McMillan M*, Gomez N*, Hsieh C, Bekier M, Li X, Miguez R, Tank EM, Barmada SJ. RNA methylation influences TDP43 binding and disease pathogenesis in models of amyotrophic lateral sclerosis and frontotemporal dementia. Molecular Cell. 2023 Jan 4;S1097-2765(22)01176-5. doi: 10.1016/j.molcel.2022.12.019.
|
Malik AM, Wu JJ, Gillies CA, Doctrove QA, Li X, Huang H, Tank EM, Shakkottai VG, Barmada SJ. Neuronal activity regulates Matrin 3 levels and function in a calcium-dependent manner through calpain cleavage and calmodulin binding. Proceedings of the National Academy of Sciences. 2023 April 3 120 (15) e2206217120
|
Krishnan G, Raitcheva D, Bartlett D, Prudencio M, McKenna-Yasek D, Douthwright C, Oskarsson B, Fiorelli R, King O, Barmada SJ, Miller T, Bowser R, Watts J, Petrucelli L, Brown R, Kankel M, and Gao F-B. Poly(GR) and Poly(GA) in Cerebrospinal Fluid as Potential Biomarkers for C9ORF72-ALS/FTD. Nature Communications. 2022. May 19;13(1):2799. doi: 10.1038/s41467-022-30387-4.
|
Chua C, Bedi K, Paulsen MT, Ljungman M, Tank EM, Kim ES, Colón-Mercado JM, Ward ME, Weisman LS, Santoro N, Barmada SJ. Myotubularin-related phosphatase 5 is a critical determinant of autophagy in neurons. Current Biology. 2022. Jun 20;32(12):2581-2595.e6
|
François-Moutal L, Scott DD, Dissanayake K, May DG, Carlson JM, Ambrose AJ, Zerio CJ, Barbieri E, Moutal A, Chapman E, Roux KJ, Shorter J, Khanna R, Barmada SJ, McGurk L, Khanna M. Heat shock protein Grp78/BiP/HspA5 binds directly to TDP-43 and mitigates toxicity associated with disease pathology. Scientific Reports. 2022. May 17;12(1):8140.
|
Safren N, Tank EM, Malik M, Chua J, Santoro N, Barmada SJ. Development of a specific live-cell assay for native autophagic flux. Journal of Biochemistry. 2021 Sep;297(3):101003. doi: 10.1016/j.jbc.2021.101003.
|
Sidibé H, Khalfallah Y, Xiao S, Gómez NB, Tank EMH, Di Tomasso G, Bareke E, Aulas A, McKeever P, Melamed Z, Destroimaisons L, Deshaies J-E, Zinman L, Parker JA, Legault P, Tétreault M, Barmada SJ, Robertson J, and Vande Velde C. Transcripts encoding the central stress granule protein G3BP1 are stabilized by TDP-43: potential relevance to ALS/FTD. Brain 2021 Dec 16;144(11):3461-3476.
doi: 10.1093/brain/awab217 |
Maimon R*, Ankol L*, Altman T, Ionescu A, Weissova R, Gradus TP, Ostrovsky M, Tank EM, Alexandra G, Shelestovich N, Opatowsky Y, Dori A, Barmada SJ, Balastik M, Perlson E. CRMP4-Dynein Complex Formation Mediate Retrograde Death Signal in ALS. European Molecular Biology (EMBO) Journal 2021 Sep 1;40(17):e107586. doi: 10.15252/embj.2020107586
|
Chua J, de Calbiac H, Kabashi E, Barmada SJ. Autophagy and ALS: Mechanistic Insights and Therapeutic Implications. Autophagy 2021 May 31;1-29. doi: 10.1080/15548627.2021.1926656
|
Safren N, Sharkey L, Barmada SJ. Neuronal puncta/aggregate formation by WT and mutant UBQLN2. Methods in Molecular Biology. 2023;2551:561-573. doi: 10.1007/978-1-0716-2597-2_34.
|
Moinuddin O, Khandwala NS, Young KZ, Sathrasala SK, Barmada SJ, Albin R, and Besirli CG. The Role of Optical Coherence Tomography in Identifying Retinal Biomarkers in FTD: A Review. Neurology: Clinical Practice. January 25, 2021, DOI: https://doi.org/10.1212/CPJ.0000000000001041
|
Malik A and Barmada SJ. Matrin 3 in neuromuscular disease: physiology and pathophysiology. Journal of Clinical Investigation Insight. 2021 Jan 11; 6(1): e143948.
|
Mollasalehi N, Francois-Moutal L, Scott D, Tello J, Williams H, Mahoney B, Carlson J, Dong Y, Li X, Miranda V, Gokhale V, Wang W, Barmada SJ, Khanna M. An allosteric modulator of RNA binding targeting the N-terminal domain of TDP-43 yields neuroprotective properties. ACS Chemical Biology. 2020 Nov 20;15(11):2854-2859.
|
Gerson JE, Safren N, Fischer S, Patel R, Crowley EV, Welday JP, Windle AK, Barmada SJ, Paulson HL, Sharkey. Ubiquilin-2 differentially regulates polyglutamine disease proteins. Human Molecular Genetics 29(15):2596.
|
He F, Flores BN, Krans A, Frazer M, Natla S, Niraula S, Adefioye O, Barmada SJ, Todd PK. The carboxyl termini of RAN translated GGGGCC nucleotide repeat expansions modulate toxicity in models of ALS/FTD. Acta Neuropathologica Communications. 2020 Aug 4;8(1):122.
|
Malik AM, Barmada SJ. TDP-43 Nuclear Bodies: A NEAT response to stress? Molecular Cell. 2020 Aug 6;79(3):362-364.
|
Rodriguez CM, Wright SE, Kearse MG, Haenfler J, Flores BN, Liu Y, Ifrim MF, Glineburg MR, Krans A, Paymaan JN, Sutton M, Bassell GJ, Parent JM, Rigo F, Barmada SJ, Todd PK. A native function for RAN translation and CGG repeats in regulating fragile X protein synthesis. Nature Neuroscience. 2020 23, 386–397.
|
Weskamp K, Tank EM, Miguez R, McBride JP, Gómez NB, White M, Lin Z, Moreno Gonzalez C, Serio A, Sreedharan J, Barmada SJ. Shortened TDP43 isoforms upregulated by neuronal hyperactivity drive TDP43 pathology in ALS. Journal of Clinical Investigation. 2020 Mar 2;130(3):1139-1155.
|
Green KM, Sheth U, Flores BN, Wright SE, Sutter A, Kearse MG, Barmada SJ, Ivanova MI, Todd PK. High-throughput screening yields several small-molecule inhibitors of repeat-associated non-AUG translation. Journal of Biological Chemistry 2019 Oct 23. pii: jbc.RA119.009951.
|
Linsalata AE, He F, Malik AM, Glineburg MR, Green KM, Natla S, Flores BN, Krans A, Archbold HC, Fedak SJ, Barmada SJ, Todd PK. DDX3X and specific initiation factors modulate FMR1 repeat-associated non-AUG-initiated translation. EMBO Reports 2019 Sep;20(9):e47498.
|
Flores BN, Malik A, Li X, Martinez Jose, Beg AA, and Barmada SJ. An Intramolecular Salt Bridge Linking TDP43's RNA Recognition Motifs Dictates RNA Binding, Protein Stability and TDP43-Dependent Neurodegeneration. Cell Reports 2019, 27:1133-1150.
|
Weskamp K*, Safren N*, Miguez R, and Barmada SJ. Monitoring neuronal survival via longitudinal fluorescence microscopy. Journal of Visualized Experiments 2019 (143), e59036, doi:10.3791/59036
|
Sharkey LM*, Safren N*, Pithadia AS*, Gerson JE, Dulchavsky M, Fischer S, Patel R, Lantis G, Ashraf N, Kim JH, Meliki A, Minakawa EN, Barmada SJ#, Ivanova MI#, Paulson HL#. Mutant UBQLN2 promotes toxicity by modulating intrinsic self-assembly. Proceedings of the National Academy of Sciences of the United States of America 2018 Oct 30;115(44): E10495.
|
Malik A, Miguez R, Li X, Ho YS, Feldman EL, and Barmada SJ. Matrin 3-dependent neurotoxicity is modified by nucleic acid binding and nucleocytoplasmic localization. eLife 2018;7:e35977.
|
Tank EM, Figueroa-Romero C, Hinder LM, Bedi K, Archbold HC, Li X, Weskamp K, Safren N, Paez-Colasante X, Pacut C, Thumma S, Paulsen MT, Guo K, Hur J, Ljungman M, Feldman EL, and Barmada SJ. Abnormal RNA stability in amyotrophic lateral sclerosis. Nature Communications 2018. 9:2845.
|
Archbold HC, Jackson K, Arora A, Weskamp K, Tank EM, Li X, Miguez R, Dayton RD, Tamir S, Klein RL, and Barmada SJ. TDP43 nuclear export and neurodegeneration in models of amyotrophic lateral sclerosis and frontotemporal dementia. Scientific Reports 2018. Mar 15;8(1):4606
|
Weskamp K, and Barmada SJ. TDP43 and RNA instability in amyotrophic lateral sclerosis. Brain Research, 2018 Jan 29. pii: S0006-8993(18)30023-4.
|
Al-Ramahi I, Panapakkam Giridharan SS, Chen YC, Patnaik S, Safren N, Hasegawa J, de Haro M, Wagner Gee AK, Titus SA, Jeong H, Clarke J, Krainc D, Zheng W, Irvine RF, Barmada S, Ferrer M, Southall N, Weisman LS, Botas J, Marugan JJ. Inhibition of PIP4Kγ ameliorates the pathological effects of mutant huntingtin protein. Elife. 2017 Dec 26;6. pii: e29123.
|
Green KM, Glineburg MR, Kearse MG, Flores BN, Linsalata AE, Fedak SJ, Goldstrohm AC, Barmada SJ, Todd PK. RAN translation at C9orf72-associated repeat expansions is selectively enhanced by the integrated stress response. Nat Commun. 2017 Dec 8;8(1):2005.
|
Wang B, Zeng L, Merillat SA, Fischer S, Ochaba J, Thompson LM, Barmada SJ, Scaglione KM, Paulson HL. The ubiquitin conjugating enzyme Ube2W regulates solubility of the Huntington's disease protein, huntingtin. Neurobiol Dis. 2018 Jan;109(Pt A):127-136.
|
Park SK, Hong JY, Arslan F, Kanneganti V, Patel B, Tietsort A, Tank EMH, Li X, Barmada SJ, Liebman SW. Overexpression of the essential Sis1 chaperone reduces TDP-43 effects on toxicity and proteolysis. PLoS Genet. 2017 May 22;13(5):e1006805.
|
Gupta R, Lan M, Mojsilovic-Petrovic J, Choi WH, Safren N, Barmada S, Lee MJ, Kalb R. The Proline/Arginine Dipeptide from Hexanucleotide Repeat Expanded C9ORF72 Inhibits the Proteasome. eNeuro. 2017 Jan 31;4(1).
|
Flores BN, Dulchavsky ME, Krans A, Sawaya MR, Paulson HL, Todd PK, Barmada SJ, Ivanova MI. Distinct C9orf72-Associated Dipeptide Repeat Structures Correlate with Neuronal Toxicity. PLoS One. 2016 Oct 24;11(10):e0165084.
|
Barmada SJ, Ju S, Arjun A, Batarse A, Archbold HC, Peisach D, Li X, Zhang Y, Tank EMH, Qiu H, Huang EJ, Ringe D, Petsko G, Finkbeiner S. Amelioration of toxicity in neuronal models of amyotrophic lateral sclerosis by hUPF1. Proc Natl Acad Sci U S A. 2015 Jun 8. pii: 201509744.
|
Barmada SJ. Linking RNA dysregulation and neurodegeneration in amyotrophic lateral sclerosis. Neurotherapeutics. 2015 Feb 18.
|
Barmada SJ, Serio A, Arjun A, Bilican B, Daub A, Ando DM, Tsvetkov A, Pleiss M, Li X, Peisach D, Shaw C, Chandran S, Finkbeiner S. Autophagy induction enhances TDP43 turnover and survival in neuronal ALS models. Nat Chem Biol. 2014 Aug;10(8):677-85.
|
Qiu H, Lee S, Shang Y, Wang WY, Au KF, Kamiya S, Barmada SJ, Finkbeiner S, Lui H, Carlton CE, Tang AA, Oldham MC, Wang H, Shorter J, Filiano AJ, Roberson ED, Tourtellotte WG, Chen B, Tsai LH, Huang EJ. ALS-associated mutation FUS-R521C causes DNA damage and RNA splicing defects. J Clin Invest. 2014 Mar 3;124(3):981-99.
|
Tsvetkov AS, Arrasate M, Barmada S, Ando DM, Sharma P, Shaby BA, Finkbeiner S. Proteostasis of polyglutamine varies among neurons and predicts neurodegeneration. Nat Chem Biol. 2013 Sep;9(9):586-92.
|
Serio A, Bilican B, Barmada SJ, Ando DM, Zhao C, Siller R, Burr K, Haghi G, Story D, Nishimura AL, Carrasco MA, Phatnani HP, Shum C, Wilmut I, Maniatis T, Shaw CE, Finkbeiner S, Chandran S. Astrocyte pathology and the absence of non-cell autonomy in an induced pluripotent stem cell model of TDP-43 proteinopathy. Proc Natl Acad Sci U S A. 2013 Mar 19;110(12):4697-702.
|
Armakola M, Higgins MJ, Figley MD, Barmada SJ, Scarborough EA, Diaz Z, Fang X, Shorter J, Krogan NJ, Finkbeiner S, Farese RV Jr, Gitler AD. Inhibition of RNA lariat debranching enzyme suppresses TDP-43 toxicity in ALS disease models. Nat Genet. 2012 Dec;44(12):1302-9.
|
Martens LH, Zhang J, Barmada SJ, Zhou P, Kamiya S, Sun B, Min SW, Gan L, Finkbeiner S, Huang EJ, Farese RV Jr. Progranulin deficiency promotes neuroinflammation and neuron loss following toxin-induced injury. J Clin Invest. 2012 Nov 1;122(11):3955-9.
|
Bilican B, Serio A, Barmada SJ, Nishimura AL, Sullivan GJ, Carrasco M, Phatnani HP, Puddifoot CA, Story D, Fletcher J, Park IH, Friedman BA, Daley GQ, Wyllie DJ, Hardingham GE, Wilmut I, Finkbeiner S, Maniatis T, Shaw CE, Chandran S. Mutant induced pluripotent stem cell lines recapitulate aspects of TDP-43 proteinopathies and reveal cell-specific vulnerability. Proc Natl Acad Sci U S A. 2012 Apr 10;109(15):5803-8.
|
Barmada SJ, Finkbeiner S. Pathogenic TARDBP mutations in amyotrophic lateral sclerosis and frontotemporal dementia: disease-associated pathways. Rev Neurosci. 2010;21(4):251-72.
|
Barmada SJ, Skibinski G, Korb E, Rao EJ, Wu JY, Finkbeiner S. Cytoplasmic mislocalization of TDP-43 is toxic to neurons and enhanced by a mutation associated with familial amyotrophic lateral sclerosis. J Neurosci. 2010 Jan 13;30(2):639-49.
|