Product info:

This gene encodes a large protein that resides in the limiting membrane of endosomes and lysosomes and mediates intracellular cholesterol trafficking via binding of cholesterol to its N-terminal domain. It is predicted to have a cytoplasmic C-terminus, 13 transmembrane domains, and 3 large loops in the lumen of the endosome - the last loop being at the N-terminus. This protein transports low-density lipoproteins to late endosomal/lysosomal compartments where they are hydrolized and released as free cholesterol. Defects in this gene cause Niemann-Pick type C disease, a rare autosomal recessive neurodegenerative disorder characterized by over accumulation of cholesterol and glycosphingolipids in late endosomal/lysosomal compartments.

Alternative names:

NPC1; NPC; Niemann-Pick C1 protein

Species reactivity:

Human, Mouse, Rat

Host:

Rabbit

Cellular localisation:

Late endosome membrane, Lysosome membrane, Multi-pass membrane protein

Isotype:

IgG

Immunogen:

A synthesized peptide derived from human Niemann Pick C1.

Positive control:

A-549, Mouse liver, Rat lung

AA Sequence:

Email info@sobekbio.com for sequence

Purification:

Affinity purification

Molecular weight:

Calculated MW: 180kDa Observed MW: 180KDa

Form:

Liquid

Applications:

Request info at info@sobekbio.com

Dilution:

WB 1:500 - 1:2000 IHC 1:50 - 1:200 IF 1:50 - 1:200

Storage buffer:

Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.

Storage:

Store at 4°C short term. For long-term storage, aliquot and store at -20°C or below. Stable for 12 months at -20°C. Avoid repeated freeze-thaw cycles.

More info:

Email: info@sobekbio.com

Orders:

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