A mild PUM1 mutation is associated with adult-onset ataxia, whereas haploinsufficiency causes developmental delay and seizures VA Gennarino, EE Palmer, LM McDonell, L Wang, CJ Adamski, A Koire, ... Cell 172 (5), 924-936. e11, 2018 | 126 | 2018 |
ATXN1-CIC complex is the primary driver of cerebellar pathology in spinocerebellar ataxia type 1 through a gain-of-function mechanism MWC Rousseaux, T Tschumperlin, HC Lu, EP Lackey, VV Bondar, ... Neuron 97 (6), 1235-1243. e5, 2018 | 104 | 2018 |
CLN8 is an endoplasmic reticulum cargo receptor that regulates lysosome biogenesis A di Ronza, L Bajaj, J Sharma, D Sanagasetti, P Lotfi, CJ Adamski, ... Nature cell biology 20 (12), 1370-1377, 2018 | 93 | 2018 |
Deep sequencing of systematic combinatorial libraries reveals β-lactamase sequence constraints at high resolution Z Deng, W Huang, E Bakkalbasi, NG Brown, CJ Adamski, K Rice, ... Journal of molecular biology 424 (3-4), 150-167, 2012 | 87 | 2012 |
An autism-linked missense mutation in SHANK3 reveals the modularity of Shank3 function L Wang, K Pang, K Han, CJ Adamski, W Wang, L He, JK Lai, VV Bondar, ... Molecular psychiatry 25 (10), 2534-2555, 2020 | 70 | 2020 |
Molecular basis for the catalytic specificity of the CTX-M extended-spectrum β-lactamases CJ Adamski, AM Cardenas, NG Brown, LB Horton, B Sankaran, ... Biochemistry 54 (2), 447-457, 2015 | 64 | 2015 |
RBM17 interacts with U2SURP and CHERP to regulate expression and splicing of RNA-processing proteins A De Maio, HK Yalamanchili, CJ Adamski, VA Gennarino, Z Liu, J Qin, ... Cell reports 25 (3), 726-736. e7, 2018 | 59 | 2018 |
Reduction of protein kinase A-mediated phosphorylation of ATXN1-S776 in Purkinje cells delays onset of Ataxia in a SCA1 mouse model JMP Ortiz, N Mollema, N Toker, CJ Adamski, B O'Callaghan, L Duvick, ... Neurobiology of disease 116, 93-105, 2018 | 32 | 2018 |
A kinome-wide RNAi screen identifies ERK2 as a druggable regulator of Shank3 stability L Wang, CJ Adamski, VV Bondar, E Craigen, JR Collette, K Pang, K Han, ... Molecular psychiatry 25 (10), 2504-2516, 2020 | 26 | 2020 |
Removal of the side chain at the active-site serine by a glycine substitution increases the stability of a wide range of serine β-lactamases by relieving steric strain V Stojanoski, CJ Adamski, L Hu, SC Mehta, B Sankaran, P Zwart, ... Biochemistry 55 (17), 2479-2490, 2016 | 26 | 2016 |
PAK1 regulates ATXN1 levels providing an opportunity to modify its toxicity in spinocerebellar ataxia type 1 VV Bondar, CJ Adamski, TS Onur, Q Tan, L Wang, J Diaz-Garcia, J Park, ... Human molecular genetics 27 (16), 2863-2873, 2018 | 24 | 2018 |
Synergistic effects of functionally distinct substitutions in β-lactamase variants shed light on the evolution of bacterial drug resistance MP Patel, L Hu, CA Brown, Z Sun, CJ Adamski, V Stojanoski, B Sankaran, ... Journal of Biological Chemistry 293 (46), 17971-17984, 2018 | 23 | 2018 |
Inhibition of ERK1/2 restores GSK3β activity and protein synthesis levels in a model of tuberous sclerosis R Pal, VV Bondar, CJ Adamski, GG Rodney, M Sardiello Scientific reports 7 (1), 4174, 2017 | 20 | 2017 |
Doublecortin-like kinase 1 regulates α-synuclein levels and toxicity GE Vázquez-Vélez, KA Gonzales, JP Revelli, CJ Adamski, FA Naini, ... Journal of Neuroscience 40 (2), 459-477, 2020 | 19 | 2020 |
Role of β‐lactamase residues in a common interface for binding the structurally unrelated inhibitory proteins BLIP and BLIP‐II BG Fryszczyn, CJ Adamski, NG Brown, K Rice, W Huang, T Palzkill Protein Science 23 (9), 1235-1246, 2014 | 15 | 2014 |
Deep sequencing of random mutant libraries reveals the active site of the narrow specificity CphA metallo-β-lactamase is fragile to mutations Z Sun, SC Mehta, CJ Adamski, RA Gibbs, T Palzkill Scientific Reports 6 (1), 33195, 2016 | 14 | 2016 |
Dual targeting of brain region‐specific kinases potentiates neurological rescue in Spinocerebellar ataxia type 1 WS Lee, L Lavery, MWC Rousseaux, EB Rutledge, Y Jang, YW Wan, ... The EMBO journal 40 (7), e106106, 2021 | 11 | 2021 |
Cross-species genetic screens identify transglutaminase 5 as a regulator of polyglutamine-expanded ataxin-1 WS Lee, I Al-Ramahi, HH Jeong, Y Jang, T Lin, CJ Adamski, LA Lavery, ... The Journal of clinical investigation 132 (9), 2022 | 7 | 2022 |
A structural study of the cytoplasmic chaperone effect of 14-3-3 proteins on ataxin-1 S Leysen, RJ Burnley, E Rodriguez, LG Milroy, L Soini, CJ Adamski, ... Journal of molecular biology 433 (19), 167174, 2021 | 7 | 2021 |
Systematic substitutions at BLIP position 50 result in changes in binding specificity for class A β-lactamases CJ Adamski, T Palzkill BMC biochemistry 18, 1-11, 2017 | 7 | 2017 |