Anti-NPC1 Rabbit Polyclonal Antibody

Leverandør: Thermo Fisher Scientific
Ratings: Ingen bedømmelse

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PIEAPA116817EA 0 DKK
PIEAPA116817
Anti-NPC1 Rabbit Polyclonal Antibody
Antistoffer
By Western blot, PA1-16817 detects heterogeneously glycosylated NPC1 protein with prominent bands at ~170 and ~220 kDa. Suggested positive control: human fibroblast cell lysate. Niemann-Pick type C1 (NPC1) is a member of a family of genes encoding membrane-bound proteins containing putative sterol sensing domains. NPC1 protein regulates cholesterol transport from late endosomes-lysosomes to other intracellular compartments. NPC1 overexpression increases the rate of trafficking of low density lipoprotein cholesterol to the endoplasmic reticulum and the rate of delivery of endosomal cholesterol to the plasma membrane. NPC disease is an inherited neurovisceral lipid storage disorder of unesterified cholesterol accumulation in lysosomes. It is characterized by progressive neural and liver degeneration, resulting from inactivating mutations in NPC1, in most cases.

Type: Primary
Antigen: NPC1
Clonality: Polyclonal
Clone:
Conjugation: Unconjugated
Epitope:
Host: Rabbit
Isotype: IgG
Reactivity: Hamster, Human, Primates, Mouse, Porcine, Rat
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