R
Rodrigo Lopez-Gonzalez
Researcher at University of Massachusetts Medical School
Publications - 13
Citations - 1774
Rodrigo Lopez-Gonzalez is an academic researcher from University of Massachusetts Medical School. The author has contributed to research in topics: Trinucleotide repeat expansion & C9orf72. The author has an hindex of 10, co-authored 12 publications receiving 1286 citations.
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Journal ArticleDOI
GGGGCC repeat expansion in C9orf72 compromises nucleocytoplasmic transport
Brian D. Freibaum,Yubing Lu,Rodrigo Lopez-Gonzalez,Nam Chul Kim,Sandra Almeida,Kyung-Ha Lee,Nisha M. Badders,Marc Valentine,Bruce L. Miller,Philip C. Wong,Leonard Petrucelli,Hong Joo Kim,Fen-Biao Gao,J. Paul Taylor +13 more
TL;DR: It is shown that a primary consequence of G4C2 repeat expansion is the compromise of nucleocytoplasmic transport through the nuclear pore, revealing a novel mechanism of neurodegeneration.
Journal ArticleDOI
Poly(GR) in C9ORF72-Related ALS/FTD Compromises Mitochondrial Function and Increases Oxidative Stress and DNA Damage in iPSC-Derived Motor Neurons.
Rodrigo Lopez-Gonzalez,Yubing Lu,Tania F. Gendron,Anna Karydas,Helene Tran,Dejun Yang,Leonard Petrucelli,Bruce L. Miller,Sandra Almeida,Fen-Biao Gao +9 more
TL;DR: It is found that DNA damage was greater, in an age-dependent manner, in motor neurons differentiated from iPSCs of multiple C9ORF72 patients than control neurons, revealing another pathogenic mechanism in C9 ORF72-related ALS and FTD.
Journal ArticleDOI
C9ORF72 GGGGCC repeat-associated non-AUG translation is upregulated by stress through eIF2α phosphorylation.
Weiwei Cheng,Shaopeng Wang,Alexander A. Mestre,Chenglai Fu,Andres Makarem,Fengfan Xian,Lindsey R. Hayes,Rodrigo Lopez-Gonzalez,Kevin Drenner,Jie Jiang,Don W. Cleveland,Shuying Sun +11 more
TL;DR: It is demonstrated that the repeat-associated non-AUG (RAN) translation of (GGGGCC)n-containing RNAs into poly-dipeptides can initiate in vivo without a 5′-cap.
Journal ArticleDOI
Dysregulated molecular pathways in amyotrophic lateral sclerosis-frontotemporal dementia spectrum disorder.
TL;DR: Intensive studies reveal the complexity of the pathogenic mechanisms of FTD and ALS and suggest promising molecular targets for future therapeutic interventions in these devastating disorders.
Journal ArticleDOI
C9ORF72-ALS/FTD-associated poly(GR) binds Atp5a1 and compromises mitochondrial function in vivo.
So Yoen Choi,Rodrigo Lopez-Gonzalez,Gopinath Krishnan,Hannah L. Phillips,Alissa Nana Li,William W. Seeley,Wei-Dong Yao,Sandra Almeida,Fen-Biao Gao +8 more
TL;DR: The authors generated a mouse model of C9ORF72-related amyotrophic lateral sclerosis and frontotemporal dementia based on dipeptide repeat proteins and report that defects in mitochondria may contribute to disease pathogenesis.