Apilimod dimesylate in C9orf72 amyotrophic lateral sclerosis: a randomized phase 2a clinical trial.

Autor: Babu, Suma, Nicholson, Katharine A, Rothstein, Jeffrey D, Swenson, Andrea, Sampognaro, Paul J, Pant, Pravin, Macklin, Eric A, Spruill, Susan, Paganoni, Sabrina, Gendron, Tania F, Prudencio, Mercedes, Petrucelli, Leonard, Nix, Darrell, Landrette, Sean, Nkrumah, Esther, Fandrick, Keith, Edwards, Joan, Young, Peter R

Publikováno v: Brain: A Journal of Neurology; Sep2024, Vol. 147 Issue 9, p2998-3008, 11p

Processing of progranulin into granulins involves multiple lysosomal proteases and is affected in frontotemporal lobar degeneration.

Autor: Mohan, Swetha¹ (AUTHOR), Sampognaro, Paul J.¹ (AUTHOR), Argouarch, Andrea R.¹ (AUTHOR), Maynard, Jason C.² (AUTHOR), Welch, Mackenzie¹ (AUTHOR), Patwardhan, Anand¹ (AUTHOR), Courtney, Emma C.¹ (AUTHOR), Zhang, Jiasheng³ (AUTHOR), Mason, Amanda¹ (AUTHOR), Li, Kathy H.² (AUTHOR), Huang, Eric J.³ (AUTHOR), Seeley, William W.¹ (AUTHOR), Miller, Bruce L.¹ (AUTHOR), Burlingame, Alma² (AUTHOR), Jacobson, Mathew P.² (AUTHOR), Kao, Aimee W.¹ (AUTHOR) aimee.kao@ucsf.edu

Publikováno v: Molecular Neurodegeneration. 8/3/2021, Vol. 16 Issue 1, p1-18. 18p.

Additional file 1 of Mutations in α-synuclein, TDP-43 and tau prolong protein half-life through diminished degradation by lysosomal proteases

Autor: Sampognaro, Paul J., Arya, Shruti, Knudsen, Giselle M., Gunderson, Emma L., Sandoval-Perez, Angelica, Hodul, Molly, Bowles, Katherine, Craik, Charles S., Jacobson, Matthew P., Kao, Aimee W.

Additional file 1: Figure S1. Proteases that do not cleave α-syn, TDP-43, or tau are active against a fluorogenic casein substrate. Fluorogenic protease activity assays using casein at pH 4.5 was performed on all enzymes that do not cleave α-syn, T

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::cc448f1361abd0c81227ae7cfd50104f

Additional file 1 of Processing of progranulin into granulins involves multiple lysosomal proteases and is affected in frontotemporal lobar degeneration

Autor: Mohan, Swetha, Sampognaro, Paul J., Argouarch, Andrea R., Maynard, Jason C., Welch, Mackenzie, Patwardhan, Anand, Courtney, Emma C., Zhang, Jiasheng, Mason, Amanda, Li, Kathy H., Huang, Eric J., Seeley, William W., Miller, Bruce L., Burlingame, Alma, Jacobson, Mathew P., Kao, Aimee W.

Additional file 1 Fig. S1. Enzymes that do not cleave PGRN are biologically active. a, 1 μM of mature CTSD was incubated with 400 ng recombinant human PGRN or 400 ng BSA at the indicated pH. The incubation was stopped after 60 min or 16 h, and react

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::482e98f8b5653ac8fe85bcf3ec0d2548

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