DNAPK an atypical protein kinase of the PIKK family. Involved in DNA nonhomologous end joining (NHEJ) required for double-strand break (DSB) repair and V(D)J recombination. and modulation of transcription. Must be bound to DNA to express its catalytic properties. Promotes processing of hairpin DNA structures in V(D)J recombination by activation of the hairpin endonuclease artemis (DCLRE1C). The assembly of the DNA-PK complex at DNA ends is also required for the NHEJ ligation step. Required to protect and align broken ends of DNA. May also act as a scaffold protein to aid the localization of DNA repair proteins to the site of damage. Found at the ends of chromosomes, suggesting a further role in the maintenance of telomeric stability and the prevention of chromosomal end fusion. Also involved in modulation of transcription. Defects cause severe combined immune deficiency (SCID) which is characterized by a lack of mature functional lymphocytes and a high susceptibility to lethal opportunistic infections. Required for repair of radiation-induced dsDNA breaks. Loss of function in mice or horses leads to the SCID (severe combined immune deficiency) phenotype due to failure of immunoglobulin rearrangement. Target of mutation in mismatch repair-deficient colorectal cancer. Inhibitor: KU-7059. 2 isoforms of the human protein are produced by alternative splicing. Note: This description may include information from UniProtKB.
Protein type: ATYPICAL group; DNA repair, damage; DNAPK subfamily; EC; Kinase, protein; Nucleolus; PIKK family; Protein kinase, Ser/Thr (non-receptor); Protein kinase, atypical
Chromosomal Location of Human Ortholog: 16 10.09 cM|16 A2
Cellular Component:  DNA-dependent protein kinase-DNA ligase 4 complex; nonhomologous end joining complex; nucleolus; nucleoplasm; nucleus; protein-containing complex; protein-DNA complex; transcription factor complex
Molecular Function:  ATP binding; DNA binding; DNA-dependent protein kinase activity; double-stranded DNA binding; enzyme binding; kinase activity; nucleotide binding; protein binding; protein domain specific binding; protein kinase activity; protein serine/threonine kinase activity; transcription factor binding; transferase activity
Biological Process:  activation of innate immune response; B cell lineage commitment; brain development; cellular response to DNA damage stimulus; cellular response to insulin stimulus; DNA repair; double-strand break repair; double-strand break repair via nonhomologous end joining; ectopic germ cell programmed cell death; heart development; immune system process; immunoglobulin production; immunoglobulin V(D)J recombination; innate immune response; intrinsic apoptotic signaling pathway in response to DNA damage; lymphocyte differentiation; negative regulation of apoptotic process; negative regulation of cellular senescence; negative regulation of immunoglobulin production; negative regulation of protein phosphorylation; negative regulation of response to gamma radiation; peptidyl-serine phosphorylation; phosphorylation; positive regulation of apoptotic process; positive regulation of developmental growth; positive regulation of double-strand break repair via nonhomologous end joining; positive regulation of fibroblast proliferation; positive regulation of immune system process; positive regulation of transcription by RNA polymerase II; pro-B cell differentiation; protein destabilization; protein phosphorylation; regulation of circadian rhythm; regulation of epithelial cell proliferation; regulation of smooth muscle cell proliferation; response to gamma radiation; response to ionizing radiation; rhythmic process; signal transduction involved in mitotic G1 DNA damage checkpoint; somitogenesis; spleen development; T cell differentiation in thymus; T cell lineage commitment; T cell receptor V(D)J recombination; telomere capping; telomere maintenance; thymus development; V(D)J recombination
Reference #:  P97313 (UniProtKB)
Alt. Names/Synonyms: AI326420; AU019811; DNA-dependent protein kinase catalytic subunit; DNA-PK catalytic subunit; DNA-PKcs; DNAPDcs; DNAPK; DNPK1; HYRC1; p460; Prkdc; protein kinase, DNA activated, catalytic polypeptide; scid; severe combined immunodeficiency; slip; Xrcc7
Gene Symbols: Prkdc
Molecular weight: 471,471 Da
Basal Isoelectric point: 6.73  Predict pI for various phosphorylation states
CST Pathways:  ErbB/HER Signaling  |  G2/M DNA Damage Checkpoint
Select Structure to View Below


Protein Structure Not Found.

Substrate Sequence Logo
Open Substrate Page

Cross-references to other databases:  STRING  |  Reactome  |  BioGPS  |  KinBase  |  Pfam  |  ENZYME  |  Phospho.ELM  |  NetworKIN  |  UniProtKB  |  Entrez-Gene  |  Ensembl Gene