NPC1 antibody (AA 34-174)
Quick Overview for NPC1 antibody (AA 34-174) (ABIN1724840)
Target
See all NPC1 AntibodiesReactivity
Host
Clonality
Conjugate
Application
Clone
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Binding Specificity
- AA 34-174
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Purpose
- NPC1 Antibody
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Purification
- Purified antibody
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Immunogen
- Purified recombinant fragment of human NPC1 (AA: 34-174) expressed in E. Coli.
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Isotype
- IgG1
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Application Notes
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ELISA: 1/10000
FCM: 1/200 - 1/400
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Restrictions
- For Research Use only
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Format
- Liquid
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Buffer
- Purified antibody in PBS with 0.05 % sodium azide.
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Preservative
- Sodium azide
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Precaution of Use
- This product contains Sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
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Storage
- 4 °C,-20 °C
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Storage Comment
- Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze/thaw cycles.
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Deficiency of Niemann-Pick C1 protein protects against diet-induced gallstone formation in mice." in: Liver international : official journal of the International Association for the Study of the Liver, Vol. 30, Issue 6, pp. 887-97, (2010) (PubMed).
: "Defects of synaptic vesicle turnover at excitatory and inhibitory synapses in Niemann-Pick C1-deficient neurons." in: Neuroscience, Vol. 167, Issue 3, pp. 608-20, (2010) (PubMed).
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Deficiency of Niemann-Pick C1 protein protects against diet-induced gallstone formation in mice." in: Liver international : official journal of the International Association for the Study of the Liver, Vol. 30, Issue 6, pp. 887-97, (2010) (PubMed).
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- NPC1 (Niemann-Pick Disease, Type C1 (NPC1))
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Alternative Name
- NPC1
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Background
- 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.
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Molecular Weight
- 142.2 kDa
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Gene ID
- 4864
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UniProt
- O15118
Target
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