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James Shorter

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Articles 185
Citations 12797
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Recent Articles
1.
Aikio M, Odeh H, Wobst H, Lee B, Chan U, Mauna J, et al.
Cell Rep . 2025 Feb; 44(3):115386. PMID: 39977265
No abstract available.
2.
Aikio M, Odeh H, Wobst H, Lee B, Chan U, Mauna J, et al.
Cell Rep . 2025 Jan; 44(1):115205. PMID: 39817908
Amyotrophic lateral sclerosis (ALS) is a devastating neurodegenerative disorder typically characterized by insoluble inclusions of hyperphosphorylated TDP-43. The mechanisms underlying toxic TDP-43 accumulation are not understood. Persistent activation of p38...
3.
Kim K, Girdhar A, Cicardi M, Kankate V, Hayashi M, Yang R, et al.
Commun Biol . 2025 Jan; 8(1):2. PMID: 39747573
Arginine-rich dipeptide repeat proteins (R-DPRs) are highly toxic proteins found in patients with C9orf72-linked amyotrophic lateral sclerosis and frontotemporal dementia (C9-ALS/FTD). R-DPRs can cause toxicity by disrupting the natural phase...
4.
Lin J, Carman P, Gambogi C, Kendsersky N, Chuang E, Gates S, et al.
Cell Rep . 2024 Dec; 43(12):115005. PMID: 39671291
The hexameric AAA+ disaggregase, Hsp104, collaborates with Hsp70 and Hsp40 via its autoregulatory middle domain (MD) to solubilize aggregated proteins. However, how ATP- or ADP-specific MD configurations regulate Hsp104 hexamers...
5.
Buchholz H, Dorweiler J, Guereca S, Wisniewski B, Shorter J, Manogaran A
PLoS Genet . 2024 Oct; 20(10):e1011424. PMID: 39361717
Molecular chaperones play a central role in protein disaggregation. However, the molecular determinants that regulate this process are poorly understood. Hsp104 is an AAA+ ATPase that disassembles stress granules and...
6.
Vazquez-Sanchez S, Tilkin B, Gasset-Rosa F, Zhang S, Piol D, McAlonis-Downes M, et al.
bioRxiv . 2024 Jun; PMID: 38895337
RNA binding proteins have emerged as central players in the mechanisms of many neurodegenerative diseases. In particular, a proteinopathy of fu sed in s arcoma (FUS) is present in some...
7.
Vazquez-Sanchez S, Tilkin B, Gasset-Rosa F, Zhang S, Piol D, McAlonis-Downes M, et al.
Mol Neurodegener . 2024 Jun; 19(1):46. PMID: 38862967
RNA binding proteins have emerged as central players in the mechanisms of many neurodegenerative diseases. In particular, a proteinopathy of fused in sarcoma (FUS) is present in some instances of...
8.
Lin J, Carman P, Gambogi C, Kendsersky N, Chuang E, Gates S, et al.
bioRxiv . 2024 May; PMID: 38712168
The hexameric AAA+ disaggregase, Hsp104, collaborates with Hsp70 and Hsp40 via its autoregulatory middle domain (MD) to solubilize aggregated protein conformers. However, how ATP- or ADP-specific MD configurations regulate Hsp104...
9.
Sweeney K, Chantarawong S, Barbieri E, Cajka G, Liu M, Spruce L, et al.
PLoS Genet . 2024 Feb; 20(2):e1011138. PMID: 38315730
The presence of large protein inclusions is a hallmark of neurodegeneration, and yet the precise molecular factors that contribute to their formation remain poorly understood. Screens using aggregation-prone proteins have...
10.
Khalil B, Linsenmeier M, Smith C, Shorter J, Rossoll W
Mol Neurodegener . 2024 Jan; 19(1):8. PMID: 38254150
Amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) are fatal neurodegenerative disorders on a disease spectrum that are characterized by the cytoplasmic mislocalization and aberrant phase transitions of prion-like RNA-binding...