal
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Clone
Polyclonal antibody
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Concentration
1ug per 1ul
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Immunogen_range
127-148/429
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Subcellular location
Lysosome
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Source
KLH conjugated synthetic peptide derived from human Galactosidase alpha
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Gene ID number
2717
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Swiss Prot
P06280
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Tested applications
WB, IHC-P
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Recommended dilutions
WB(1:100-1000), IHC-P(1:100-500)
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Crossreactivity
Human, Mouse, Rat
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Crossreactive species details
Due to limited amount of testing and knowledge, not every possible cross-reactivity is known.
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Antigen 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.
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Purification method
This antibody was purified via Protein A.
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Storage conditions
Keep the antibody in an aqueous buffered solution containing 1% BSA, 50% glycerol and 0.09% sodium azide. Store refrigerated at 2 to 8 degrees Celcius for up to 1 year.
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Excitation Emission
N/A
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Synonyms
GALA; Alpha-galactosidase A; Alpha-D-galactosidase A; Alpha-D-galactoside galactohydrolase; Melibiase; GLA
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Description
The Anti-Galactosidase alpha is a α- or alpha protein sometimes glycoprotein present in blood.
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Properties
If you buy Antibodies supplied by Bioss Primary Conjugated Antibodies they should be stored frozen at - 24°C for long term storage and for short term at + 5°C. Biotin conjugates can be detected by horseradish peroxidase, alkaline phosphatase substrates or anti biotin conjugated antibodies. Avidin and Streptavidin bind to the small biotin and are couple to HRP or AP for ELISA. To break the streptavidin Biotin bond we suggest to use a 6 molar guanidine HCl solution with acidity of pH 1.6.
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Additional conjugation
Biotinylated
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French translation
anticorps
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Gene target
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Short name
Anti-Galactosidase alpha
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Technique
Antibody, antibodies against human proteins, antibodies for, antibody Conjugates
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Isotype
Immunoglobulin G (IgG)
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Label
Biotin
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Alternative name
Anti-Galactosidase alpha PAb Biotin
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Alternative technique
antibodies
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MeSH Data
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Name
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Concept
Scope note:
Identification of proteins or peptides that have been electrophoretically separated by blot transferring from the electrophoresis gel to strips of nitrocellulose paper, followed by labeling with antibody probes.
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Tree numbers
- E05.196.401.143
- E05.301.300.096
- E05.478.566.320.200
- E05.601.262
- E05.601.470.320.200
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Qualifiers
ethics, trends, veterinary, history, classification, economics, instrumentation, methods, standards, statistics & numerical data
Product images
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