- Catalog number
- PA1784-Dylight550
- Size / count
- 100 µg
- Dry ice
- No
- Hazardous
- No
- Discontinued
- Yes
- Background
- ATM (ataxia telangiectasia mutated),also known as TEL1 or TELO1, is a serine/threonine protein kinase that is recruited and activated by DNA double-strand breaks. The ATM protein is a member of the phosphatidylinositol 3-kinase family of proteins that respond to DNA damage by phosphorylating key substrates involved in DNA repair and/or cell cycle control. Linkage analysis of ataxia-telangiectasia led to mapping of the ATM gene to chromosome 11q22.3. Using an antiserum developed to a peptide corresponding to the deduced amino acid sequence of ATM, the ATM protein is a single, high molecular weight protein predominantly confined to the nucleus of human fibroblasts, although it is present in both nuclear and microsomal fractions from human lymphoblast cells and peripheral blood lymphocytes. Overexpression of ATM cDNA in AT cells enhanced their survival after radiation exposure, decreased radiation-induced chromosome aberrations, reduced radioresistant DNA synthesis, and partially corrected defective cell cycle checkpoints and induction of stress-activated protein kinase. ATM has an essential role in the reconstitutive capacity of hematopoietic stem cells but is not as important for the proliferation or differentiation of progenitors, in a telomere-independent manner. ATM functions ly in the repair of chromosomal DNA double-stranded breaks by maintaining DNA ends in repair complexes generated during lymphocyte gene assembly.
- Synonyms
- Serine-protein kinase ATM;2.7.11.1;Ataxia telangiectasia mutated;A-T mutated;ATM;
- Gene Name
- Serine-protein kinase ATM
- UniProt
- Q13315
- Host
- Rabbit
- Reactivity
- Rat,Mouse,Human
- Cross Reactivity
- No cross-reactivity with other proteins
- Immunogen
- A synthetic peptide corresponding to a sequence at the N-terminus of human ATM, different from the related rat and mouse sequences by two amino acids.
- Clonality
- Polyclonal
- Tissue Specificity
- Found in pancreas, kidney, skeletal muscle, liver, lung, placenta, brain, heart, spleen, thymus, testis, ovary, small intestine, colon and leukocytes.
- Applications
- WB
- Purification
- Immunogen affinity purified.
- Concentration
- Adding 0.2 ml of distilled water will yield a concentration of 500 μg/ml.
- Form
- Lyophilized
- Reconstitution
- Add 0.2ml of distilled water will yield a concentration of 500ug/ml.
- Function
- Serine/threonine protein kinase which activates checkpoint signaling upon double strand breaks (DSBs), apoptosis and genotoxic stresses such as ionizing ultraviolet A light (UVA), thereby acting as a DNA damage sensor. Recognizes the substrate consensus sequence [ST]-Q. Phosphorylates 'Ser-139' of histone variant H2AX/H2AFX at double strand breaks (DSBs), thereby regulating DNA damage response mechanism. Also plays a role in pre-B cell allelic exclusion, a process leading to expression of a single immunoglobulin heavy chain allele to enforce clonality and monospecific recognition by the B-cell antigen receptor (BCR) expressed on individual B-lymphocytes. After the introduction of DNA breaks by the RAG complex on one immunoglobulin allele, acts by mediating a repositioning of the second allele to pericentromeric heterochromatin, preventing accessibility to the RAG complex and recombination of the second allele. Also involved in signal transduction and cell cycle control. May function as a tumor suppressor. Necessary for activation of ABL1 and SAPK. Phosphorylates DYRK2, CHEK2, p53/TP53, FANCD2, NFKBIA, BRCA1, CTIP, nibrin (NBN), TERF1, RAD9 and DCLRE1C. May play a role in vesicle and/or protein transport. Could play a role in T-cell development, gonad and neurological function. Plays a role in replication-dependent histone mRNA degradation. Binds DNA ends. Phosphorylation of DYRK2 in nucleus in response to genotoxic stress prevents its MDM2-mediated ubiquitination and subsequent proteasome degradation. Phosphorylates ATF2 which stimulates its function in DNA damage response. .
- Components
- Each vial contains 5mg BSA, 0.9mg NaCl, 0.2mg Na2HPO4, 0.05mg Thimerosal, 0.05mg NaN3.
- References & Citations
- 1. Allen, D. M., van Praag, H., Ray, J., Weaver, Z., Winrow, C. J., Carter, T. A., Braquet, R., Harrington, E., Ried, T., Brown, K. D., Gage, F. H., Barlow, C. Ataxia telangiectasia mutated is essential during adult neurogenesis. Genes Dev. 15: 554-566, 2001.
2. Bakkenist, C. J., Kastan, M. B. DNA damage activates ATM through intermolecular autophosphorylation and dimer dissociation.Nature 421: 499-506, 2003.
3. Chong, M. J., Murray, M. R., Gosink, E. C., Russell, H. R. C., Srinivasan, A., Kapsetaki, M., Korsmeyer, S. J., McKinnon, P. J. Atm and Bax cooperate in ionizing radiation-induced apoptosis in the central nervous system. Proc. Nat. Acad. Sci. 97: 889-894, 2000.
- Storage Conditions
- Store at -20℃ for one year from date of receipt. After reconstitution, at 4℃ for one month. It can also be aliquotted and stored frozen at -20℃ for six months. Avoid repeated freeze-thaw cycles.
- Calculated Molecular Weight
- 350687 MW
- Observed Molecular Weight
- 38 kDa
- Fragment
- Rabbit IgG
- Specificity
- No cross reactivity with other proteins.
- Applications Notes
- Western blot, 0.1-0.5μg/ml, Human, Rat, Mouse<br>
- Subcellular Location
- Nucleus. Cytoplasmic vesicle. Primarily nuclear. Found also in endocytic vesicles in association with beta-adaptin.
- Sequence Similarities
- Belongs to the PI3/PI4-kinase family. ATM subfamily.
- Protein Name
- Serine-protein kinase ATM
