Anticorps Recombinant de lapin anti-NPC1

NPC1 Recombinant Antibody for WB, Indirect ELISA

Hôte / Isotype

Lapin / IgG

Réactivité testée

Humain

Applications

WB, Indirect ELISA

Conjugaison

Non conjugué

CloneNo.

250854D2

N° de cat : 86233-5-PBS

Synonymes

Niemann Pick C1 protein, Niemann Pick disease, type C1, Niemann-Pick C1 protein, NPC intracellular cholesterol transporter 1

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Galerie de données de validation

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  WB analysis using 86233-5-RR (same clone as 86233-5-PBS)

  100℃ incubated and 37℃ incubated HEK-293 cell lysates were subjected to SDS PAGE followed by western blot with 86233-5-RR (NPC1 antibody) at dilution of 1:5000 incubated at room temperature for 1.5 hours. This data was developed using the same antibody clone with 86233-5-PBS in a different storage buffer formulation.

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Informations sur le produit

86233-5-PBS cible NPC1 dans les applications de WB, Indirect ELISA et montre une réactivité avec des échantillons Humain

• Réactivité: Humain
• Hôte / Isotype: Lapin / IgG
• Clonalité: Recombinant
• Type: Anticorps
• Immunogène: NPC1 Protéine recombinante Ag4946
• Nom complet: Niemann-Pick disease, type C1
• Masse moléculaire calculée: 142 kDa
• Poids moléculaire observé: 160-200 kDa
• Numéro d’acquisition GenBank: BC063302
• Symbole du gène: NPC1
• Identification du gène (NCBI): 4864
• Conjugaison: Non conjugué
• Forme: Liquide
• Méthode de purification: Purification par protéine A
• Tampon de stockage: PBS only
• Conditions de stockage: Store at -80°C. 20ul contiennent 0,1% de BSA.

Informations générales

Niemann-Pick Type C (NPC) disease is a lysosomal storage disorder characterized by the 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.

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