Search Results - "van der Meulen, Jack H"

Mitochondrial redox signaling enables repair of injured skeletal muscle cells by Horn, Adam, Van der Meulen, Jack H, Defour, Aurelia, Hogarth, Marshall, Sreetama, Sen Chandra, Reed, Aaron, Scheffer, Luana, Chandel, Navdeep S, Jaiswal, Jyoti K

“…Strain and physical trauma to mechanically active cells, such as skeletal muscle myofibers, injures their plasma membranes, and mitochondrial function is…”
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Effect of the IL-1 Receptor Antagonist Kineret® on Disease Phenotype in mdx Mice by Benny Klimek, Margaret E, Sali, Arpana, Rayavarapu, Sree, Van der Meulen, Jack H, Nagaraju, Kanneboyina

“…Duchenne muscular dystrophy (DMD) is an X-linked muscle disease caused by mutations in the dystrophin gene. The pathology of DMD manifests in patients with…”
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Metabolic remodeling agents show beneficial effects in the dystrophin-deficient mdx mouse model by Jahnke, Vanessa E, Van Der Meulen, Jack H, Johnston, Helen K, Ghimbovschi, Svetlana, Partridge, Terrence, Hoffman, Eric P, Nagaraju, Kanneboyina

“…Duchenne muscular dystrophy is a genetic disease involving a severe muscle wasting that is characterized by cycles of muscle degeneration/regeneration and…”
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Membrane sealant Poloxamer P188 protects against isoproterenol induced cardiomyopathy in dystrophin deficient mice by Spurney, Christopher F, Guerron, Alfredo D, Yu, Qing, Sali, Arpana, van der Meulen, Jack H, Hoffman, Eric P, Nagaraju, Kanneboyina

“…Cardiomyopathy in Duchenne muscular dystrophy (DMD) is an increasing cause of death in patients. The absence of dystrophin leads to loss of membrane integrity,…”
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Lateral transmission of force is impaired in skeletal muscles of dystrophic mice and very old rats by Ramaswamy, Krishnan S., Palmer, Mark L., van der Meulen, Jack H., Renoux, Abigail, Kostrominova, Tatiana Y., Michele, Daniel E., Faulkner, John A.

“…Non‐technical summary  The force developed by a single fibre in frog muscles is transmitted laterally to the muscle surface with little or no loss. To…”
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Membrane Stabilization by Modified Steroid Offers a Potential Therapy for Muscular Dystrophy Due to Dysferlin Deficit by Sreetama, Sen Chandra, Chandra, Goutam, Van der Meulen, Jack H., Ahmad, Mohammad Mahad, Suzuki, Peter, Bhuvanendran, Shivaprasad, Nagaraju, Kanneboyina, Hoffman, Eric P., Jaiswal, Jyoti K.

“…Mutations of the DYSF gene leading to reduced dysferlin protein level causes limb girdle muscular dystrophy type 2B (LGMD2B). Dysferlin facilitates sarcolemmal…”
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Secreted acid sphingomyelinase as a potential gene therapy for limb girdle muscular dystrophy 2B by Bittel, Daniel C, Sreetama, Sen Chandra, Chandra, Goutam, Ziegler, Robin, Nagaraju, Kanneboyina, Van der Meulen, Jack H, Jaiswal, Jyoti K

“…Efficient sarcolemmal repair is required for muscle cell survival, with deficits in this process leading to muscle degeneration. Lack of the sarcolemmal…”
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FUNCTIONAL EVALUATION OF NERVE–SKELETAL MUSCLE CONSTRUCTS ENGINEERED IN VITRO by LARKIN, LISA M, VAN DER MEULEN, JACK H, DENNIS, ROBERT G, KENNEDY, JEFFREY B

“…Previously, we have engineered three-dimensional (3-D) skeletal muscle constructs that generate force and display a myosin heavy-chain (MHC) composition of…”
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Suppression of autophagy permits successful enzyme replacement therapy in a lysosomal storage disorder-murine Pompe disease by Raben, Nina, Schreiner, Cynthia, Baum, Rebecca, Takikita, Shoichi, Xu, Sengen, Xie, Tao, Myerowitz, Rachel, Komatsu, Masaaki, Van Der Meulen, Jack H., Nagaraju, Kanneboyina, Ralston, Evelyn, Plotz, Paul H.

“…Autophagy, an intracellular system for delivering portions of cytoplasm and damaged organelles to lysosomes for degradation/recycling, plays a role in many…”
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The molecular basis of skeletal muscle weakness in a mouse model of inflammatory myopathy by Coley, William, Rayavarapu, Sree, Pandey, Gouri S., Sabina, Richard L., Van der Meulen, Jack H., Ampong, Beryl, Wortmann, Robert L., Rawat, Rashmi, Nagaraju, Kanneboyina

“…Objective It is generally believed that muscle weakness in patients with polymyositis and dermatomyositis is due to autoimmune and inflammatory processes…”
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Preclinical drug trials in the mdx mouse: Assessment of reliable and sensitive outcome measures by Spurney, Christopher F., Gordish-Dressman, Heather, Guerron, Alfredo D., Sali, Arpana, Pandey, Gouri S., Rawat, Rashmi, Van Der Meulen, Jack H., Cha, Hee-Jae, Pistilli, Emidio E., Partridge, Terence A., Hoffman, Eric P., Nagaraju, Kanneboyina

“…The availability of animal models for Duchenne muscular dystrophy has led to extensive preclinical research on potential therapeutics. Few studies have focused…”
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Activation of the Ubiquitin Proteasome Pathway in a Mouse Model of Inflammatory Myopathy: A Potential Therapeutic Target by Rayavarapu, Sree, Coley, William, Meulen, Jack H., Cakir, Erdinc, Tappeta, Kathyayini, Kinder, Travis B., Dillingham, Blythe C., Brown, Kristy J., Hathout, Yetrib, Nagaraju, Kanneboyina

“…Objective Myositis is characterized by severe muscle weakness. We and others have previously shown that endoplasmic reticulum (ER) stress plays a role in the…”
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Mitochondria mediate cell membrane repair and contribute to Duchenne muscular dystrophy by Vila, Maria C, Rayavarapu, Sree, Hogarth, Marshall W, Van der Meulen, Jack H, Horn, Adam, Defour, Aurelia, Takeda, Shin’ichi, Brown, Kristy J, Hathout, Yetrib, Nagaraju, Kanneboyina, Jaiswal, Jyoti K

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Mitochondrial dysfunction and consequences in calpain-3-deficient muscle by Jahnke, Vanessa E, Peterson, Jennifer M, Van Der Meulen, Jack H, Boehler, Jessica, Uaesoontrachoon, Kitipong, Johnston, Helen K, Defour, Aurelia, Phadke, Aditi, Yu, Qing, Jaiswal, Jyoti K, Nagaraju, Kanneboyina

“…Nonsense or loss-of-function mutations in the non-lysosomal cysteine protease calpain-3 result in limb-girdle muscular dystrophy type 2A (LGMD2A). While…”
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The age‐related failure of adaptive responses to contractile activity in skeletal muscle is mimicked in young mice by deletion of Cu,Zn superoxide dismutase by Vasilaki, Aphrodite, van der Meulen, Jack H., Larkin, Lisa, Harrison, Dawn C., Pearson, Timothy, Van Remmen, Holly, Richardson, Arlan, Brooks, Susan V., Jackson, Malcolm J., McArdle, Anne

“…Summary In muscle, aging is associated with a failure of adaptive responses to contractile activity, and this is hypothesized to play an important role in…”
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Characterization of dysferlin deficient SJL/J mice to assess preclinical drug efficacy: fasudil exacerbates muscle disease phenotype by Rayavarapu, Sree, Van der Meulen, Jack H, Gordish-Dressman, Heather, Hoffman, Eric P, Nagaraju, Kanneboyina, Knoblach, Susan M

“…The dysferlin deficient SJL/J mouse strain is commonly used to study dysferlin deficient myopathies. Therefore, we systematically evaluated behavior in…”
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Daily supplementation of D-ribose shows no therapeutic benefits in the MHC-I transgenic mouse model of inflammatory myositis by Coley, William, Rayavarapu, Sree, van der Meulen, Jack H, Duba, Ayyappa S, Nagaraju, Kanneboyina

“…Current treatments for idiopathic inflammatory myopathies (collectively called myositis) focus on the suppression of an autoimmune inflammatory response within…”
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The proton pump inhibitor lansoprazole improves the skeletal phenotype in dystrophin deficient mdx mice by Sali, Arpana, Many, Gina M, Gordish-Dressman, Heather, van der Meulen, Jack H, Phadke, Aditi, Spurney, Christopher F, Cnaan, Avital, Hoffman, Eric P, Nagaraju, Kanneboyina

“…In Duchenne muscular dystrophy (DMD), loss of the membrane stabilizing protein dystrophin results in myofiber damage. Microinjury to dystrophic myofibers also…”
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Effect of genetic background on the dystrophic phenotype in mdx mice by Coley, William D, Bogdanik, Laurent, Vila, Maria Candida, Yu, Qing, Van Der Meulen, Jack H, Rayavarapu, Sree, Novak, James S, Nearing, Marie, Quinn, James L, Saunders, Allison, Dolan, Connor, Andrews, Whitney, Lammert, Catherine, Austin, Andrew, Partridge, Terence A, Cox, Gregory A, Lutz, Cathleen, Nagaraju, Kanneboyina

“…Genetic background significantly affects phenotype in multiple mouse models of human diseases, including muscular dystrophy. This phenotypic variability is…”
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Mitochondria mediate cell membrane repair and contribute to Duchenne muscular dystrophy by Vila, Maria C, Rayavarapu, Sree, Hogarth, Marshall W, Van der Meulen, Jack H, Horn, Adam, Defour, Aurelia, Takeda, Shin’ichi, Brown, Kristy J, Hathout, Yetrib, Nagaraju, Kanneboyina, Jaiswal, Jyoti K

“…Dystrophin deficiency is the genetic basis for Duchenne muscular dystrophy (DMD), but the cellular basis of progressive myofiber death in DMD is not fully…”
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