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Product Name
NPC2 antibody
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Description
NPC2 Rabbit Polyclonal antibody. Positive WB detected in HT-1080 cells, mouse kidney tissue. Observed molecular weight by Western-blot: 21-23 kDa
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Tested applications
ELISA, WB
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Species reactivity
human, mouse, rat; other species not tested.
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Alternative names
NPC2 antibody; HE1 antibody; Niemann Pick disease antibody; type C2 antibody
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Isotype
Rabbit IgG
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Preparation
This antibody was obtained by immunization of NPC2 recombinant protein (Accession Number: NM_006432). Purification method: Antigen affinity purified.
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Clonality
Polyclonal
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Formulation
PBS with 0.02% sodium azide and 50% glycerol pH 7.3.
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Storage instructions
Store at -20℃. DO NOT ALIQUOT
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Applications
Recommended Dilution:
WB: 1:200-1:2000
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Validations
HT-1080 cells were subjected to SDS PAGE followed by western blot with Catalog No:113210(NPC2 antibody) at dilution of 1:600
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Background
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 function of these two proteins within lysosomes are linked closely. NPC2 is also named human epididymis-specific protein 1 (HE1), defects of which are the cause of Niemann-Pick disease type C2, characterized as a lysosomal storage disorder that affects the viscera and the central nervous system. Recent finding suggests that NPC2 may serve as a novel intracrine/autocrine factor that controls adipocyte differentiation and function as well as potential therapeutic target for the treatment of type 2 diabetes and related metabolic disorders.
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