Search Results - "BLONDELLE, Jordan"

The Role of Cullin-RING Ligases in Striated Muscle Development, Function, and Disease by Blondelle, Jordan, Biju, Andrea, Lange, Stephan

“…The well-orchestrated turnover of proteins in cross-striated muscles is one of the fundamental processes required for muscle cell function and survival…”
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Transplantation of wild-type mouse hematopoietic stem and progenitor cells ameliorates deficits in a mouse model of Friedreich's ataxia by Rocca, Celine J, Goodman, Spencer M, Dulin, Jennifer N, Haquang, Joseph H, Gertsman, Ilya, Blondelle, Jordan, Smith, Janell L M, Heyser, Charles J, Cherqui, Stephanie

“…Friedreich's ataxia (FRDA) is an incurable autosomal recessive neurodegenerative disease caused by reduced expression of the mitochondrial protein frataxin due…”
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MLP and CARP are linked to chronic PKCα signalling in dilated cardiomyopathy by Lange, Stephan, Gehmlich, Katja, Lun, Alexander S., Blondelle, Jordan, Hooper, Charlotte, Dalton, Nancy D., Alvarez, Erika A., Zhang, Xiaoyu, Bang, Marie-Louise, Abassi, Yama A., dos Remedios, Cristobal G., Peterson, Kirk L., Chen, Ju, Ehler, Elisabeth

“…MLP (muscle LIM protein)-deficient mice count among the first mouse models for dilated cardiomyopathy (DCM), yet the exact role of MLP in cardiac signalling…”
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Semaphorin-PlexinD1 Signaling Limits Angiogenic Potential via the VEGF Decoy Receptor sFlt1 by Zygmunt, Tomasz, Gay, Carl Michael, Blondelle, Jordan, Singh, Manvendra K., Flaherty, Kathleen McCrone, Means, Paula Casey, Herwig, Lukas, Krudewig, Alice, Belting, Heinz-Georg, Affolter, Markus, Epstein, Jonathan A., Torres-Vázquez, Jesús

“…Sprouting angiogenesis expands the embryonic vasculature enabling survival and homeostasis. Yet how the angiogenic capacity to form sprouts is allocated among…”
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Hacd2 deficiency in mice leads to an early and lethal mitochondrial disease by Khadhraoui, Nahed, Prola, Alexandre, Vandestienne, Aymeline, Blondelle, Jordan, Guillaud, Laurent, Courtin, Guillaume, Bodak, Maxime, Prost, Bastien, Huet, Hélène, Wintrebert, Mélody, Péchoux, Christine, Solgadi, Audrey, Relaix, Frédéric, Tiret, Laurent, Pilot-Storck, Fanny

“…Mitochondria fuel most animal cells with ATP, ensuring proper energetic metabolism of organs. Early and extensive mitochondrial dysfunction often leads to…”
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HACD1, a regulator of membrane composition and fluidity, promotes myoblast fusion and skeletal muscle growth by Blondelle, Jordan, Ohno, Yusuke, Gache, Vincent, Guyot, Stéphane, Storck, Sébastien, Blanchard-Gutton, Nicolas, Barthélémy, Inès, Walmsley, Gemma, Rahier, Anaëlle, Gadin, Stéphanie, Maurer, Marie, Guillaud, Laurent, Prola, Alexandre, Ferry, Arnaud, Aubin-Houzelstein, Geneviève, Demarquoy, Jean, Relaix, Frédéric, Piercy, Richard J, Blot, Stéphane, Kihara, Akio, Tiret, Laurent, Pilot-Storck, Fanny

“…The reduced diameter of skeletal myofibres is a hallmark of several congenital myopathies, yet the underlying cellular and molecular mechanisms remain elusive…”
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Murine obscurin and Obsl1 have functionally redundant roles in sarcolemmal integrity, sarcoplasmic reticulum organization, and muscle metabolism by Blondelle, Jordan, Marrocco, Valeria, Clark, Madison, Desmond, Patrick, Myers, Stephanie, Nguyen, Jim, Wright, Matthew, Bremner, Shannon, Pierantozzi, Enrico, Ward, Samuel, Estève, Eric, Sorrentino, Vincenzo, Ghassemian, Majid, Lange, Stephan

“…Biological roles of obscurin and its close homolog Obsl1 (obscurin-like 1) have been enigmatic. While obscurin is highly expressed in striated muscles, Obsl1…”
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Cullin E3 Ligase Activity Is Required for Myoblast Differentiation by Blondelle, Jordan, Shapiro, Paige, Domenighetti, Andrea A., Lange, Stephan

“…The role of cullin E3-ubiquitin ligases for muscle homeostasis is best known during muscle atrophy, as the cullin-1 substrate adaptor atrogin-1 is among the…”
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Comparative molecular analysis of two HFpEF/diastolic dysfunction models – in search of a unifying disease mechanism by Fujita, Kyohei, Rudbaeck, Petter, Kourieh, Antonio, Desmond, Patrick, Marrocco, Valeria, Blondelle, Jordan, Sotak, Matus, Rajan, Meenu, Esteve, Eric, Velmurugan, Anush, Myers, Stephanie, Chan, Yunghang, Clark, Madison, Wright, Matthew, Gu, Yusu, Dalton, Nancy, Ghassemian, Majid, Klos, Matthew, Börgeson, Emma, Peterson, Kirk, Lange, Stephan

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Cathepsins in heart disease-chewing on the heartache? by Blondelle, Jordan, Lange, Stephan, Greenberg, Barry H, Cowling, Randy T

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Targeted Lipidomic Analysis of Myoblasts by GC-MS and LC-MS/MS by Blondelle, Jordan, Pais de Barros, Jean-Paul, Pilot-Storck, Fanny, Tiret, Laurent

“…Lipids represent ∼10% of the cell dry mass and play essential roles in membrane composition and physical properties, energy storage, and signaling pathways. In…”
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Progressive Structural Defects in Canine Centronuclear Myopathy Indicate a Role for HACD1 in Maintaining Skeletal Muscle Membrane Systems by Walmsley, Gemma L, Blot, Stéphane, Venner, Kerrie, Sewry, Caroline, Laporte, Jocelyn, Blondelle, Jordan, Barthélémy, Inès, Maurer, Marie, Blanchard-Gutton, Nicolas, Pilot-Storck, Fanny, Tiret, Laurent, Piercy, Richard J

“…Mutations in HACD1/PTPLA cause recessive congenital myopathies in humans and dogs. Hydroxyacyl-coA dehydratases are required for elongation of very long chain…”
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Cullin-3–dependent deregulation of ACTN1 represents a pathogenic mechanism in nemaline myopathy by Blondelle, Jordan, Tallapaka, Kavya, Seto, Jane T., Ghassemian, Majid, Clark, Madison, Laitila, Jenni M., Bournazos, Adam, Singer, Jeffrey D., Lange, Stephan

“…Nemaline myopathy is a congenital neuromuscular disorder characterized by muscle weakness, fiber atrophy, and presence of nemaline bodies within myofibers…”
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Ion Channel Activity in a 3‐hydroxyacyl‐coA dehydratase 1 (HACD1) Deficient Muscle Cell Line by Morgan, Rhiannon Sarah, Walmsley, Gemma, Dyer, Steven, O'Brien, Fiona, Staunton, Caroline, Blondelle, Jordan, Tiret, Laurent, Piercy, Richard, Barrett‐Jolley, Richard

“…Centronuclear myopathy in Labrador retrievers is the most common inherited neuromuscular disorder in dogs: this is caused by a mutation in a fatty acid…”
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Cardiolipin content controls mitochondrial coupling and energetic efficiency in muscle by Prola, Alexandre, Blondelle, Jordan, Vandestienne, Aymeline, Piquereau, Jérôme, Denis, Raphaël G P, Guyot, Stéphane, Chauvin, Hadrien, Mourier, Arnaud, Maurer, Marie, Henry, Céline, Khadhraoui, Nahed, Gallerne, Cindy, Molinié, Thibaut, Courtin, Guillaume, Guillaud, Laurent, Gressette, Mélanie, Solgadi, Audrey, Dumont, Florent, Castel, Julien, Ternacle, Julien, Demarquoy, Jean, Malgoyre, Alexandra, Koulmann, Nathalie, Derumeaux, Geneviève, Giraud, Marie-France, Joubert, Frédéric, Veksler, Vladimir, Luquet, Serge, Relaix, Frédéric, Tiret, Laurent, Pilot-Storck, Fanny

“…Unbalanced energy partitioning participates in the rise of obesity, a major public health concern in many countries. Increasing basal energy expenditure has…”
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Murine obscurin and Obsl1 have functionally redundant roles in sarcolemmal integrity, sarcoplasmic reticulum organization, and muscle metabolism by Blondelle, Jordan, Marrocco, Valeria, Clark, Madison, Desmond, Patrick, Myers, Stephanie, Nguyen, Jim, Wright, Matthew, Bremner, Shannon, Pierantozzi, Enrico, Ward, Samuel, Estève, Eric, Sorrentino, Vincenzo, Ghassemian, Majid, Lange, Stephan

“…Biological roles of obscurin and its close homolog Obsl1 (obscurin-like 1) have been enigmatic. While obscurin is highly expressed in striated muscles, Obsl1…”
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Cullin-3 dependent deregulation of ACTN1 represents a new pathogenic mechanism in nemaline myopathy by Blondelle, Jordan, Tallapaka, Kavya, Seto, Jane T, Ghassemian, Majid, Clark, Madison, Laitila, Jenni M, Bournazos, Adam, Singer, Jeffrey D, Lange, Stephan

“…Nemaline myopathy is a congenital neuromuscular disorder characterized by muscle weakness, fiber atrophy and presence of nemaline bodies within myofibers…”
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Beyond guidance: A novel role for Sema–PlxnD1 signaling in vascular development by Zygmunt, Tomasz, Gay, Carl M., Blondelle, Jordan, Flaherty, Kathleen McCrone, Means, Paula Casey, Herwig, Lukas, Krudewig, Alice, Belting, Heinz-Georg, Affolter, Markus, Torres-Vazquez, Jesus

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Semaphorin-PlexinD1 Signaling Limits Angiogenic Potential via the VEGF Decoy Receptor sFIt1 by ZYGMUNT, Tomasz, GAY, Carl Michael, EPSTEIN, Jonathan A, TORRES-VAZQUEZ, Jesus, BLONDELLE, Jordan, SINGH, Manvendra K, MCCRONE FLAHERTY, Kathleen, CASEY MEANS, Paula, HERWIG, Lukas, KRUDEWIG, Alice, BELTING, Heinz-Georg, AFFOLTER, Markus

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MLP and CARP are linked to chronic PKC[alpha] signalling in dilated cardiomyopathy by Lange, Stephan, Gehmlich, Katja, Lun, Alexander S, Blondelle, Jordan, Hooper, Charlotte, Dalton, Nancy D, Alvarez, Erika A, Zhang, Xiaoyu, Bang, Marie-louise, Abassi, Yama A, Dos Remedios, Cristobal G, Peterson, Kirk L, Chen, Ju, Ehler, Elisabeth

“…MLP (muscle LIM protein)-deficient mice count among the first mouse models for dilated cardiomyopathy (DCM), yet the exact role of MLP in cardiac signalling…”
Get full text