Niemann Pick C1 polyclonal Antibody | BS90945

Niemann Pick C1 polyclonal Antibody | BS90945 | Gentaur UK, US & Europe Distribution

Host: Rabbit

Reactivity: Human, Mouse, Rat

Application: WB ICC IF IHC FC

Application Range: WB:1:500-1:1,000 ICC/IF:1:50-1:200 IHC:1:50-1:200 FC:1:50-1:100

Background: 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. 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.

Storage & Stability: Store at +4°C after thawing. Aliquot store at -20°C or -80°C. Avoid repeated freeze / thaw cycles.

Specificity: Niemann Pick C1 polyclonal Antibody detects endogenous levels of Niemann Pick C1 protein.

Molecular Weight: 142 kDa

Note: For research use only, not for use in diagnostic procedure.

Alternative Names: NPC1, Niemann Pick C1 protein precursor, Niemann Pick disease, type C1, Niemann-Pick C1 protein, NPC, NPC1, NPC1,

Immunogen: Recombinant protein

Conjugate: Unconjugated

Modification: Unmodification

Purification & Purity: ProA affinity purified

Pathway: