Search Results - "Benatar, Michael"

Lost in translation: Treatment trials in the SOD1 mouse and in human ALS by Benatar, Michael

“…Abstract Therapeutic success in the superoxide dismutase (SOD1) mouse model of amyotrophic lateral sclerosis (ALS) has not translated into effective therapy…”
Get full text

Neurofilament light: A candidate biomarker of presymptomatic amyotrophic lateral sclerosis and phenoconversion by Benatar, Michael, Wuu, Joanne, Andersen, Peter M., Lombardi, Vittoria, Malaspina, Andrea

“…Objective To evaluate neurofilament light (NfL) as a biomarker of the presymptomatic phase of amyotrophic lateral sclerosis (ALS). Methods The study population…”
Get full text

Clinical Effects of the Self-administered Subcutaneous Complement Inhibitor Zilucoplan in Patients With Moderate to Severe Generalized Myasthenia Gravis: Results of a Phase 2 Randomized, Double-Blind, Placebo-Controlled, Multicenter Clinical Trial by Howard, Jr, James F, Nowak, Richard J, Wolfe, Gil I, Freimer, Miriam L, Vu, Tuan H, Hinton, John L, Benatar, Michael, Duda, Petra W, MacDougall, James E, Farzaneh-Far, Ramin, Kaminski, Henry J, Barohn, Richard, Dimachkie, Mazen, Pasnoor, Mamatha, Farmakidis, Constantine, Liu, Tina, Colgan, Samantha, Benatar, Michael G, Bertorini, Tulio, Pillai, Rekha, Henegar, Robert, Bromberg, Mark, Gibson, Summer, Janecki, Teresa, Freimer, Miriam, Elsheikh, Bakri, Matisak, Paige, Genge, Angela, Guidon, Amanda, David, William, Habib, Ali A, Mathew, Veena, Mozaffar, Tahseen, Hewitt, William, Barnett, Deborah, Sullivan, Patricia, Ho, Doreen, Traub, Rebecca E, Chopra, Manisha, Aly, Radwa, Bayat, Elham, Abu-Rub, Mohammad, Khan, Shaida, Lange, Dale, Holzberg, Shara, Khatri, Bhupendra, Lindman, Emily, Olapo, Tayo, Sershon, Lisa M, Lisak, Robert P, Bernitsas, Evanthia, Jia, Kelly, Malik, Rabia, Lewis-Collins, Tiffany D, Nicolle, Michael, Sharma, Aditi, Roy, Bhaskar, Nye, Joan, Pulley, Michael, Berger, Alan, Shabbir, Yasmeen, Sachdev, Amit, Patterson, Kimberly, Siddiqi, Zaeem, Sivak, Mark, Bratton, Joan, Small, George, Kohli, Anem, Fetter, Mary, Vu, Tuan, Lam, Lucy, Harvey, Brittany, Silvestri, Nicholas, Patrick, Kara, Zakalik, Karen, MacDougall, James, Pontius, Angela, Hoarty, Michelle

“…Many patients with generalized myasthenia gravis (gMG) have substantial clinical disability, persistent disease burden, and adverse effects attributable to…”
Get more information

Amyotrophic lateral sclerosis - frontotemporal spectrum disorder (ALS-FTSD): Revised diagnostic criteria by Strong, Michael J., Abrahams, Sharon, Goldstein, Laura H., Woolley, Susan, Mclaughlin, Paula, Snowden, Julie, Mioshi, Eneida, Roberts-South, Angie, Benatar, Michael, HortobáGyi, Tibor, Rosenfeld, Jeffrey, Silani, Vincenzo, Ince, Paul G, Turner, Martin R.

“…This article presents the revised consensus criteria for the diagnosis of frontotemporal dysfunction in amyotrophic lateral sclerosis (ALS) based on an…”
Get full text

Neurofilaments in pre-symptomatic ALS and the impact of genotype by Benatar, Michael, Wuu, Joanne, Lombardi, Vittoria, Jeromin, Andreas, Bowser, Robert, Andersen, Peter M., Malaspina, Andrea

“…Objective. To evaluate serum and cerebrospinal fluid (CSF) levels of phosphorylated neurofilament heavy (pNfH), and to compare these to levels of neurofilament…”
Get full text

Mutations in prion-like domains in hnRNPA2B1 and hnRNPA1 cause multisystem proteinopathy and ALS by Kim, Hong Joo, Kim, Nam Chul, Wang, Yong-Dong, Scarborough, Emily A., Moore, Jennifer, Diaz, Zamia, MacLea, Kyle S., Freibaum, Brian, Li, Songqing, Molliex, Amandine, Kanagaraj, Anderson P., Carter, Robert, Boylan, Kevin B., Wojtas, Aleksandra M., Rademakers, Rosa, Pinkus, Jack L., Greenberg, Steven A., Trojanowski, John Q., Traynor, Bryan J., Smith, Bradley N., Topp, Simon, Gkazi, Athina-Soragia, Miller, Jack, Shaw, Christopher E., Kottlors, Michael, Kirschner, Janbernd, Pestronk, Alan, Li, Yun R., Ford, Alice Flynn, Gitler, Aaron D., Benatar, Michael, King, Oliver D., Kimonis, Virginia E., Ross, Eric D., Weihl, Conrad C., Shorter, James, Taylor, J. Paul

“…Algorithms designed to identify canonical yeast prions predict that around 250 human proteins, including several RNA-binding proteins associated with…”
Get full text

A randomized, double-blind, placebo-controlled phase II study of eculizumab in patients with refractory generalized myasthenia gravis by Howard Jr, James F., Barohn, Richard J., Cutter, Gary R., Freimer, Miriam, Juel, Vern C., Mozaffar, Tahseen, Mellion, Michelle L., Benatar, Michael G., Farrugia, Maria Elena, Wang, Jing Jing, Malhotra, Suneil S., Kissel, John T.

“…ABSTRACT Introduction: Complement activation at the neuromuscular junction is a primary cause of acetylcholine receptor loss and failure of neuromuscular…”
Get full text

Exome sequencing in amyotrophic lateral sclerosis implicates a novel gene, DNAJC7, encoding a heat-shock protein by Farhan, Sali M. K., Howrigan, Daniel P., Abbott, Liam E., Klim, Joseph R., Topp, Simon D., Byrnes, Andrea E., Churchhouse, Claire, Phatnani, Hemali, Smith, Bradley N., Rampersaud, Evadnie, Wu, Gang, Wuu, Joanne, Shatunov, Aleksey, Iacoangeli, Alfredo, Al Khleifat, Ahmad, Mordes, Daniel A., Ghosh, Sulagna, Eggan, Kevin, Rademakers, Rosa, McCauley, Jacob L., Schüle, Rebecca, Züchner, Stephan, Benatar, Michael, Taylor, J. Paul, Nalls, Michael, Gotkine, Marc, Shaw, Pamela J., Morrison, Karen E., Al-Chalabi, Ammar, Traynor, Bryan, Shaw, Christopher E., Goldstein, David B., Harms, Matthew B., Daly, Mark J., Neale, Benjamin M.

“…To discover novel genes underlying amyotrophic lateral sclerosis (ALS), we aggregated exomes from 3,864 cases and 7,839 ancestry-matched controls. We observed…”
Get full text

Increased functional connectivity common to symptomatic amyotrophic lateral sclerosis and those at genetic risk by Menke, Ricarda A L, Proudfoot, Malcolm, Wuu, Joanne, Andersen, Peter M, Talbot, Kevin, Benatar, Michael, Turner, Martin R

“…ObjectiveTo discern presymptomatic changes in brain structure or function using advanced MRI in carriers of mutations predisposing to amyotrophic lateral…”
Get full text

Mind the gap: The mismatch between clinical and imaging metrics in ALS by Verstraete, Esther, Turner, Martin R., Grosskreutz, Julian, Filippi, Massimo, Benatar, Michael

“…Advanced magnetic resonance imaging (MRI) techniques hold the promise to capture upper motor neuron loss and extramotor brain changes in amyotrophic lateral…”
Get full text

CRISPR/Cas9-mediated excision of ALS/FTD-causing hexanucleotide repeat expansion in C9ORF72 rescues major disease mechanisms in vivo and in vitro by Meijboom, Katharina E., Abdallah, Abbas, Fordham, Nicholas P., Nagase, Hiroko, Rodriguez, Tomás, Kraus, Carolyn, Gendron, Tania F., Krishnan, Gopinath, Esanov, Rustam, Andrade, Nadja S., Rybin, Matthew J., Ramic, Melina, Stephens, Zachary D., Edraki, Alireza, Blackwood, Meghan T., Kahriman, Aydan, Henninger, Nils, Kocher, Jean-Pierre A., Benatar, Michael, Brodsky, Michael H., Petrucelli, Leonard, Gao, Fen-Biao, Sontheimer, Erik J., Brown, Robert H., Zeier, Zane, Mueller, Christian

“…A GGGGCC 24+ hexanucleotide repeat expansion (HRE) in the C9ORF72 gene is the most common genetic cause of amyotrophic lateral sclerosis (ALS) and…”
Get full text

Phosphorylated neurofilament heavy chain: A biomarker of survival for C9ORF72‐associated amyotrophic lateral sclerosis by Gendron, Tania F., Daughrity, Lillian M., Heckman, Michael G., Diehl, Nancy N., Wuu, Joanne, Miller, Timothy M., Pastor, Pau, Trojanowski, John Q., Grossman, Murray, Berry, James D., Hu, William T., Ratti, Antonia, Benatar, Michael, Silani, Vincenzo, Glass, Jonathan D., Floeter, Mary Kay, Jeromin, Andreas, Boylan, Kevin B., Petrucelli, Leonard

“…As potential treatments for C9ORF72‐associated amyotrophic lateral sclerosis (c9ALS) approach clinical trials, the identification of prognostic biomarkers for…”
Get full text

Ocular Myasthenia by Al-Haidar, Mohammed, MD, Benatar, Michael, MD, PhD, Kaminski, Henry J., MD

“…Ocular myasthenia is a form of myasthenia gravis in which weakness is restricted to the ocular muscles and may produce significant visual disability. Patients…”
Get full text

Impaired corticomuscular and interhemispheric cortical beta oscillation coupling in amyotrophic lateral sclerosis by Proudfoot, Malcolm, van Ede, Freek, Quinn, Andrew, Colclough, Giles L., Wuu, Joanne, Talbot, Kevin, Benatar, Michael, Woolrich, Mark W., Nobre, Anna C., Turner, Martin R.

“…•Cortical motor output was measured in terms of neural oscillations using magnetoencephalography.•Patients with ALS demonstrate impaired corticomuscular…”
Get full text

Efficacy of prednisone for the treatment of ocular myasthenia (EPITOME): A randomized, controlled trial by Benatar, Michael, Mcdermott, Michael P., Sanders, Donald B., Wolfe, Gil I., Barohn, Richard J., Nowak, Richard J., Hehir, Michael, Juel, Vern, Katzberg, Hans, Tawil, Rabi

“…ABSTRACT Introduction: In this study we evaluated the safety, tolerability, and efficacy of prednisone in patients with ocular myasthenia gravis (OMG)…”
Get full text

Functional alterations in large-scale resting-state networks of amyotrophic lateral sclerosis: A multi-site study across Canada and the United States by Bharti, Komal, J Graham, Simon, Benatar, Michael, Briemberg, Hannah, Chenji, Sneha, Dupré, Nicolas, Dionne, Annie, Frayne, Richard, Genge, Angela, Korngut, Lawrence, Luk, Collin, Zinman, Lorne, Kalra, Sanjay

“…Amyotrophic lateral sclerosis (ALS) is a multisystem neurodegenerative disorder characterized by progressive degeneration of upper motor neurons and lower…”
Get full text

The Inverse Lhermitte Phenomenon Suggests Nitrous Oxide-Induced Myelopathy: Case Report and Review of the Literature by Sharma, Sonali, Benatar, Michael, Herskovitz, Steven, Granit, Volkan

“…Nitrous oxide-induced myelopathy is a relatively well-known clinical entity. Less well-known, however, is the rare inverse Lhermitte phenomenon, where neck…”
Get full text

Dipeptide repeat proteins inhibit homology-directed DNA double strand break repair in C9ORF72 ALS/FTD by Andrade, Nadja S, Ramic, Melina, Esanov, Rustam, Liu, Wenjun, Rybin, Mathew J, Gaidosh, Gabriel, Abdallah, Abbas, Del'Olio, Samuel, Huff, Tyler C, Chee, Nancy T, Anatha, Sadhana, Gendron, Tania F, Wahlestedt, Claes, Zhang, Yanbin, Benatar, Michael, Mueller, Christian, Zeier, Zane

“…The C9ORF72 hexanucleotide repeat expansion is the most common known genetic cause of amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD),…”
Get full text

FGF23, a novel muscle biomarker detected in the early stages of ALS by Si, Ying, Kazamel, Mohamed, Benatar, Michael, Wuu, Joanne, Kwon, Yuri, Kwan, Thaddaeus, Jiang, Nan, Kentrup, Dominik, Faul, Christian, Alesce, Lyndsy, King, Peter H.

“…Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease characterized by progressive muscle weakness. Skeletal muscle is a prime source for…”
Get full text

Batoclimab as induction and maintenance therapy in patients with myasthenia gravis: rationale and study design of a phase 3 clinical trial by Benatar, Michael, Wiendl, Heinz, Nowak, Richard, Zheng, Yan, Macias, William

“…IntroductionBatoclimab, a fully human monoclonal antibody that inhibits the neonatal fragment crystallisable receptor, has shown promising phase 2 clinical…”
Get full text