Rabbit anti-Human FUCA1 Polyclonal Antibody | anti-FUCA1 antibody

Anti-FUCA1 Antibody, Rabbit Polyclonal

Cat. Num. AAA8107132

• Gene Names: FUCA1; FUCA
• Reactivity: Human
• Applications: Western Blot, ELISA, Immunoprecipitation
• Purity: Protein A & Antigen Affinity
• Synonyms: FUCA1; Polyclonal Antibody; Anti-FUCA1 Antibody; Rabbit Polyclonal; FUCA1 Antibody; Rabbit PAb; Antigen Affinity Purified; fucosidase; alpha-L-1; tissue; Anti-FUCA Antibody; anti-FUCA1 antibody

• Host: Rabbit
• Reactivity: Human
• Clonality: Polyclonal
• Isotype: Rabbit IgG
• Specificity: Human FUCA1
• Purity/Purification: Protein A & Antigen Affinity
• Form/Format: Liquid; 0.2um filtered solution in PBS

• Applicable Applications for anti-FUCA1 antibody: Western Blot (WB), ELISA (EIA), Immunoprecipitation (IP)
• Application Notes: WB: 1:500-1:1000; ELISA: 1:25,000-1:50,000; IP: 1-2uL/mg of lysate
• Immunogen: Recombinant Human FUCA1 Protein
• Conjugation: Unconjugated
• Preparation: Produced in rabbits immunized with purified, recombinant Human FUCA1 (rh FUCA1). FUCA1 specific IgG was purified by Human FUCA1 affinity chromatography.
• Preparation and Storage: This antibody can be stored at 2-8 degree C for one month without detectable loss of activity. Antibody products are stable for twelve months from date of receipt when stored at -20 degree C to -80 degree C. Preservative-Free. Avoid repeated freeze-thaw cycles.

Western Blot (WB)

(Anti-FUCA1 rabbit polyclonal antibody at 1:500 dilutionLane A: K562 Whole Cell LysateLysates/proteins at 30 ug per lane.SecondaryGoat Anti- Rabbit  IgG H&L (Dylight 800)  at 1/10000 dilution.Developed using the Odyssey technique. Performed under reducing conditions.Predicted band size:54 kDaObserved band size:54 kDa)

Immunoprecipitation (IP)

(FUCA1 was immunoprecipitated using:Lane A:0.5 mg Hela Whole Cell Lysate1 uL anti-FUCA1 rabbit polyclonal antibody and 15 ul of 50 % Protein G agarose.Primary antibody:Anti-FUCA1 rabbit polyclonal antibody,at 1:500 dilution Secondary antibody:Clean-Blot IP Detection Reagent (HRP) at 1:500 dilution Developed using the DAB staining technique.Performed under reducing conditions.Predicted band size: 54 kDaObserved band size: 54 kDa)

Related Product Information for anti-FUCA1 antibody

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.

References

Yang M, et al. (1993) A mutation generating a stop codon in the alpha-L-fucosidase gene of a fucosidosis patient. Biochem Biophys Res Commun. 189(2):1063-8. Fukushima H, et al. (1991) Sequencing and expression of a full-length cDNA for human alpha-L-fucosidase. J Inherit Metab Dis. 13(5):761-5. Kretz KA, et al. (1990) Characterization of EcoRI mutation in fucosidosis patients: a stop codon in the open reading frame. J Mol Neurosci. 1(3):177-80.

NCBI and Uniprot Product Information

• NCBI GI #: 119360348
• NCBI GeneID: 2517
• NCBI Accession #: NP_000138.2
• NCBI GenBank Nucleotide #: NM_000147.4
• UniProt Accession #: P04066; Q14334; Q14335; Q3LID0; Q8NAC2; B2RBG3
• Molecular Weight: 53,689 Da
• NCBI Official Full Name: tissue alpha-L-fucosidase
• NCBI Official Synonym Full Names: fucosidase, alpha-L- 1, tissue
• NCBI Official Symbol: FUCA1
• NCBI Official Synonym Symbols: FUCA
• NCBI Protein Information: tissue alpha-L-fucosidase
• UniProt Protein Name: Tissue alpha-L-fucosidase
• Protein Family: Tissue alpha-L-fucosidase
• UniProt Gene Name: FUCA1
• UniProt Synonym Gene Names: Alpha-L-fucosidase 1
• UniProt Entry Name: FUCO_HUMAN

Uniprot Description

FUCA1: Alpha-L-fucosidase is responsible for hydrolyzing the alpha-1,6-linked fucose joined to the reducing-end N- acetylglucosamine of the carbohydrate moieties of glycoproteins. Defects in FUCA1 are the cause of fucosidosis (FUCA1D). FUCA1D is an autosomal recessive lysosomal storage disease characterized by accumulation of fucose-containing glycolipids and glycoproteins in various tissues. Clinical signs include facial dysmorphism, dysostosis multiplex, moderate hepatomegaly, severe intellectual deficit, deafness, and according to age, angiokeratomas. Belongs to the glycosyl hydrolase 29 family.

Protein type: EC 3.2.1.51; Glycan Metabolism - other glycan degradation; Hydrolase

Chromosomal Location of Human Ortholog: 1p34

Cellular Component: cytoplasm; lysosomal lumen

Molecular Function: alpha-L-fucosidase activity

Biological Process: fucose metabolic process; glycoside catabolic process

Disease: Fucosidosis