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James Shorter

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Articles 185
Citations 12797
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Recent Articles
21.
Fare C, Rhine K, Lam A, Myong S, Shorter J
J Biol Chem . 2022 Dec; 299(2):102806. PMID: 36529289
Karyopherin-β2 (Kapβ2) is a nuclear-import receptor that recognizes proline-tyrosine nuclear localization signals of diverse cytoplasmic cargo for transport to the nucleus. Kapβ2 cargo includes several disease-linked RNA-binding proteins with prion-like...
22.
Khalil B, Chhangani D, Wren M, Smith C, Lee J, Li X, et al.
Mol Neurodegener . 2022 Dec; 17(1):80. PMID: 36482422
Background: Cytoplasmic mislocalization and aggregation of TAR DNA-binding protein-43 (TDP-43) is a hallmark of the amyotrophic lateral sclerosis and frontotemporal dementia (ALS/FTD) disease spectrum, causing both nuclear loss-of-function and cytoplasmic...
23.
Weil T, Kirupakaran A, Le M, Rebmann P, Mieres-Perez J, Issmail L, et al.
JACS Au . 2022 Oct; 2(9):2187-2202. PMID: 36186568
The COVID-19 pandemic caused by SARS-CoV-2 presents a global health emergency. Therapeutic options against SARS-CoV-2 are still very limited but urgently required. Molecular tweezers are supramolecular agents that destabilize the...
24.
Cupo R, Rizo A, Braun G, Tse E, Chuang E, Gupta K, et al.
Cell Rep . 2022 Sep; 40(13):111408. PMID: 36170828
The AAA+ protein, Skd3 (human CLPB), solubilizes proteins in the mitochondrial intermembrane space, which is critical for human health. Skd3 variants with defective protein-disaggregase activity cause severe congenital neutropenia (SCN)...
25.
Shen H, Yanas A, Owens M, Zhang C, Fritsch C, Fare C, et al.
Mol Cell . 2022 May; 82(14):2588-2603.e9. PMID: 35588748
Sex differences are pervasive in human health and disease. One major key to sex-biased differences lies in the sex chromosomes. Although the functions of the X chromosome proteins are well...
26.
Francois-Moutal L, Scott D, Ambrose A, Zerio C, Rodriguez-Sanchez M, Dissanayake K, et al.
Sci Rep . 2022 May; 12(1):8140. PMID: 35581326
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease with no cure or effective treatment in which TAR DNA Binding Protein of 43 kDa (TDP-43) abnormally accumulates into misfolded protein...
27.
Peinado J, Chaplot K, Jarvela T, Barbieri E, Shorter J, Lindberg I
ACS Chem Neurosci . 2022 May; 13(11):1651-1665. PMID: 35549000
As neurons age, protein homeostasis becomes less efficient, resulting in misfolding and aggregation. Chaperone proteins perform vital functions in the maintenance of cellular proteostasis, and chaperone-based therapies that promote sequestration...
28.
Kim H, Mohassel P, Donkervoort S, Guo L, ODonovan K, Coughlin M, et al.
Nat Commun . 2022 Apr; 13(1):2306. PMID: 35484142
Missense variants in RNA-binding proteins (RBPs) underlie a spectrum of disease phenotypes, including amyotrophic lateral sclerosis, frontotemporal dementia, and inclusion body myopathy. Here, we present ten independent families with a...
29.
Copley K, Shorter J
Cell . 2022 Apr; 185(8):1290-1292. PMID: 35427496
Neurodegenerative diseases commonly exhibit aggregation of specific proteins that define each disease. Chang et al. (2022) establish that a C-terminal fragment of TMEM106B, a frontotemporal-lobar-degeneration risk factor, unexpectedly forms amyloid...
30.
Lin J, Shorter J, Lucius A
Biochem Soc Trans . 2022 Mar; 50(2):895-906. PMID: 35356966
Numerous ATPases associated with diverse cellular activities (AAA+) proteins form hexameric, ring-shaped complexes that function via ATPase-coupled translocation of substrates across the central channel. Cryo-electron microscopy of AAA+ proteins processing...