FUCA1 antibody - N-terminal region
Produktgrößen
25ul
£177,00
ARP54293-P050-25UL
100 ul
£412,00
ARP54293-P050-100UL
Über dieses Produkt
- SKU:
- ARP54293-P050
- Zusätzliche Namen:
- FUCA
- Klonalität:
- Polyclonal
- Konzentration:
- 0.5 mg/ml
- Weitere Details:
- 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.
- Gendetails:
- Fucosidase; alpha-L- 1; tissue
- Host:
- Rabbit
- Immunogen:
- The immunogen is a synthetic peptide directed towards the N terminal region of human FUCA1
- Molekulargewicht:
- 54kDa
- Proteindetails:
- Tissue alpha-L-fucosidase
- Aufreinigung:
- Affinity Purified
- Versandbedingungen:
- Blue Ice
- Lagerbedingungen:
- For short term use; store at 2-8C up to 1 week. For long term storage; store at -20C in small aliquots to prevent freeze-thaw cycles.
- Hersteller:
- Aviva Systems Biology
- Typ:
- Antibodies: Polyclonal Antibody
- Datenblatt des Herstellers:html_datasheet.php