Anti-NPC1 Rabbit Polyclonal Antibody

Supplier: Proteintech
10091-242EA 402 USD
Anti-NPC1 Rabbit Polyclonal Antibody
Niemann-Pick Type C (NPC) disease is a lysosomal storage disorder characterized by accumulation of unesterified cholesterol and other lipids in the endolysosomal system. NPC disease results from a defect in either of two distinct cholesterol-binding proteins: a transmembrane protein, NPC1, and a small soluble protein, NPC2. NPC1 or NPC2 deficiency models showed that the functions of these two proteins within lysosomes are linked closely. NPC1 is a typical transmembrane protein and contains a number of modification sites for glycosylation. Defects in NPC1 are the cause of Niemann-Pick disease type C1 which exhibits highly variable clinical phenotype. Moreover, NPC1 may play a role in vesicular trafficking in glia, a process that may be crucial for maintaining the structural and functional integrity of nerve terminals.

Western Blot: HEK-293 Cells, 1:500-1:5000; IHC: Human Brain Tissue, 1:20-1:200

Type: Primary
Antigen: NPC1
Clonality: Polyclonal
Conjugation: Unconjugated
Host: Rabbit
Isotype: IgG
Reactivity: Human
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