Article
Cell Biology
Travis D. Carney, Rucha Y. Hebalkar, Evgeniia Edeleva, Ibrahim Omer cicek, Halyna R. Shcherbata
Summary: Deficiencies in the human dystrophin glycoprotein complex (DGC) cause muscular dystrophies, which are incurable disorders associated with muscle, brain, and eye abnormalities. This study investigated the transcriptomic changes in mutants of four DGC components under stress conditions, revealing the novel function of DGC in stress-response signaling. The findings provide new insights into the etiology of muscular dystrophy symptoms and potential treatment directions.
DISEASE MODELS & MECHANISMS
(2023)
Review
Medicine, Research & Experimental
Isela C. Valera, Amanda L. Wacker, Hyun Seok Hwang, Christina Holmes, Orlando Laitano, Andrew P. Landstrom, Michelle S. Parvatiyar
Summary: The dynamic remodeling of the DGC is a critical factor in various heart diseases, beyond genetic causes, and can affect the transmission of mechanical force to the extracellular matrix.
ADVANCES IN MEDICAL SCIENCES
(2021)
Article
Medicine, Research & Experimental
Zachary M. Howard, Lisa E. Dorn, Jeovanna Lowe, Megan D. Gertzen, Pierce Ciccone, Neha Rastogi, Guy L. Odom, Federica Accornero, Jeffrey S. Chamberlain, Jill A. Rafael-Fortney
Summary: Gene replacement therapy for DMD using micro-dystrophins is being tested in clinical trials, but its effectiveness in preventing heart failure is uncertain. Current animal models are not sufficient to evaluate the efficacy of micro-dystrophins on DMD-related heart failure. A new mouse model has been created to study heart failure progression in DMD and test the effectiveness of micro-dystrophin gene therapy for the first time.
Review
Biology
Darren Graham Samuel Wilson, Andrew Tinker, Thomas Iskratsch
Summary: This review summarizes the function and mechanisms of the Dystrophic Glycoprotein Complex (DGC) in mechanosignaling, with a particular focus on its role in muscular dystrophy. Dystrophin plays a crucial role in connecting the actin cytoskeleton to the extracellular matrix, and loss of a functional DGC leads to various muscular dystrophies and dilated cardiomyopathy. Evidence suggests that the DGC is involved in mechanosignaling, although this role is not fully understood. This review aims to provide an up-to-date perspective on the DGC's role in mechanotransduction and potential future interventions.
COMMUNICATIONS BIOLOGY
(2022)
Article
Biotechnology & Applied Microbiology
Lakmini P. Wasala, Thais B. Watkins, Nalinda B. Wasala, Matthew J. Burke, Yongping Yue, Yi Lai, Gang Yao, Dongsheng Duan
Summary: Duchenne muscular dystrophy (DMD) is a fatal muscle disease caused by dystrophin deficiency. In this study, it was found that mu Dys with both H1 and H4 effectively localized to the muscle membrane, restored the dystrophin-associated protein complex, and improved muscle force. On the other hand, mu Dys with only H1 or without H4 did not have the same effects. Therefore, H4 is essential for mu Dys function and H1 facilitates force production. These findings will contribute to the development of next-generation mu Dys gene therapy.
HUMAN GENE THERAPY
(2023)
Article
Cell Biology
Guillaume Gilbert, Chandan Kadur Nagaraju, Robin Duelen, Matthew Amoni, Pierre Bobin, Thomas Eschenhagen, H. Llewelyn Roderick, Maurilio Sampaolesi, Karin R. Sipido
Summary: The study found that hiPSC-CM express dystrophin, but lack several other DAPC proteins compared to adult cardiomyocytes. This suggests that commonly used hiPSC-CM maturation protocols may not be suitable for comprehensive assessment of DAPC function.
FRONTIERS IN CELL AND DEVELOPMENTAL BIOLOGY
(2021)
Article
Clinical Neurology
Thom T. J. Veeger, Erik W. Zwet, Diaa Mohamad, Karin J. Naarding, Nienke M. Velde, Melissa T. Hooijmans, Andrew G. Webb, Erik H. Niks, Jurriaan H. Groot, Hermien E. Kan
Summary: This study assessed the role of muscle architecture in fat replacement progression in Duchenne and Becker muscular dystrophies. The results showed that muscles with long fibers and large PCSAs were associated with increased fat fraction, with a stronger effect in muscles with larger PCSAs. This provides insight into the mechanical role in the pathophysiology of muscular dystrophies and could help support the selection and development of therapies.
Article
Biochemistry & Molecular Biology
Can Xin, Xiangyu Chu, Wenzhong Wei, Biao Kuang, Yiqing Wang, Ying Tang, Jincao Chen, Hongbo You, Chengwen Li, Bing Wang
Summary: This study demonstrated the efficacy of combined delivery of human mini-dystrophin and human VEGF genes to the temporalis muscles in improving muscle function and pathology in a DMD mouse model. The combined treatment group showed significant improvements in temporalis muscle pathology, function, inflammatory cell infiltration, central nucleation, and fibrosis.
HUMAN MOLECULAR GENETICS
(2021)
Review
Spectroscopy
Emily H. Canessa, Rita Spathis, James S. Novak, Aaron Beedle, Kanneboyina Nagaraju, Luca Bello, Elena Pegoraro, Eric P. Hoffman, Yetrib Hathout
Summary: The dystrophin-associated protein complex (DAPC) plays a crucial role in muscle fiber structure integrity and cell signaling. Mass spectrometry can be used to characterize the DAPC interactome and post-translational glycan modifications, providing insights into the role of this complex in muscle pathogenesis.
MASS SPECTROMETRY REVIEWS
(2022)
Review
Immunology
Rafael T. Han, Rachel D. Kim, Anna Molofsky, Shane A. Liddelow
Summary: This article reviews the various functions of astrocytes and their changes in reactive states, emphasizing interactions between astrocytes and microglia, vasculature, and protective barriers in the CNS, as well as discussing recent insights into different sub-states of astrocytes identified by transcriptional profiling. The goal is to stimulate research on how molecular identifiers link to specific functional changes in astrocytes and to define the implications of these heterogeneous molecular and functional changes in brain function and pathology, with the potential to lead to new therapies in CNS injury, infection, and disease.
Review
Physiology
Kay Ohlendieck, Dieter Swandulla
Summary: Duchenne muscular dystrophy is a progressive muscle wasting disorder with systemic effects on various organs, requiring comprehensive biomarker signatures and novel disease markers for differential diagnosis, prognosis, and therapy monitoring. Current therapeutic approaches to combat muscular dystrophy are also summarised.
PFLUGERS ARCHIV-EUROPEAN JOURNAL OF PHYSIOLOGY
(2021)
Review
Physiology
Katherine M. M. Kaplan, Kathleen G. G. Morgan
Summary: This review details the role of dystrophin and the dystrophin associated proteins (DAPs) in the vascular smooth muscle and emphasizes the need for further research.
FRONTIERS IN PHYSIOLOGY
(2022)
Review
Biochemistry & Molecular Biology
Nicolas Dubuisson, Romain Versele, Chloe Planchon, Camille M. Selvais, Laurence Noel, Michel Abou-Samra, Maria A. Davis-Lopez de Carrizosa
Summary: Duchenne muscular dystrophy (DMD) is a progressive disease caused by the loss of function of the protein dystrophin. There is currently no cure for DMD, but advances in genetic and exon-skipping therapies show promise. Histological assessment is a crucial tool for evaluating degeneration and regeneration, but it faces challenges due to the complexity of molecular events and the multitude of markers involved.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2022)
Article
Neurosciences
Nadia Skauli, Ekaterina Savchenko, Ole Petter Ottersen, Laurent Roybon, Mahmood Amiry-Moghaddam
Summary: This study suggests that the canonical BMP-signaling pathway may play a role in regulating the expression of AQP4 and the subcellular compartmentation of the associated DAP complex, thereby impacting the development of neurological conditions.
FRONTIERS IN CELLULAR NEUROSCIENCE
(2022)
Article
Surgery
Oliver J. Miles, James L. Wiffen, Damien G. Grinsell
Summary: Nipple delay (ND) surgery can reduce ischemic complications in nipple-sparing mastectomy (NSM), especially in patients with larger breasts.
JOURNAL OF PLASTIC RECONSTRUCTIVE AND AESTHETIC SURGERY
(2022)
Article
Multidisciplinary Sciences
Jose Rodrigues do Carmo Neto, Marcos Vinicius da Silva, Yarlla Loyane Lira Braga, Arthur Wilson Florencio da Costa, Simone Goncalves Fonseca, Patricia Resende Alo Nagib, Mara Rubia Nunes Celes, Milton Adriano Pelli Oliveira, Juliana Reis Machado
Summary: In a study using C57Bl/6 mice infected with T. cruzi Y strain, it was found that BMP2 and IFN gamma production in the intestine increased during the acute phase and correlated with inflammatory infiltrate. A decrease in the number of neurons in the myenteric plexus was observed during this phase. Collagen deposition gradually increased throughout the infection, especially in the chronic phase.
Review
Pathology
Simone Gusmao Ramos, Bruna Amanda da Cruz Rattis, Giulia Ottaviani, Mara Rubia Nunes Celes, Eliane Pedra Dias
Summary: COVID-19, caused by SARS-CoV-2, produces extensive damage to the body and affects important organ systems, leading to changes in microcirculation and injury mechanisms in different organs. The research provides a new assessment of COVID-19, exploring it as a transient molecular disease, and offers new insights for a rational treatment strategy.
AMERICAN JOURNAL OF PATHOLOGY
(2021)
Article
Cell Biology
Bruna A. C. Rattis, Ana C. Freitas, Jordana F. Oliveira, Joao L. A. Calandrini-Lima, Maria J. Figueiredo, Danilo F. Soave, Simone G. Ramos, Mara R. N. Celes
Summary: Sepsis-induced myocardial dysfunction leads to altered expression of CAV3 in the heart, with potential implications for L-type calcium channel regulation. This study suggests a crucial role for CAV3 in the development of cardiac dysfunction in sepsis and highlights the potential link between L-type calcium channels and CAV3 expression.
OXIDATIVE MEDICINE AND CELLULAR LONGEVITY
(2021)
Article
Cardiac & Cardiovascular Systems
Fernando Fonseca Franca Ribeiro, Henrique Turin Moreira, Antonio Carlos Leite de Barros-Filho, Denise M. Tanaka, Camila G. Fabricio, Luciano F. L. Oliveira, Cibele M. Prado, Marcus V. Simoes, Andre Schmidt, Benedito C. Maciel, Jose A. Marin-Neto, Minna Moreira Dias Romano
Summary: This study found that in the Syrian hamster model of Chagas disease, speckle tracking echocardiography parameters (GLS and GCS) showed early decrease, while changes in LVEF, LVESD, and GLS were correlated with myocardial inflammation but not with fibrosis.
INTERNATIONAL JOURNAL OF CARDIOVASCULAR IMAGING
(2022)
Article
Cell Biology
Jose Rodrigues Neto, Arthur Wilson Florencio da Costa, Yarlla Loyane Lira Braga, Fernanda Helia Lucio, Ana Luisa Monteiro dos Santos Martins, Marlene Antonia dos Reis, Flavia Aparecida de Oliveira, Mara Rubia Nunes Celes, Marcos Vinicius da Silva, Milton Adriano Pelli Oliveira, Juliana Reis Machado
Summary: This study aimed to evaluate the histopathological changes caused by infection with the Colombian strain of Trypanosoma cruzi in acute and chronic phases. Results showed that the infection led to weight loss, intestinal inflammatory infiltrates, increased levels of interferon gamma and tumor necrosis factor-alpha, as well as elevated interleukin-10 in the early chronic phase.
MEDIATORS OF INFLAMMATION
(2022)
Article
Multidisciplinary Sciences
Telemaco Luis da Silva, Antonio Pazin-Filho, Minna M. D. Romano, Virginia P. L. Ferriani, Jose A. Marin-Neto, Benedito C. Maciel, Andre Schmidt
Summary: The study found that mitral leaflets thickening at the time of ARF diagnosis is a consistent predictor for the subsequent evolution to RHD. Mitral valve thickness is an independent predictor of chronic valve dysfunction in patients with ARF.
Article
Immunology
Yarlla L. L. Braga, Jose R. C. Neto, Arthur W. F. Costa, Muriel V. T. Silva, Marcos V. Silva, Mara R. N. Celes, Milton A. P. Oliveira, Leo A. B. Joosten, Fatima Ribeiro-Dias, Rodrigo S. Gomes, Juliana R. Machado
Summary: IL-32 gamma plays a crucial role in controlling acute phase Chagas disease by influencing immune response characteristics and myocarditis.
JOURNAL OF IMMUNOLOGY RESEARCH
(2022)
Article
Immunology
Ana Paula Junqueira-Kipnis, Carine de Castro Souza, Ana Carolina de Oliveira Carvalho, Fabio Muniz de Oliveira, Vinnycius Pereira Almeida, Alisson Rodrigues de Paula, Mara Rubia Celes, Andre Kipnis
Summary: A new tuberculosis vaccine PEPf was developed, which can reduce lung bacterial load and improve protection. Combining PEPf with the Advax4 adjuvant and using a prime-boost strategy as a subunit vaccine significantly enhances protection.
Article
Pharmacology & Pharmacy
Andre Lopes Saraiva, Allisson Benatti Justino, Rodrigo Rodrigues Franco, Heitor Cappato Guerra Silva, Felipe dos Santos Arruda, Sandra Gabriela Klein, Mara Rubia Nunes Celes, Luiz Ricardo Goulart, Foued Salmen Espindola
Summary: In this study, the antioxidant and anti-inflammatory activities of polyphenols-rich fractions from Annona muricata L. leaves were investigated. The fractions reduced the generation of reactive oxygen species and secretion of inflammatory markers in immune cells. Furthermore, these fractions attenuated oxidative damage and inflammation in experimental acute lung injury. These findings suggest that these fractions might be explored for the development of new therapies for inflammatory conditions.
Article
Pharmacology & Pharmacy
Bruna A. C. Rattis, Henrique L. Piva, Andressa Duarte, Frederico G. F. L. R. Gomes, Janaina R. Lellis, Danilo F. Soave, Simone G. Ramos, Antonio C. Tedesco, Mara R. N. Celes
Summary: Curcumin showed cardioprotective effects in septic mice by reducing the expression of mTOR pathway-related genes, potentially achieved through modulation of the mTOR pathway.
Article
Biochemistry & Molecular Biology
Pablo Igor Ribeiro Franco, Jose Rodrigues do Carmo Neto, Marina Pacheco Miguel, Juliana Reis Machado, Mara Rubia Nunes Celes
Summary: This review summarizes the main molecular mechanisms of T. cruzi-related carcinogenesis and the mechanisms associated with tumor protection mediated by different parasite components.
Article
Pharmacology & Pharmacy
Maria Clara C. Cordeiro, Fernanda D. Tome, Felipe S. Arruda, Simone Goncalves da Fonseca, Patricia R. A. Nagib, Mara R. N. Celes
Summary: This study found that curcumin can interfere with macrophage function and polarization in malaria. It enhances the phagocytic activity of macrophages, particularly in non-inflammatory conditions, and promotes a balance in macrophage polarization by interfering with arginine availability.
Article
Infectious Diseases
Arthur Lauand Vargas, Beatriz de Paula Dias, Henrique Turin Moreira, Edgard Camilo de Oliveira-Filho, Denise Mayumi Tanaka, Marcus Vinicius Simoes, Benedito Carlos Maciel, Andre Schmidt, Jose Antonio Marin-Neto, Minna Moreira Dias Romano
Summary: This study using an experimental model found that reduced survival during the acute phase of Chagas disease was closely related to the significant reduction in left ventricular function, with a higher mortality rate in the group presenting with clinical symptoms.
REVISTA DO INSTITUTO DE MEDICINA TROPICAL DE SAO PAULO
(2021)
Article
Cardiac & Cardiovascular Systems
Matheus Rassi Fernandes Ramos, Henrique Turin Moreira, Gustavo Jardim Volpe, Minna Romano, Benedito Carlos Maciel, Andre Schmidt, Anis Rassi Junior, Jose Antonio Marin-Neto
Summary: Cardiomegaly on chest X-ray can predict the risk of death in patients with chronic Chagas cardiomyopathy, and the left ventricular end-diastolic diameter (LVEDD) on echocardiography is a reliable parameter for distinguishing cardiomegaly on chest X-ray.
ARQUIVOS BRASILEIROS DE CARDIOLOGIA
(2021)