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(ataxia telangiectasia)
9,851 results
  • Proximal tubule ATR regulates DNA repair to prevent maladaptive renal injury responses. [Journal Article]
    J Clin Invest 2019Kishi S, Brooks CR, … Bonventre JV
  • Maladaptive proximal tubule (PT) repair has been implicated in kidney fibrosis through induction of cell-cycle arrest at G2/M. We explored the relative importance of the PT DNA damage response (DDR) in kidney fibrosis by genetically inactivating ataxia telangiectasia and Rad3-related (ATR), which is a sensor and upstream initiator of the DDR. In human chronic kidney disease, ATR expression invers…
  • Regulation of the Notch-ATM-abl axis by geranylgeranyl diphosphate synthase inhibition. [Journal Article]
    Cell Death Dis 2019; 10(10):733Agabiti SS, Li J, … Wiemer AJ
  • Notch proteins drive oncogenesis of many cancers, most prominently T-cell acute lymphoblastic leukemia (T-ALL). Because geranylgeranylated Rab proteins regulate Notch processing, we hypothesized that inhibition of geranylgeranyl diphosphate synthase (GGDPS) would impair Notch processing and reduce viability of T-ALL cells that express Notch. Here, we show that GGDPS inhibition reduces Notch1 expr…
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