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Rabbit Anti-PERK/FITC Conjugated antibody
background:
The protein encoded by this gene 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. It is a type I membrane protein located in the endoplasmic reticulum (ER), where it is induced by ER stress caused by malfolded proteins. Mutations in this gene are associated with Wolcott-Rallison syndrome. [provided by RefSeq, Jan 2010]
Function:
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. Serves as a critical effector of unfolded protein response (UPR)-induced G1 growth arrest due to the loss of cyclin-D1 (CCND1).
Subunit:
Forms dimers with HSPA5/BIP in resting cells. Oligomerizes in ER-stressed cells. Interacts with DNAJC3.
Subcellular Location:
Endoplasmic reticulum membrane; Single-pass type I membrane protein.
Tissue Specificity:
Ubiquitous. A high level expression is seen in secretory tissues.
Post-translational modifications:
Oligomerization of the N-terminal ER luminal domain by ER stress promotes PERK trans-autophosphorylation of the SLCterminal cytoplasmic kinase domain at multiple residues including Thr-982 on the kinase activation loop. Autophosphorylated. Phosphorylated at Tyr-619 following endoplasmic reticulum stress, leading to activate its tyrosine-protein kinase activity. Dephosphorylated by PTPN1/TP1B, leading to inactivate its enzyme activity.
N-glycosylated.
ADP-ribosylated by PARP16 upon ER stress, which increases kinase activity.
DISEASE:
Wolcott-Rallison syndrome (WRS) [MIM:226196]: 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. Note=The disease is caused by mutations affecting the gene represented in this entry.
Similarity:
Belongs to the protein kinase superfamily. Ser/Thr protein kinase family. GCN2 subfamily.
Contains 1 protein kinase domain.
Database links:
Entrez Gene: 9451 Human
Entrez Gene: 13666 Mouse
Entrez Gene: 29702 Rat
Omim: 604032 Human
SwissProt: Q9NZJ5 Human
SwissProt: Q9Z2B5 Mouse
SwissProt: Q9Z1Z1 Rat
Unigene: 591589 Human
Unigene: 247167 Mouse
Unigene: 24897 Rat
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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