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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
A synthetic peptide corresponding to a sequence within amino acids 1179-1278 of human Niemann Pick C1 (O15118).
PBS with 0.02% sodium azide,0.05% BSA,50% glycerol,pH7.3.
Application
WB, IHC, IF/ICC
| biological source | rabbit |
| Quality Segment | 100 |
| conjugate | unconjugated |
| material | colorless |
| clone | 10B2G2, monoclonal |
| form | liquid |
| mol wt | 180 kDa |
| species reactivity | human, rat |
| concentration | 0.85 mg/mL |
| technique(s) | immunofluorescence: 1:50 - 1:200,immunohistochemistry: 1:50 - 1:200,western blot: 1:500 - 1:2000 |
| color | colorless |
| isotype | IgG |
| immunogen sequence | MKGSRVERAEEALAHMGSSVFSGITLTKFGGIVVLAFAKSQIFQIFYFRMYLAMVLLGATHGLIFLPVLLSYIGPSVNKAKSCATEERYKGTERERLLNF |
| UniProt accession no. | O15118 |
| shipped in | wet ice |
| storage temp. | −20°C |
| target post-translational modification | unmodified |
| Gene Information | human ... Niemann Pick C1(4864) |
| 长度(mm) | |
| 宽度(mm) | |
| 高度(mm) | |
| 重量(kg) |
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¥ 2528.88
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¥ 2290.21
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¥ 1779.22
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¥ 8293.41
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¥ 1956.41
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¥ 6894.69
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¥ 238.17
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¥ 3290.05
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¥ 2883.61
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¥ 4897.21
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