Diquigiovanni, Chiara Rizzardi, Nicola Cataldi-Stagetti, Erica Gozzellino, Livia Isidori, Federica Valenti, Francesca Orsini, Arianna Astolfi, Annalisa Giangregorio, Tania Pironi, Loris
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Published in
Gastroenterology
We recently identified a recessive syndrome due to DNA ligase 3 (LIG3) mutations in patients with chronic intestinal pseudo-obstruction, leukoencephalopathy, and neurogenic bladder. LIG3 mutations affect mitochondrial DNA maintenance, leading to defective energy production. We aimed at identifying altered molecular pathways and developing possible ...
Romero-Zerbo, Silvana-Yanina Valverde, Nadia Claros, Silvia Zamorano-Gonzalez, Pablo Boraldi, Federica Lofaro, Francesco-Demetrio Lara, Estrella Pavia, Jose Garcia-Fernandez, Maria Gago, Belen
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Published in
Journal of advanced research
One of the hallmarks of Parkinsońs Disease (PD) is oxidative distress, leading to mitochondrial dysfunction and neurodegeneration. Insulin-like growth factor II (IGF-II) has been proven to have antioxidant and neuroprotective effects in some neurodegenerative diseases, including PD. Consequently, there isgrowing interest in understanding the differ...
Shinno, Kanako Miura, Yuri Iijima, Koichi M. Suzuki, Emiko Ando, Kanae
Published in
bioRxiv
Neuronal aging and neurodegenerative diseases are accompanied by proteostasis collapse, while cellular factors that trigger it are not identified. Impaired mitochondrial transport in the axon is another feature of aging and neurodegenerative diseases. Using Drosophila , we found that genetic depletion of axonal mitochondria causes dysregulation of ...
Watson, William D Arvidsson, Per M Miller, Jack JJ Lewis, Andrew J Rider, Oliver J
In health, the human heart is able to match ATP supply and demand perfectly. It requires 6 kg of ATP per day to satisfy demands of external work (mechanical force generation) and internal work (ion movements and basal metabolism). The heart is able to link supply with demand via direct responses to ADP and AMP concentrations but calcium concentrati...
Li, Huan Liu, Ying Wang, Dan Wang, Ya-Hong Sheng, Ruo-Cheng Kong, Zhi-Qiang Klosterman, Steven Chen, Jie-Yin Subbarao, Krishna Chen, Feng-Mao
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BACKGROUND: The complete mitochondrial respiratory chain is a precondition for maintaining cellular energy supply, development, and metabolic balance. Due to the evolutionary differentiation of complexes and the semi-autonomy of mitochondria, respiratory chain subunits have become critical targets for crop improvement and fungal control. In fungi, ...
Awalt, Jon Su, Wenyin Nguyen, William Loi, Katie Jarman, Kate Penington, Jocelyn Ramesh, Saishyam Fairhurst, Kate Yeo, Tomas Park, Heekuk
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Drug resistance against antimalarials is rendering them increasingly ineffective and so there is a need for the development of new antimalarials. To discover new antimalarial chemotypes a phenotypic screen of the Janssen Jumpstarter library against the P. falciparum asexual stage was undertaken, uncovering the cyclopropyl carboxamide structural hit...
Chabi, Béatrice
International audience
Varuzhanyan, Grigor Chen, Chia-Chun Freeland, Jack He, Tian Tran, Wendy Song, Kai Wang, Liang Cheng, Donghui Xu, Shili Dibernardo, Gabriella A
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Adenocarcinomas from multiple tissues can converge to treatment-resistant small cell neuroendocrine (SCN) cancers composed of ASCL1, POU2F3, NEUROD1, and YAP1 subtypes. We investigated how mitochondrial metabolism influences SCN cancer (SCNC) progression. Extensive bioinformatics analyses encompassing thousands of patient tumors and human cancer ce...
Jovanovic, Milena Marini, Joan C
Published in
Calcified tissue international
Osteogenesis imperfecta (OI) is a heterogeneous heritable skeletal dysplasia characterized by bone fragility and deformity, growth deficiency, and other secondary connective tissue defects. OI is now understood as a collagen-related disorder caused by defects of genes whose protein products interact with collagen for folding, post-translational mod...
Distefano, Giovanna Harrison, Stephanie Lynch, John Link, Thomas M Kramer, Philip A Ramos, Sofhia V Mau, Theresa Coen, Paul M Sparks, Lauren M Goodpaster, Bret H
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ObjectiveOur objective was to investigate the overall and sex-specific relationships between the presence and severity of knee osteoarthritis (KOA) and muscle composition, power, and energetics in older adults.MethodsMale and female patients (n = 655, mean ± SD age 76.1 ± 4.9 years; 57% female) enrolled in the Study of Muscle, Mobility, and Aging c...
