IDUA Rekombinanter Antikörper

IDUA Rekombinant Antikörper für WB, Sandwich ELISA, Indirect ELISA

Wirt / Isotyp

Kaninchen / IgG

Getestete Reaktivität

human, Maus, Ratte

Anwendung

WB, Sandwich ELISA, Indirect ELISA

Konjugation

Unkonjugiert

CloneNo.

251274F3

Kat-Nr. : 86499-1-PBS

Synonyme

Alpha-L-iduronidase, EC:3.2.1.76, MPS1



Geprüfte Anwendungen

Produktinformation

86499-1-PBS bindet in WB, Sandwich ELISA, Indirect ELISA IDUA und zeigt Reaktivität mit human, Maus, Ratten

Getestete Reaktivität human, Maus, Ratte
Wirt / Isotyp Kaninchen / IgG
Klonalität Rekombinant
Typ Antikörper
Immunogen IDUA fusion protein Ag30658
Vollständiger Name iduronidase, alpha-L-
Berechnetes Molekulargewicht 73 kDa
Beobachtetes Molekulargewicht73-75 kDa
GenBank-ZugangsnummerNM_000203
Gene symbol IDUA
Gene ID (NCBI) 3425
Konjugation Unkonjugiert
Form Liquid
Reinigungsmethode Protein-A-Reinigung
Lagerungspuffer PBS only
LagerungsbedingungenStore at -80°C. 20ul Größen enthalten 0,1% BSA.

Hintergrundinformationen

Iduronidase (L-iduronidase, alpha-L-iduronidase, laronidase) is an enzyme with the systematic name glycosaminoglycan alpha-L-iduronohydrolase. This enzyme catalyzes the hydrolysis of unsulfated alpha-L-iduronosidic linkages in dermatan sulfate. It is a glycoprotein enzyme found in the lysosomes of cells. It is involved in the degeneration of glycosaminoglycans such as dermatan sulfate and heparan sulfate. The enzyme acts by hydrolyzing the terminal alpha-L-iduronic acid residues of these molecules, degrading them (PMID: 4993544,30407). A deficiency in the IDUA protein is associated with mucopolysaccharidoses (MPS). MPS, a type of lysosomal storage disease, is typed I through VII. In this syndrome, glycosaminoglycans accumulate in the lysosomes and cause substantial disease in many different tissues of the body. IDUA mutations result in the MPS 1 phenotype, which is inherited in an autosomal recessive fashion. The defective alpha-L-iduronidase results in an accumulation of heparan and dermatan sulfate within phagocytes, endothelium, smooth muscle cells, neurons, and fibroblasts. Prenatal diagnosis of this enzyme deficiency is possible (PMID:8242073).