Anti-Erlin-2/ERLIN2 Antibody Picoband® (monoklonale, 3H9A2) fluoro550 Vervoeging

Erlin-2 is a protein that in humans is encoded by the ERLIN2 gene. This gene encodes a member of the SPFH domain-containing family of lipid raft-associated proteins. The encoded protein is localized to lipid rafts of the endoplasmic reticulum and plays a critical role in inositol 1,4,5-trisphosphate (IP3) signaling by mediating ER-associated degradation of activated IP3 receptors. Mutations in this gene are a cause of spastic paraplegia-18 (SPG18) . Alternatively spliced transcript variants encoding multiple isoforms have been observed for this gene.

T-complex protein 1 subunit gamma; TCP-1-gamma; CCT-gamma; hTRiC5; CCT3; CCTG; TRIC5

ERLIN2

11160

O94905

Human

No cross-reactivity with other proteins.

E.coli-derived human Erlin-2/ERLIN2 recombinant protein (Position: D87-N339) .

Clone: 3H9A2

Ubiquitously expressed with highest levels in spleen, thymus and immature brain.

Flow Cytometry

Chaperones, Protein Trafficking, Signal Transduction

Immunogen affinity purified.

Liquid

Molecular chaperone; assists the folding of proteins upon ATP hydrolysis. As part of the BBS/CCT complex may play a role in the assembly of BBSome, a complex involved in ciliogenesis regulating transports vesicles to the cilia. Known to play a role, in vitro, in the folding of actin and tubulin.

1. Al-Saif, A., Bohlega, S., Al-Mohanna, F. Loss of ERLIN2 function leads to juvenile primary lateral sclerosis. Ann. Neurol. 72: 510-516, 2012. 2. Alazami, A. M., Adly, N., Al Dhalaan, H., Alkuraya, F. S. A nullimorphic ERLIN2 mutation defines a complicated hereditary spastic paraplegia locus (SPG18) . Neurogenetics 12: 333-336, 2011. 3. Browman, D. T., Resek, M. E., Zajchowski, L. D., Robbins, S. M. Erlin-1 and erlin-2 are novel members of the prohibitin family of proteins that define lipid-raft-like domains of the ER. J. Cell Sci. 119: 3149-3160, 2006.

At -20 ̊C for one year from date of receipt. Avoid repeated freezing and thawing. Protect from light.

6

Chaperonin containing TCP1 subunit 3

Cytoplasm.

Mouse IgG2b

Each vial contains 50% glycerol, 0.9% NaCl, 0.2% Na2HPO4, 0.02% NaN3.