Ontology highlight
ABSTRACT:
SUBMITTER: Gao R
PROVIDER: S-EPMC6529219 | biostudies-literature | 2019 Apr
REPOSITORIES: biostudies-literature
Gao Rui R Chakraborty Anirban A Geater Charlene C Pradhan Subrata S Gordon Kara L KL Snowden Jeffrey J Yuan Subo S Dickey Audrey S AS Choudhary Sanjeev S Ashizawa Tetsuo T Ellerby Lisa M LM La Spada Albert R AR Thompson Leslie M LM Hazra Tapas K TK Sarkar Partha S PS
eLife 20190417
How huntingtin (HTT) triggers neurotoxicity in Huntington's disease (HD) remains unclear. We report that HTT forms a transcription-coupled DNA repair (TCR) complex with RNA polymerase II subunit A (POLR2A), ataxin-3, the DNA repair enzyme polynucleotide-kinase-3'-phosphatase (PNKP), and cyclic AMP-response element-binding (CREB) protein (CBP). This complex senses and facilitates DNA damage repair during transcriptional elongation, but its functional integrity is impaired by mutant HTT. Abrogated ...[more]