Aberrant Phase Separation of FUS Leads to Lysosome Sequestering and Acidification

Autor: Franziska Trnka, Christian Hoffmann, Han Wang, Roberto Sansevrino, Branislava Rankovic, Benjamin R. Rost, Dietmar Schmitz, H. Broder Schmidt, Dragomir Milovanovic

Publikováno v: Frontiers in Cell and Developmental Biology, Vol 9 (2021)

Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease that leads to the death of upper and lower motor neurons. While most cases of ALS are sporadic, some of the familial forms of the disease are caused by mutations in the ge

Externí odkaz: https://doaj.org/article/cb81296dec8f42cabb270272b88aecfb

TDP-43 represses cryptic exon inclusion in the FTD–ALS gene UNC13A

Autor: X. Rosa Ma, Mercedes Prudencio, Yuka Koike, Sarat C. Vatsavayai, Garam Kim, Fred Harbinski, Adam Briner, Caitlin M. Rodriguez, Caiwei Guo, Tetsuya Akiyama, H. Broder Schmidt, Beryl B. Cummings, David W. Wyatt, Katherine Kurylo, Georgiana Miller, Shila Mekhoubad, Nathan Sallee, Gemechu Mekonnen, Laura Ganser, Jack D. Rubien, Karen Jansen-West, Casey N. Cook, Sarah Pickles, Björn Oskarsson, Neill R. Graff-Radford, Bradley F. Boeve, David S. Knopman, Ronald C. Petersen, Dennis W. Dickson, James Shorter, Sua Myong, Eric M. Green, William W. Seeley, Leonard Petrucelli, Aaron D. Gitler

Publikováno v: Nature. 603:124-130

A hallmark pathological feature of the neurodegenerative diseases amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) is the depletion of RNA-binding protein TDP-43 from the nucleus of neurons in the brain and spinal cord1. A major

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_________::f758b8c837195288f92794e856595a83
https://doi.org/10.1038/s41586-022-04424-7

Aberrant Phase Separation of FUS Leads to Lysosome Sequestering and Acidification

Autor: Christian Hoffmann, Branislava Rankovic, Dietmar Schmitz, Benjamin R. Rost, Roberto Sansevrino, Franziska Trnka, H. Broder Schmidt, Dragomir Milovanovic, Han Wang

Publikováno v: Frontiers in Cell and Developmental Biology, Vol 9 (2021)
Frontiers in Cell and Developmental Biology
Frontiers in cell and developmental biology 9, 716919 (2021). doi:10.3389/fcell.2021.716919

Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease that leads to the death of upper and lower motor neurons. While most cases of ALS are sporadic, some of the familial forms of the disease are caused by mutations in the ge

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::8d92df18bfc37276d671bee0b6b2939a
https://www.frontiersin.org/articles/10.3389/fcell.2021.716919/full

Oxaliplatin disrupts nucleolar function through biophysical disintegration

Autor: H. Broder Schmidt, Zane A. Jaafar, B. Erik Wulff, Jason J. Rodencal, Kibeom Hong, Mohammad O. Aziz-Zanjani, Peter K. Jackson, Manuel D. Leonetti, Scott J. Dixon, Rajat Rohatgi, Onn Brandman

Publikováno v: Cell reports, vol 41, iss 6

Platinum (Pt) compounds such as oxaliplatin are among the most commonly prescribed anti-cancer drugs. Despite their considerable clinical impact, the molecular basis of platinum cytotoxicity and cancer specificity remain unclear. Here we show that ox

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::1feb3218ab03b38ddc4c3a3ca960a908
https://doi.org/10.1016/j.celrep.2022.111629

Transport Selectivity of Nuclear Pores, Phase Separation, and Membraneless Organelles

Autor: H. Broder Schmidt, Dirk Görlich

Publikováno v: Trends in Biochemical Sciences

Nuclear pore complexes (NPCs) provide a selective passageway for receptor-mediated active transport between nucleus and cytoplasm, while maintaining the distinct molecular compositions of both compartments at large. In this review we discuss how NPCs

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::222e81b7a3f467ffe7a1639599e8aae9
https://pubmed.ncbi.nlm.nih.gov/26705895