Niemann Pick C1 Recombinant Rabbit Monoclonal Antibody [JB87-33]

About this product

Niemann-Pick disease, type C1 (NPC1) is a disease of a membrane protein that mediates intracellular cholesterol trafficking in mammals. In humans the protein is encoded by the NPC1 gene (chromosome location 18q11). NPC1 was identified as the gene that when mutated, results in Niemann-Pick disease, type C. Niemann-Pick disease, type C is a rare neurovisceral lipid storage disorder resulting from autosomal recessively inherited loss-of-function mutations in either NPC1 or NPC2. This disrupts intracellular lipid transport, leading to the accumulation of lipid products in the late endosomes and lysosomes. Approximately 95% of NPC patients are found to have mutations in the NPC1 gene. NPC1 encodes a putative integral membrane protein containing sequence motifs consistent with a role in intracellular transport of cholesterol to post-lysosomal destinations.

Specifications

Catalog number

ET7107-57

Size / count

100 µL

Dry ice

No

Hazardous

No

Discontinued

No

CAS Number

9000-83-3

Product Name Alternative

Niemann Pick C1 protein precursor antibody Niemann Pick disease, type C1 antibody Niemann-Pick C1 protein antibody NPC antibody NPC1 antibody NPC1_HUMAN antibody

Abbreviation

NPC1

Swiss Prot

O15118 Human, O35604 Mouse,

Cellular Locus

Endosome. Lysosome.

Host

Rabbit

Species Reactivity

Human, Mouse, Rat

Immunogen

Recombinant protein within Human Niemann Pick C1 aa 1,160-1,278 / 1,278.

Isotype

IgG

Conjugation

Non-conjugated

Type

Recombinant Rabbit monoclonal Antibody

Applications

WB, IF-Cell, IF-Tissue, IHC-P, FC

Positive Control

SiHa cell lysates, Wild-type HEK293 whole cell lysates, A549, HepG2, PC-3M, human kidney tissue, rat kidney tissue, mouse testis tissue, SH-SY5Y.

Concentration

1 mg/mL

Dilution

WB: 1:500-1:1,000;IF-Cell: 1:50-1:200;IF-Tissue: 1:50-1:200;IHC-P: 1:50-1:200;FC: 1:50-1:100

Purity

Protein A affinity purified.

Form

Liquid

Buffer

1*TBS (pH7.4), 0.05% BSA, 40% Glycerol. Preservative: 0.05% Sodium Azide.

Molecular Weight

Predicted band size: 142 kDa

Storage Conditions

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

Recombinant Antibody

Yes