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
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-Zugangsnummer | BC063302 |
| Gene symbol | NPC1 |
| Gene ID (NCBI) | 4864 |
| Konjugation | Unkonjugiert |
| Form | Liquid |
| Reinigungsmethode | Protein-A-Reinigung |
| Lagerungspuffer | PBS only |
| Lagerungsbedingungen | Store 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.

