Dysregulation of upstream binding factor-1 acetylation at K352 is linked to impaired ribosomal DNA transcription in Huntington's disease

@article{Lee2011DysregulationOU,
  title={Dysregulation of upstream binding factor-1 acetylation at K352 is linked to impaired ribosomal DNA transcription in Huntington's disease},
  author={J Lee and Yong Jin Hwang and Jung Hyun Boo and Deqiang Han and Oh Kwang Kwon and Krasimira Todorova and Neil W. Kowall and Young-Joo Kim and H Ryu},
  journal={Cell Death and Differentiation},
  year={2011},
  volume={18},
  pages={1726-1735}
}
Huntington's disease (HD) is an autosomal-dominant neurological disorder caused by expanded CAG repeats in the Huntingtin (Htt) gene, but it is not known how this mutation causes neurodegeneration. Herein, we found that dysfunction of upstream binding factor-1 (UBF-1) is linked to reduced ribosomal DNA (rDNA) transcription in HD. We identified that UBF1 acetylation at Lys (K) 352 by CREB binding protein (CBP) is crucial for the transcriptional activity of rDNA. UBF1 mutation (K352A, K352Q, and… CONTINUE READING

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