Citations 4
Poly(ADP-ribose) promotes toxicity of C9ORF72 arginine-rich dipeptide repeat proteins
Gao, Junli , Mewborne, Quinlan T. , Girdhar, Amandeep , Sheth, Udit , Coyne, Alyssa N. , Punathil, Ritika , Kang, Bong Gu , Dasovich, Morgan , Veire, Austin M. , Hernandez, Mariely DeJesus, Liu, Shuaichen , Shi, Zheng , Dafinca, Ruxandra , Talbot, Kevin , Zhang, Yong‐Jie , Petrucelli, Leonard , Blitterswijk, Marka van , Guo, Lin , Dawson, Ted M. , Veire, Austin , DeJesus Hernandez, Mariely, Fouquerel, Elise , Talbot, Kevin , Kam, Tae-In , Zhang, Yong-Jie , Dickson, Dennis W , Petrucelli, Leonard , van Blitterswijk, Marka , Guo, Lin , Dawson, Ted M , Dawson, Valina L , Leung, Anthony K L , Lloyd, Thomas E , Gendron, Tania F , Rothstein, Jeffrey D , Zhang, Ke , Coyne, Alyssa N , Sheth, Udit , Liu, Shuaichen, Kam, Tae‐In , Dickson, Dennis W. , Dawson, Valina L. , Gendron, Tania F. , Rothstein, Jeffrey D. , Zhang, Ke
Science Translational Medicine | September 14, 2022 | Volume: 14 | Issue: 662 | Pages: No page available
Arginine-rich dipeptide repeat proteins (R-DPRs), abnormal translational products of a GGGGCC hexanucleotide repeat expansion in C9ORF72 , play a critical role in C9ORF72 -related amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD), the most common genetic form of the disorders (c9ALS/FTD). R-DPRs form liquid condensates in vitro, induce stress granule formation in cultured cells, aggregate, and sometimes coaggregate with TDP-43 in postmortem tissue from patients with c9ALS/FTD. However, how these processes are regulated is unclear. Here, we show that loss of poly(ADP-ribose) (PAR) suppresses neurodegeneration in c9ALS/FTD fly models and neurons differentiated from patient-derived induced pluripotent stem cells. Mechanistically, PAR induces R-DPR condensation and promotes R-DPR–induced stress granule formation and TDP-43 aggregation. Moreover, PAR associates with insoluble R-DPR and TDP-43 in postmortem tissue from patients. These findings identified PAR as a promoter of R-DPR toxicity and thus a potential target for treating c9ALS/FTD.
IC‐02‐06: LONGITUDINAL CHANGES IN BRAIN MRI AND NEUROPSYCHOLOGICAL MEASURES IN ASYMPTOMATIC AND SYMPTOMATIC FAMILIAL FRONTOTEMPORAL LOBAR DEGENERATION WITH MUTATIONS IN MAPT
Weigand, Stephen D. , Przybelski, Scott A. , Graff‐Radford, Jonathan , Mathew, Senjem, Gunter, Jeffrey L. , Whitwell, Jennifer , Graff‐Radford, Neill R. , Josephs, Kieth, Fields, Julie A. , Lucas, John A. , Lowe, Val J. , Gavrilova, Ralitza, Kuntz, Karen M. , Hernandez, Mariely DeJesus, Rademakers, Rosa , Petersen, Ronald C. , Jones, David T. , Knopman, David S. , Wszołek, Zbigniew K. , Vemuri, Prashanthi , Machulda, Mary M. , Ferman, Tanis J. , Baker, Matthew , Kantarci, Kejal , Jack, Clifford R. , Boeve, Bradley F.
Alzheimer's & Dementia | September 6, 2014 | Volume: 10 | Issue: 4S_Part_1 | Pages: No page available
P1‐264: LONGITUDINAL CHANGES IN BRAIN MRI AND NEUROPSYCHOMETRICS IN ASYMPTOMATIC AND SYMPTOMATIC FAMILIAL FRONTOTEMPORAL LOBAR DEGENERATION WITH MUTATIONS IN MAPT
Weigand, Stephen D. , Przybelski, Scott A. , Graff‐Radford, Jonathan , Gunter, Jeffrey L. , Whitwell, Jennifer , Graff‐Radford, Neill R., Josephs, Keith A. , Fields, Julie A. , Lucas, John A. , Lowe, Val J. , Gavrilova, Ralitza H. , Karen, Kuntz, Hernandez, Mariely DeJesus, Rademakers, Rosa , Petersen, Ronald C. , Jones, David T. , Senjem, Matthew L. , Knopman, David S. , Graff‐Radford, Neill R., Wszołek, Zbigniew K. , Vemuri, Prashanthi , Machulda, Mary M. , Ferman, Tanis J. , Baker, Matthew , Kantarci, Kejal , Jack, Clifford R. , Boeve, Bradley F.
Alzheimer's & Dementia | September 6, 2014 | Volume: 10 | Issue: 4S_Part_10 | Pages: No page available
O3‐03‐05: LONGITUDINAL MRI AND NEUROPSYCHOLOGICAL CHANGES IN SYMPTOMATIC FRONTOTEMPORAL LOBAR DEGENERATION SUBJECTS WITH MUTATIONS IN MAPT, PGRN, AND C9ORF72
Graff‐Radford, Jonathan, Weigand, Stephen D. , Przybelski, Scott A. , Whitwell, Jennifer , Graff‐Radford, Neill R. , Josephs, Keith A. , Fields, Julie A. , Lucas, John A. , Lowe, Val J. , Gavrilova, Ralitza H. , Kuntz, Karen M. , Hernandez, Mariely DeJesus, Rademakers, Rosa , Petersen, Ronald C. , Graff‐Radford, Jonathan , Jones, David T. , Senjem, Matthew L. , Knopman, David S. , Wszołek, Zbigniew K. , Vemuri, Prashanthi , Ferman, Tanis J. , Baker, Matthew , Kantarci, Kejal , Jack, Clifford R. , Boeve, Bradley F.
Alzheimer's & Dementia | September 6, 2014 | Volume: 10 | Issue: 4S_Part_3 | Pages: No page available