NPC1 Polyclonal antibody proteintech 13926-1-AP
$449.00
In stock
SKU
13926-1-AP
| Host / Isotype: Rabbit / IgG | Class: Polyclonal |
| Reactivity: Human, Mouse And More (2) | Immunogen: CatNo: Ag4946 Product name: Recombinant human NPC1 protein Source: e coli.-derived, PGEX-4T Tag: GST Domain: 23-266 aa of BC063302 Sequence: QSCVWYGECGIAYGDKRYNCEYSGPPKPLPKDGYDLVQELCPGFFFGNVSLCCDVRQLQTLKDNLQLPLQFLSRCPSCFYNLLNLFCELTCSPRQSQFLNVTATEDYVDPVTNQTKTNVKELQYYVGQGFANAMYNACRDVEAPSSNDKALGLLCGKDADACNATNWIEYMFNKDNGQAPFTITPVFSDFPVHGMEPMNNATKGCDESVDEVTAPCSCQDCSIVCGPKPQPPPPPAPWTILGLD Predict reactive species |
| Applications: WB, IHC, IF/ICC, FC (Intra), ELISA | Observed Molecular Weight: 142 kDa |
| Formulation: PBS, Azide, Glycerol | GenBank Accession Number: BC063302 |
| Conjugate: Unconjugated | Gene Symbol: NPC1 |
| Tested Applications: Positive WB detected in | Gene ID (NCBI): 4864 |
| Application: Western Blot (WB) | RRID: AB_2152050 |
| Dilution: WB : 1:500-1:2000 | Conjugate: Unconjugated |
| Tested Reactivity: Human, Mouse | Form: Liquid |
| Host / Isotype: Rabbit / IgG | Background Information: 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. |
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