Mouse Monoclonal Antibody to NPC1 | ProMab 30538 product information

This webpage contains legacy information. The product is either no longer available from the supplier or has been delisted at Labome.

product summary

company name :

ProMab

product type :

antibody

product name :

Mouse Monoclonal Antibody to NPC1

catalog :

30538

quantity :

0.1 mg

clonality :

monoclonal

host :

mouse

conjugate :

nonconjugated

clone name :

8D10B6

The same clone is also sold as:

Invitrogen: MA5-31807

reactivity :

human

application :

western blot, ELISA, immunohistochemistry

product information

Catalog :

30538

Title :

Mouse Monoclonal Antibody to NPC1

Species :

Human;

Clone :

8D10B6;

lsotype :

Mouse IgG1

Size :

0.1 mg

Storage :

4℃; -20℃ for long term storage

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 :

Purified recombinant fragment of human NPC1 (AA: 34-174) expressed in E. Coli.

Formulation :

Purified antibody in PBS with 0.05% sodium azide

Applications :

ELISA: 1/10000; WB: 1/500 - 1/2000; IHC: 1/200 - 1/1000

References :

1. Liver Int. 2010 Jul;30(6):887-97. ; 2. Neuroscience. 2010 May 19;167(3):608-20. ;

Type :

Monoclonal Antibody

MW :

142.2kDa

Aliases :

NPC