Recombinant Human Serine/threonine-protein kinase ATR (ATR), Truncated

Uniprot Id

Q13535

Target Species

Human

Target Name

ATR

Target Full Name

Serine/threonine-protein kinase ATR

Target Function

Serine/threonine protein kinase which activates checkpoint signaling upon genotoxic stresses such as ionizing radiation (IR), ultraviolet light (UV), or DNA replication stalling, thereby acting as a DNA damage sensor. Recognizes the substrate consensus sequence [ST]-Q. Phosphorylates BRCA1, CHEK1, MCM2, RAD17, RPA2, SMC1 and p53/TP53, which collectively inhibit DNA replication and mitosis and promote DNA repair, recombination and apoptosis. Phosphorylates 'Ser-139' of histone variant H2AX at sites of DNA damage, thereby regulating DNA damage response mechanism. Required for FANCD2 ubiquitination. Critical for maintenance of fragile site stability and efficient regulation of centrosome duplication. Positively regulates the restart of stalled replication forks following activation by the KHDC3L-OOEP scaffold complex.

Target Involvement

Seckel syndrome 1 (SCKL1); Cutaneous telangiectasia and cancer syndrome, familial (FCTCS)

Target Subcellular Location

Nucleus. Chromosome. Note=Depending on the cell type, it can also be found in PML nuclear bodies. Recruited to chromatin during S-phase. Redistributes to discrete nuclear foci upon DNA damage, hypoxia or replication fork stalling.

Target Protein Families

PI3/PI4-kinase family, ATM subfamily

Target Tissue Specificity

Ubiquitous, with highest expression in testis. Isoform 2 is found in pancreas, placenta and liver but not in heart, testis and ovary.

Target Synonyms

Ataxia telangiectasia and Rad3 related; Ataxia telangiectasia and Rad3-related protein; ATR; ATR_HUMAN; FCTCS; FRAP Related Protein 1; FRAP-related protein 1; FRP1; MEC1; MEC1 mitosis entry checkpoint 1 homolog; Protein kinase ATR; RAC3; Rad3 related protein; SCKL; SCKL1; Serine/threonine protein kinase ATR; Serine/threonine-protein kinase ATR

Target Background

The protein encoded by this gene is a serine/threonine kinase and DNA damage sensor, activating cell cycle checkpoint signaling upon DNA stress. The encoded protein can phosphorylate and activate several proteins involved in the inhibition of DNA replication and mitosis, and can promote DNA repair, recombination, and apoptosis. This protein is also important for fragile site stability and centrosome duplication. Defects in this gene are a cause of Seckel syndrome 1.