Search Results - "Widrick, Jeffrey"

In vivo gene editing in dystrophic mouse muscle and muscle stem cells by Tabebordbar, Mohammadsharif, Zhu, Kexian, Cheng, Jason K. W., Chew, Wei Leong, Widrick, Jeffrey J., Yan, Winston X., Maesner, Claire, Wu, Elizabeth Y., Xiao, Ru, Ran, F. Ann, Le Cong, Zhang, Feng, Vandenberghe, Luk H., Church, George M., Wagers, Amy J.

“…Frame-disrupting mutations in the DMD gene, encoding dystrophin, compromise myofiber integrity and drive muscle deterioration in Duchenne muscular dystrophy…”
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Directed evolution of a family of AAV capsid variants enabling potent muscle-directed gene delivery across species by Tabebordbar, Mohammadsharif, Lagerborg, Kim A., Stanton, Alexandra, King, Emily M., Ye, Simon, Tellez, Liana, Krunnfusz, Allison, Tavakoli, Sahar, Widrick, Jeffrey J., Messemer, Kathleen A., Troiano, Emily C., Moghadaszadeh, Behzad, Peacker, Bryan L., Leacock, Krystynne A., Horwitz, Naftali, Beggs, Alan H., Wagers, Amy J., Sabeti, Pardis C.

“…Replacing or editing disease-causing mutations holds great promise for treating many human diseases. Yet, delivering therapeutic genetic modifiers to specific…”
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An open source microcontroller based flume for evaluating swimming performance of larval, juvenile, and adult zebrafish by Widrick, Jeffrey J, Gibbs, Devin E, Sanchez, Benjamin, Gupta, Vandana A, Pakula, Anna, Lawrence, Christian, Beggs, Alan H, Kunkel, Louis M

“…Zebrafish are a preferred vertebrate model for delineating genotype-phenotype relationships. One of the most studied features of zebrafish is their exceptional…”
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Evaluation of Electrical Impedance as a Biomarker of Myostatin Inhibition in Wild Type and Muscular Dystrophy Mice by Sanchez, Benjamin, Li, Jia, Yim, Sung, Pacheck, Adam, Widrick, Jeffrey J, Rutkove, Seward B

“…Non-invasive and effort independent biomarkers are needed to better assess the effects of drug therapy on healthy muscle and that affected by muscular…”
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MicroRNA-486-dependent modulation of DOCK3/PTEN/AKT signaling pathways improves muscular dystrophy-associated symptoms by Alexander, Matthew S, Casar, Juan Carlos, Motohashi, Norio, Vieira, Natássia M, Eisenberg, Iris, Marshall, Jamie L, Gasperini, Molly J, Lek, Angela, Myers, Jennifer A, Estrella, Elicia A, Kang, Peter B, Shapiro, Frederic, Rahimov, Fedik, Kawahara, Genri, Widrick, Jeffrey J, Kunkel, Louis M

“…Duchenne muscular dystrophy (DMD) is caused by mutations in the gene encoding dystrophin, which results in dysfunctional signaling pathways within muscle…”
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DOCK3 regulates normal skeletal muscle regeneration and glucose metabolism by Samani, Adrienne, Karuppasamy, Muthukumar, English, Katherine G., Siler, Colin A., Wang, Yimin, Widrick, Jeffrey J., Alexander, Matthew S.

“…DOCK (dedicator of cytokinesis) is an 11‐member family of typical guanine nucleotide exchange factors (GEFs) expressed in the brain, spinal cord, and skeletal…”
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Passive force and viscoelastic properties of single fibers in human aging muscles by Lim, Jae-Young, Choi, Seung Jun, Widrick, Jeffrey J., Phillips, Edward M., Frontera, Walter R.

“…Purpose Changes in stiffness or extensibility of the muscle or muscle–tendon unit with aging could lead to impaired function and an increased vulnerability to…”
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SPEG-deficient skeletal muscles exhibit abnormal triad and defective calcium handling by Huntoon, Virginia, Widrick, Jeffrey J, Sanchez, Colline, Rosen, Samantha M, Kutchukian, Candice, Cao, Siqi, Pierson, Christopher R, Liu, Xiaoli, Perrella, Mark A, Beggs, Alan H, Jacquemond, Vincent, Agrawal, Pankaj B

“…Abstract Centronuclear myopathies (CNM) are a subtype of congenital myopathies (CM) characterized by skeletal muscle weakness and an increase in the number of…”
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IMP2 Increases Mouse Skeletal Muscle Mass and Voluntary Activity by Enhancing Autocrine Insulin-Like Growth Factor 2 Production and Optimizing Muscle Metabolism by Regué, Laura, Ji, Fei, Flicker, Daniel, Kramer, Dana, Pierce, William, Davidoff, Teekhon, Widrick, Jeffrey J., Houstis, Nicholas, Minichiello, Liliana, Dai, Ning, Avruch, Joseph

“…Insulin-like growth factor 2 (IGF2) mRNA binding protein 2 (IMP2) was selectively deleted from adult mouse muscle; two phenotypes were observed: decreased…”
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Electrical impedance myography detects age-related skeletal muscle atrophy in adult zebrafish by Rutkove, Seward B., Callegari, Santiago, Concepcion, Holly, Mourey, Tyler, Widrick, Jeffrey, Nagy, Janice A., Nath, Anjali K.

“…Age-related deficits in skeletal muscle function, termed sarcopenia, are due to loss of muscle mass and changes in the intrinsic mechanisms underlying…”
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Optimizing assays of zebrafish larvae swimming performance for drug discovery by Widrick, Jeffrey J, Lambert, Matthias R, Kunkel, Louis M, Beggs, Alan H

“…Zebrafish larvae are one of the few vertebrates amenable to large-scale drug discovery screens. Larval swimming behavior is often used as an outcome variable…”
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PDE10A Inhibition Reduces the Manifestation of Pathology in DMD Zebrafish and Represses the Genetic Modifier PITPNA by Lambert, Matthias R., Spinazzola, Janelle M., Widrick, Jeffrey J., Pakula, Anna, Conner, James R., Chin, Janice E., Owens, Jane M., Kunkel, Louis M.

“…Duchenne muscular dystrophy (DMD) is a severe genetic disorder caused by mutations in the DMD gene. Absence of dystrophin protein leads to progressive…”
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The SINE Compound KPT-350 Blocks Dystrophic Pathologies in DMD Zebrafish and Mice by Hightower, Rylie M., Reid, Andrea L., Gibbs, Devin E., Wang, Yimin, Widrick, Jeffrey J., Kunkel, Louis M., Kastenschmidt, Jenna M., Villalta, S. Armando, van Groen, Thomas, Chang, Hua, Gornisiewicz, Savanna, Landesman, Yosef, Tamir, Sharon, Alexander, Matthew S.

“…Duchenne muscular dystrophy (DMD) is an X-linked muscle wasting disease that is caused by the loss of functional dystrophin protein in cardiac and skeletal…”
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Muscle dysfunction in a zebrafish model of Duchenne muscular dystrophy by Widrick, Jeffrey J, Alexander, Matthew S, Sanchez, Benjamin, Gibbs, Devin E, Kawahara, Genri, Beggs, Alan H, Kunkel, Louis M

“…zebrafish lack the protein dystrophin and are the smallest vertebrate model of Duchenne muscular dystrophy (DMD). Their small size makes them ideal for…”
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Aerobic Capacity With Hybrid FES Rowing in Spinal Cord Injury: Comparison With Arms-Only Exercise and Preliminary Findings With Regular Training by Taylor, J. Andrew, PhD, Picard, Glen, MA, Widrick, Jeffrey J., PhD

“…Objective To determine the magnitude and range of increases in peak aerobic capacity with hybrid–functional electrical stimulation (FES) rowing versus…”
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Calcium-activated force of human muscle fibers following a standardized eccentric contraction by Choi, Seung Jun, Widrick, Jeffrey J

“…Peak Ca(2+)-activated specific force (force/fiber cross-sectional area) of human chemically skinned vastus lateralis muscle fiber segments was determined…”
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RNA helicase, DDX27 regulates skeletal muscle growth and regeneration by modulation of translational processes by Bennett, Alexis H, O'Donohue, Marie-Francoise, Gundry, Stacey R, Chan, Aye T, Widrick, Jeffrey, Draper, Isabelle, Chakraborty, Anirban, Zhou, Yi, Zon, Leonard I, Gleizes, Pierre-Emmanuel, Beggs, Alan H, Gupta, Vandana A

“…Gene expression in a tissue-specific context depends on the combined efforts of epigenetic, transcriptional and post-transcriptional processes that lead to the…”
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Transgenic zebrafish model of DUX4 misexpression reveals a developmental role in FSHD pathogenesis by Pakula, Anna, Lek, Angela, Widrick, Jeffrey, Mitsuhashi, Hiroaki, Bugda Gwilt, Katlynn M, Gupta, Vandana A, Rahimov, Fedik, Criscione, June, Zhang, Yuanfan, Gibbs, Devin, Murphy, Quinn, Manglik, Anusha, Mead, Lillian, Kunkel, Louis

“…Abstract Facioscapulohumeral dystrophy type 1 (FSHD-1) is the most common autosomal dominant form of muscular dystrophy with a prevalence of ∼1 in 8000…”
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Dynamic regulation of inter-organelle communication by ubiquitylation controls skeletal muscle development and disease onset by Mansur, Arian, Joseph, Remi, Kim, Euri S, Jean-Beltran, Pierre M, Udeshi, Namrata D, Pearce, Cadence, Jiang, Hanjie, Iwase, Reina, Milev, Miroslav P, Almousa, Hashem A, McNamara, Elyshia, Widrick, Jeffrey, Perez, Claudio, Ravenscroft, Gianina, Sacher, Michael, Cole, Philip A, Carr, Steven A, Gupta, Vandana A

“…Ubiquitin-proteasome system (UPS) dysfunction is associated with the pathology of a wide range of human diseases, including myopathies and muscular atrophy…”
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Enzyme replacement therapy rescues weakness and improves muscle pathology in mice with X-linked myotubular myopathy by Lawlor, Michael W, Armstrong, Dustin, Viola, Marissa G, Widrick, Jeffrey J, Meng, Hui, Grange, Robert W, Childers, Martin K, Hsu, Cynthia P, O'Callaghan, Michael, Pierson, Christopher R, Buj-Bello, Anna, Beggs, Alan H

“…No effective treatment exists for patients with X-linked myotubular myopathy (XLMTM), a fatal congenital muscle disease caused by deficiency of the lipid…”
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