EIF2AK3, ID (PEK, WRS, PERK, DKFZp781H1925) (PE)

EIF2AK3, a member of the GCN2 subfamily of Ser/Thr protein kinases, phosphorylates the alpha subunit of eukaryotic translation-initiation factor 2 (EIF2), leading to its inactivation and thus to a rapid reduction of translational initiation and repression of global protein synthesis. This protein serves as a critical effector of unfolded protein response (UPR)-induced G1 growth arrest due to the loss of cyclin D1. It is proposed that perturbation in protein folding in the endoplasmic reticulum (ER) promotes reversible dissociation from HSPA5/BIP and oligomerization, resulting in transautophosphorylation and kinase activity induction Expression of this Type I membrane protein is ubiquitous, with a high level expression in secretory tissues. Defects in EIF2AK3 are the cause of Wolcott-Rallison syndrome (WRS), also known as multiple epiphyseal dysplasia with early-onset diabetes mellitus. WRS is a rare autosomal recessive disorder, characterized by permanent neonatal or early infancy insulin-dependent diabetes and, at a later age, epiphyseal dysplasia, osteoporosis, growth retardation and other multisystem manifestations, such as hepatic and renal dysfunctions, mental retardation and cardiovascular abnormalities.\n\nApplications:\nSuitable for use in Immunohistochemistry, ELISA\n\nRecommended Dilution:\nELISA: 1:1,000\nImmunohistochemistry: 1:10-50\n\nStorage and Stability:\nMay be stored at 4 degrees C for short-term only. Aliquot to avoid repeated freezing and thawing. Do not freeze R-Phycoerythrin conjugates. Light sensitive. Aliquots are stable for 6 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

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SPECIFICATIONS

Catalog Number

034988-PE

Size

200ul

Applications

ELISA, IHC

Hosts

Rabbit

Reactivities

Hum

Form

Supplied as a liquid in PBS, pH 7.2. Contains ~0.09% sodium azide. Conjugated to R-Phycoerythrin (R-PE).

P Type

Pab

Purity

Purified by Protein A affinity chromatography.

Isotype

IgG

References

Delepine, M., et al., Nat. Genet. 25(4):406-409 (2000).\nShi, Y., et al., J. Biol. Chem. 274(9):5723-5730 (1999).\nSood, R., et al., Biochem. J. 346 Pt 2, 281-293 (2000).

Additional Info

Human

Alternative Names

PEK, WRS, PERK, DKFZp781H1925