Monoclonal Anti-NPC1 antibody produced in mouse
SIGMA/WH0004864M2 - clone 4H2, purified immunoglobulin, buffered aqueous solution
Synonym: Anti-NPC; Anti-Niemann-Pick disease, type C1
MDL Number: MFCD03455661
Product Type: Chemical
Western Blotting NPC1 monoclonal antibody, clone 4H2. Western Blot analysis of NPC1 expression in HeLa.
Western Blotting QC Western Blot detection against Immunogen (36.63 kDa).
ELISA Detection limit for recombinant GST tagged NPC1 is approximately 0.03 ng/mL as a capture antibody.
| antibody form | purified immunoglobulin |
| antibody product type | primary antibodies |
| biological source | mouse |
| clone | 4H2, monoclonal |
| conjugate | unconjugated |
| form | buffered aqueous solution |
| GenBank accession no. | BC063302   |
| isotype | IgG2aκ |
| Quality Level | 100 |
| shipped in | dry ice |
| species reactivity | human |
| storage temp. | −20°C |
| target post-translational modification | unmodified |
| technique(s) | indirect ELISA: suitable |
| western blot: 1-5 μg/mL | |
| UniProt accession no. | O15118 |
| Disclaimer: | Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals. |
| General description: | This gene encodes a large protein that resides in the limiting membrane of endosomes and lysosomes and mediates intracellular cholesterol trafficking via binding of cholesterol to its N-terminal domain. It is predicted to have a cytoplasmic C-terminus, 13 transmembrane domains, and 3 large loops in the lumen of the endosome - the last loop being at the N-terminus. This protein transports low-density lipoproteins to late endosomal/lysosomal compartments where they are hydrolized and released as free cholesterol. Defects in this gene cause Niemann-Pick type C disease, a rare autosomal recessive neurodegenerative disorder characterized by over accumulation of cholesterol and glycosphingolipids in late endosomal/lysosomal compartments |
| Immunogen: | NPC1 (AAH63302, 151 a.a. ~ 250 a.a) partial recombinant protein with GST tag. MW of the GST tag alone is 26 KDa. Sequence GFANAMYNACRDVEAPSSNDKALGL |
| Legal Information: | GenBank is a registered trademark of United States Department of Health and Human Services |
| Physical form: | Solution in phosphate buffered saline, pH 7.4 |
| RIDADR | NONH for all modes of transport |
| Flash Point(F) | Not applicable |
| Flash Point(C) | Not applicable |
| Storage Temp. | −20°C |
| UNSPSC | 12352203 |