bs-11736R-HRP

DATASHEET

Host: Rabbit

Target Protein: NPC2/Niemann Pick C2

Immunogen Range: 20-80/151

Clonality: Polyclonal

Isotype: IgG

Source: KLH conjugated synthetic peptide derived from human Niemann Pick C2

Purification: Purified by Protein A.

Storage Buffer: Aqueous buffered solution containing 0.01M TBS (pH 7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.

Storage: Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.

Background:

NPC2 is a secreted protein mapping against gene 14q24.3 (1,2). NPC2 regulates the lipid composition of sperm membranes during maturation in the epididymis(1,2). Mutations in the NPC2 gene may cause Nieman-Pick type C2 disease and frontal lobe atrophy (1,2,3). Nieman-Pick type C2 is a fatal hereditary disease characterized by defective lysosome release of cholesterol (3). The disease is caused by HE1 deficiency, a lysosmal protein proven to be undetectable in fibroblasts from NPC2 patients (3). This differentiates NPC2 from NPC1, as NPC1 has HE1 protein present (3).

Conjugation: HRP

Excitation/ Emission: N/A

Size: 100ul

Concentration: 1ug/ul

Applications: WB(1:300-5000)
ELISA(1:500-1000)

Predicted Molecular Weight: 15

Cross Reactive Species: Mouse

Predicted Cross Reactive Species: Human
Rat
Cow
Sheep

For research use only. Not intended for diagnostic or therapeutic use.