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Product Name
Anti-FUCA1 antibody
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Description
Rabbit Polyclonal to Human FUCA1
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Tested applications
WB, ELISA, IP
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Species reactivity
Human FUCA1
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Alternative names
FUCA antibody; Afuc antibody; Fuca antibody; 0610006A03Rik antibody; 9530055J05Rik antibody; 0610006A03Rik antibody; 9530055J05Rik antibody; Afuc antibody; alpha-L- 1 antibody; alpha-L- 1 antibody; alpha-L-1 antibody; alpha-L-fucosidase 1 antibody; alpha-L-fucosidase 1 antibody; alpha-L-fucosidase I antibody; alpha-L-fucosidase I antibody; alpha-L-fucoside fucohydrolase 1 antibody; alpha-L-fucoside fucohydrolase 1 antibody; Fuca antibody; FUCA antibody; FUCA1 antibody; Fuca1 antibody; fucosidase antibody; fucosidase antibody; RP11-45G17.1 antibody; RP23-161N17.6 antibody; tissue antibody; tissue antibody; tissue alpha-L-fucosidase antibody; tissue alpha-L-fucosidase antibody
- Immunogen
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Isotype
Rabbit IgG
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Preparation
Produced in rabbits immunized with purified, recombinant Human FUCA1 . FUCA1 specific IgG was purified by Human FUCA1 affinity chromatography.
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Clonality
Polyclonal
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Formulation
0.2 μm filtered solution in PBS
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Storage instructions
This antibody can be stored at 2℃-8℃ for one month without detectable loss of activity. Antibody products are stable for twelve months from date of receipt when stored at -20℃ to -80℃. Preservative-Free.
Sodium azide is recommended to avoid contamination (final concentration 0.05%-0.1%). It is toxic to cells and should be disposed of properly. Avoid repeated freeze-thaw cycles. -
Applications
WB: 5-20 μg/ml
ELISA: 0.1-0.2 μg/mL
This antibody can be used at 0.1-0.2 μg/mL with the appropriate secondary reagents to detect Human FUCA1.
IP: 4-8 uL/mg of lysate
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Validations
Human FUCA1 Immunoprecipitation(IP) 15769
Human FUCA1 Western blot (WB) 15768
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Background
FUCA1 is a lysosomal enzyme involved in the degradation of fucose-containing glycoproteins and glycolipids. Mutations in FUCA1 gene are associated with fucosidosis (FUCA1D), which is an autosomal recessive lysosomal storage disease. Different phenotypes include clinical features such as neurologic deterioration, growth retardation, visceromegaly, and seizures in a severe early form; coarse facial features, angiokeratoma corporis diffusum, spasticity and delayed psychomotor development in a longer surviving form; and an unusual spondylometaphyseoepiphyseal dysplasia in yet another form.
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Please note: All products are "FOR RESEARCH USE ONLY AND ARE NOT INTENDED FOR DIAGNOSTIC OR THERAPEUTIC USE"