FUCA1 Rabbit Polyclonal Antibody

CAT#: TA336245

Rabbit Polyclonal Anti-FUCA1 Antibody

Product Images

WB Suggested Anti-FUCA1 Antibody Titration: 0.2-1 ug/ml; Positive Control: Human Liver

Specifications

Product Data

• Applications: WB
• Recommended Dilution: WB
• Reactivities: Human
• Host: Rabbit
• Isotype: IgG
• 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: Store at -20°C as received.
• Stability: Stable for 12 months from date of receipt.
• Predicted Protein Size: 54 kDa
• Gene Name: fucosidase, alpha-L- 1, tissue
• Database Link:
  NP_000138
  Entrez Gene 2517 Human
  UniProt ID P04066
• 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