FUCA1 Rabbit Polyclonal Antibody
CAT#: TA336245
Rabbit Polyclonal Anti-FUCA1 Antibody
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CNY 5,200.00
CNY 300.00
CNY 1,430.00
CNY 2,900.00
CNY 6,650.00
Specifications
Product Data | |
Applications | WB |
Recommend Dilution | WB |
Reactivity | Human |
Host | Rabbit |
Clonality | Polyclonal |
Immunogen | The immunogen for Anti-FUCA1 Antibody: synthetic peptide directed towards the N terminal of human FUCA1. Synthetic peptide located within the following region: PSPVSWNWNSKDVGPHRDLVGELGTALRKRNIRYGLYHSLLEWFHPLYLL |
Formulation | Liquid. Purified antibody supplied in 1x PBS buffer with 0.09% (w/v) sodium azide and 2% sucrose. Note that this product is shipped as lyophilized powder to China customers. |
Purification | Affinity Purified |
Conjugation | Unconjugated |
Storage Condition | Store at -20°C as received. |
Predicted Protein Size | 54 kDa |
Gene Name | fucosidase, alpha-L- 1, tissue |
Database Link | |
Background | Alpha-L-fucosidase (EC 3.2.1.51) is a lysosomal enzyme involved in the degradation of fucose-containing glycoproteins and glycolipids. At least 2 separate polymorphic alpha-L-fucosidases are recognized in man: that in tissues, FUCA1, which is deficient in fucosidosis, and that in plasma, FUCA2. Fucosidosis is an autosomal recessive lysosomal storage disease caused by defective alpha-L-fucosidase with accumulation of fucose in the tissues. 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. [supplied by OMIM]. Publication Note: This RefSeq record includes a subset of the publications that are available for this gene. Please see the Entrez Gene record to access additional publications. |
Synonyms | FUCA |
Note | Immunogen Sequence Homology: Pig: 100%; Rat: 100%; Human: 100%; Mouse: 100%; Bovine: 100%; Dog: 93%; Horse: 93%; Guinea pig: 93%; Rabbit: 79% |
Reference Data | |
Protein Families | Druggable Genome |
Protein Pathways | Lysosome, Other glycan degradation |
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