NPC1 Antibody (E-9) is a mouse monoclonal IgG2a (kappa light chain) provided at 200 µg/ml
raised against amino acids 516-630 of NPC1 of human origin
recommended for detection of NPC1 of human origin by WB, IP, IF and ELISA
m-IgG Fc BP-HRP and m-IgG2a BP-HRP are the preferred secondary detection reagents for NPC1 Antibody (E-9) for WB applications. These reagents are now offered in bundles with NPC1 Antibody (E-9) (see ordering information below).
Every item is shipped based on the best shipping method assessed for the temperature requirements of that specific item. Items are grouped and shipped together whenever
possible, and a separate shipping charge will be included for each shipping method required. Shipping charges listed below are from our US warehouses to the Contiguous US,
Alaska, Hawaii, Canada and Puerto Rico. Shipping charges for countries outside the US and Canada will be determined once order has been received
Please note: We can not ship to PO boxes
Express Blue Ice
Express Dry Ice
Animal Health Prescription Item
SHIPPING METHODS & CHARGES
Ships via FedEx Ground to Contiguous US, Alaska, Canada, Monday through Friday. This method is used for less temperature sensitive items such as lab ware and animal
health products, bulky and/or heavy items
Labware ships FedEx Ground free of charge to the contiguous US
Cells obtain cholesterol via two distinct pathways, endogenous synthesis in the endoplasmic reticulum and exogenous uptake through the low-density lipoprotein (LDL) receptor pathway. NPC1 is a protein that resides in late endosomes and lysosomes and is involved in the intracellular trafficking of cholesterol. The human NPC1 gene maps to chromosome 18q11.2 and produces proteins which undergo N-glycosylation and are expressed in brain and liver (2,4,5). NPC1 contains a cysteine-rich domain, which is critical for proper protein function, but is highly mutated. Mutations in NPC1 result in Niemann-Pick disease type C (NPC), an autosomal recessive disease characterized by the accumulation of unesterified cholesterol in the endosomal/lysosomal system. The accumulation of cholesterol results in progressive neurodegeneration and death. More than 90% of cases of NPC are due to mutations in NPC1 and patients with NPC display multiple neurological symptoms, such as hepatosplenomegaly, ataxia, dystonia and dementia (3,8).
For Research Use Only. Not Intended for Diagnostic or Therapeutic Use.