NPC1 Rekombinanter Antikörper

NPC1 Rekombinant Antikörper für WB, Indirect ELISA

Wirt / Isotyp

Kaninchen / IgG

Getestete Reaktivität

human

Anwendung

WB, Indirect ELISA

Konjugation

Unkonjugiert

CloneNo.

250854D2

Kat-Nr. : 86233-5-PBS

Synonyme

Niemann Pick C1 protein, Niemann Pick disease, type C1, Niemann-Pick C1 protein, NPC intracellular cholesterol transporter 1



Geprüfte Anwendungen

Produktinformation

86233-5-PBS bindet in WB, Indirect ELISA NPC1 und zeigt Reaktivität mit human

Getestete Reaktivität human
Wirt / Isotyp Kaninchen / IgG
Klonalität Rekombinant
Typ Antikörper
Immunogen NPC1 fusion protein Ag4946
Vollständiger Name Niemann-Pick disease, type C1
Berechnetes Molekulargewicht 142 kDa
Beobachtetes Molekulargewicht 160-200 kDa
GenBank-ZugangsnummerBC063302
Gene symbol NPC1
Gene ID (NCBI) 4864
Konjugation Unkonjugiert
Form Liquid
Reinigungsmethode Protein-A-Reinigung
Lagerungspuffer PBS only
LagerungsbedingungenStore at -80°C. 20ul Größen enthalten 0,1% BSA.

Hintergrundinformationen

Niemann-Pick Type C (NPC) disease is a lysosomal storage disorder characterized by the accumulation of unesterified cholesterol and other lipids in the endolysosomal system. NPC disease results from a defect in either of two distinct cholesterol-binding proteins: a transmembrane protein, NPC1, and a small soluble protein, NPC2. NPC1 or NPC2 deficiency models showed that the functions of these two proteins within lysosomes are linked closely. NPC1 is a typical transmembrane protein and contains a number of modification sites for glycosylation. Defects in NPC1 are the cause of Niemann-Pick disease type C1 which exhibits highly variable clinical phenotype. Moreover, NPC1 may play a role in vesicular trafficking in glia, a process that may be crucial for maintaining the structural and functional integrity of nerve terminals.