PBS with 0.05 % sodium azide and 0.5 % protein stabilizer
Preservative
Sodium azide
Precaution of Use
This product contains sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
Storage
4 °C/-20 °C
Storage Comment
Store at 4 °C or at -20 °C for long term.
Morales, Amigo, Balboa, Acuña, Castro, Molina, Miquel, Nervi, Rigotti, Zanlungo: "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).
Xu, Zhou, Xia, Xia, Chen, Duan, Luo: "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).
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.