Zobrazeno 1 - 10
of 21
pro vyhledávání: '"Laura C, Bott"'
Autor:
Akira Kitamura, Ai Fujimoto, Rei Kawashima, Yidan Lyu, Kotetsu Sasaki, Yuta Hamada, Kanami Moriya, Ayumi Kurata, Kazuho Takahashi, Reneé Brielmann, Laura C. Bott, Richard I. Morimoto, Masataka Kinjo
Publikováno v:
Communications Biology, Vol 7, Iss 1, Pp 1-21 (2024)
Abstract Carboxy terminal fragments (CTFs) of TDP-43 contain an intrinsically disordered region (IDR) and form cytoplasmic condensates containing amyloid fibrils. Such condensates are toxic and associated with pathogenicity in amyotrophic lateral scl
Externí odkaz:
https://doaj.org/article/edf11e8a7f974a75b2d3e0e0a54b502f
Autor:
Ambre J. Sala, Rogan A. Grant, Ghania Imran, Claire Morton, Renee M. Brielmann, Laura C. Bott, Jennifer Watts, Richard I. Morimoto
The ability to sense and respond to proteotoxic insults declines with age, leaving cells vulnerable to chronic and acute stressors. Reproductive cues modulate this decline in cellular proteostasis to influence organismal stress resilience inC. elegan
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_________::abb8aaa76c9f520b2b940b5c37c40346
https://doi.org/10.1101/2023.04.25.537803
https://doi.org/10.1101/2023.04.25.537803
Autor:
Christopher Grunseich, Kristen Zukosky, Ilona R. Kats, Laboni Ghosh, George G. Harmison, Laura C. Bott, Carlo Rinaldi, Ke-lian Chen, Guibin Chen, Manfred Boehm, Kenneth H. Fischbeck
Publikováno v:
Neurobiology of Disease, Vol 70, Iss , Pp 12-20 (2014)
Spinal and bulbar muscular atrophy (SBMA, Kennedy's disease) is a motor neuron disease caused by polyglutamine repeat expansion in the androgen receptor. Although degeneration occurs in the spinal cord and muscle, the exact mechanism is not clear. In
Externí odkaz:
https://doaj.org/article/3ab00d5a56dd44d8848d952bcc5d6dc8
Autor:
Laura C, Bott, Mitra, Forouhan, Maria, Lieto, Ambre J, Sala, Ruth, Ellerington, Janel O, Johnson, Alfina A, Speciale, Chiara, Criscuolo, Alessandro, Filla, David, Chitayat, Ebba, Alkhunaizi, Patrick, Shannon, Andrea H, Nemeth, Francesco, Angelucci, Wooi Fang, Lim, Pasquale, Striano, Federico, Zara, Ingo, Helbig, Mikko, Muona, Carolina, Courage, Anna-Elina, Lehesjoki, Samuel F, Berkovic, Kenneth H, Fischbeck, Francesco, Brancati, Richard I, Morimoto, Matthew J A, Wood, Carlo, Rinaldi
Publikováno v:
Brain Communications
The vacuolar H+-ATPase is a large multi-subunit proton pump, composed of an integral membrane V0 domain, involved in proton translocation, and a peripheral V1 domain, catalysing ATP hydrolysis. This complex is widely distributed on the membrane of va
Autor:
Wooi F. Lim, Richard I. Morimoto, Kenneth H. Fischbeck, Carolina Courage, Forouhan M, Ingo Helbig, Janel O. Johnson, Angelucci F, Laura C. Bott, Ruth Ellerington, Federico Zara, Maria Lieto, Francesco Brancati, Nemeth Ah, Mikko Muona, Matthew J.A. Wood, Pasquale Striano, Alfina A Speciale, Chiara Criscuolo, David Chitayat, Ambre Sala, Samuel F. Berkovic, A. Filla, Carlo Rinaldi, A E Lehesjoki
The vacuolar H+-ATPase is a large multi-subunit proton pump, composed of an integral membrane V0 domain, involved in proton translocation, and a peripheral V1 domain, catalysing ATP hydrolysis. This complex is widely distributed on the membrane of va
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_________::18dddc04cd85202f8a01172ada191fa0
https://doi.org/10.1101/2021.06.01.21257500
https://doi.org/10.1101/2021.06.01.21257500
Autor:
Nico P. Dantuma, Laura C Bott
Publikováno v:
Frontiers in Molecular Neuroscience, Vol 7 (2014)
The ubiquitin-proteasome system (UPS) has been implicated in neurodegenerative diseases based on the presence of deposits consisting of ubiquitylated proteins in affected neurons. It has been postulated that aggregation-prone proteins associated with
Externí odkaz:
https://doaj.org/article/dec3b381594945d5984608b379e4849b
Publikováno v:
Genes Dev
The proteostasis network is regulated by transcellular communication to promote health and fitness in metazoans. In Caenorhabditis elegans, signals from the germline initiate the decline of proteostasis and repression of cell stress responses at repr
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::d7f76e56483067c4e12e3d0643f52f85
https://europepmc.org/articles/PMC7197353/
https://europepmc.org/articles/PMC7197353/
Publikováno v:
The Journal of Cell Biology
This review by Morimoto and colleagues examines mechanisms by which protein homeostasis (proteostasis) is achieved in multicellular organisms and discusses the implications for health and disease.
The proteostasis network (PN) regulates protein
The proteostasis network (PN) regulates protein
Autor:
Laura C. Bott, J. Paul Taylor, Masahisa Katsuno, George G. Harmison, Nisha M. Badders, Elaine Bautista, Nico P. Dantuma, Charles C.-Y. Shih, Carlo Rinaldi, Ke-lian Chen, Kenneth H. Fischbeck, Gen Sobue
Publikováno v:
Human Molecular Genetics. 25:1979-1989
Spinal and bulbar muscular atrophy (SBMA, also known as Kennedy's disease) is one of nine neurodegenerative disorders that are caused by expansion of polyglutamine-encoding CAG repeats. Intracellular accumulation of abnormal proteins in these disease
Autor:
Carlo Rinaldi, Barrington G. Burnett, Laura C. Bott, Gen Sobue, Ke-lian Chen, Naemeh Pourshafie, Kenneth H. Fischbeck, Philip R. Lee, Masahisa Katsuno, George G. Harmison
Publikováno v:
Molecular Therapy
Spinal and bulbar muscular atrophy (SBMA) is a currently untreatable adult-onset neuromuscular disease caused by expansion of a polyglutamine repeat in the androgen receptor (AR). In SBMA, as in other polyglutamine diseases, a toxic gain of function