Review
Pharmacology & Pharmacy
Xiao-Wen Ding, Megan Robinson, Rongzi Li, Hadeel Aldhowayan, Thangiah Geetha, Jeganathan Ramesh Babu
Summary: The article discusses the pathophysiological relevance of mitochondria in Diabetes and Alzheimer's disease, as well as the potential therapeutic benefits of Szeto-Schiller-31 peptide in treating these conditions.
PHARMACOLOGICAL RESEARCH
(2021)
Review
Neurosciences
Alessandra Rufini, Florence Malisan, Ivano Condo, Roberto Testi
Summary: Friedreich ataxia is a rare neurodegenerative disorder caused by insufficient levels of the essential mitochondrial protein frataxin. It leads to reduced activity of mitochondrial enzymes, impaired mitochondrial metabolism, reduced ATP production and inefficient anti-oxidant response, resulting in the progressive loss of movement coordination and daily activity performance. Drug repositioning is a viable alternative for identifying a therapy for Friedreich ataxia.
FRONTIERS IN NEUROSCIENCE
(2022)
Article
Orthopedics
Xueying Zhang, Ying Zhang, Meng Zhang, Yusuke Nakagawa, Camila B. Caballo, Hazel H. Szeto, Xiang-Hua Deng, Scott A. Rodeo
Summary: This study found that there are changes in mitochondrial structure and function in tenocytes derived from degenerative tendons, and SS-31, as a mitochondrial protectant, could improve mitochondrial function and promote the healing of tendinopathy.
AMERICAN JOURNAL OF SPORTS MEDICINE
(2022)
Article
Neurosciences
Laura Rodriguez-Pascau, Elena Britti, Pablo Calap-Quintana, Yi Na Dong, Cristina Vergara, Fabien Delaspre, Marta Medina-Carbonero, Jordi Tamarit, Federico V. Pallardo, Pilar Gonzalez-Cabo, Joaquim Ros, David R. Lynch, Marc Martinell, Pilar Pizcueta
Summary: Friedreich ataxia (FRDA) is characterized by degeneration of large sensory neurons and spinocerebellar tracts, as well as cardiomyopathy and increased incidence in diabetes. The peroxisome proliferator-activated receptor gamma (PPAR gamma) pathway plays a key role in the pathogenesis of FRDA, with leriglitazone potentially providing an effective therapy by targeting this pathway to improve mitochondrial function and biogenesis. Leriglitazone has shown promising results in cellular and animal models, indicating its potential as a treatment for FRDA.
NEUROBIOLOGY OF DISEASE
(2021)
Article
Behavioral Sciences
Catherine Gerard, Annabelle Fortin Archambault, Camille Bouchard, Jacques P. Tremblay
Summary: Friedreich Ataxia (FRDA) is a genetic disease characterized by reduced production of the frataxin protein due to an expanded GAA repeat in the FXN gene. This disease leads to progressive neuromuscular degeneration and often results in death from heart failure. A mouse model with 800 GAA repeats has been developed and shown to accurately represent the disease's characteristics, making it a promising model for studying FRDA.
BEHAVIOURAL BRAIN RESEARCH
(2023)
Article
Genetics & Heredity
Camille Bouchard, Catherine Gerard, Solange Gni-fiene Yanyabe, Nathalie Majeau, Malek Aloui, Gabrielle Buisson, Pouire Yameogo, Vanessa Couture, Jacques P. Tremblay
Summary: This study aimed to find an optimized mouse model with a phenotype comparable to human patients for studying the impact of therapy. By comparing two mouse models (YG8sR and YG8-800) with healthy mice (Y47R) in behavior tests, significant differences were noticed between YG8sR mice injected with anti-FXN shRNA and YG8-800 mice compared to healthy mice. In conclusion, YG8sR mice have a slight phenotype, and injecting them with an AAV-PHP.B expressing anti-FXN shRNA does increase their phenotype; YG8-800 mice have a phenotype comparable to the human ataxic phenotype.
Article
Neurosciences
Daniel Fil, Robbie L. Conley, Aamir R. Zuberi, Cathleen M. Lutz, Terry Gemelli, Marek Napierala, Jill S. Napierala
Summary: Friedreich's ataxia is a neurodegenerative disease caused by reduced expression of the mitochondrial protein frataxin. This study investigated the impact of the G130V mutation on Fxn expression and its multi-system effect in vivo.
NEUROBIOLOGY OF DISEASE
(2023)
Article
Neurosciences
Elizabeth Mercado-Ayon, Nathan Warren, Sarah Halawani, Layne N. Rodden, Lucie Ngaba, Yi Na Dong, Joshua C. Chang, Carlos Fonck, Fulvio Mavilio, David R. Lynch, Hong Lin
Summary: FRDA is a neurodegenerative disorder caused by deficiency of the mitochondrial protein frataxin, leading to synaptic degeneration in the cerebellum. Knockdown of frataxin results in loss of cerebellar neurons, which can be partially reversed by frataxin restoration through gene transfer.
FRONTIERS IN NEUROSCIENCE
(2022)
Article
Genetics & Heredity
Mariana B. Angulo, Alexander Bertalovitz, Mariana A. Argenziano, Jiajia Yang, Aarti Patel, Theresa Zesiewicz, Thomas McDonald
Summary: This study utilized iPSC-derived neurons and cardiomyocytes to investigate the impact of frataxin deficiency. The results revealed specific effects on neurons and cardiomyocytes, with differential gene expression in glycolytic and extracellular matrix pathways.
MOLECULAR GENETICS & GENOMIC MEDICINE
(2023)
Article
Biochemistry & Molecular Biology
Marta Medina-Carbonero, Arabela Sanz-Alcazar, Elena Britti, Fabien Delaspre, Elisa Cabiscol, Joaquim Ros, Jordi Tamarit
Summary: Friedreich Ataxia is a rare neuro-cardiodegenerative disease caused by mutations in the frataxin gene. The most common mutation is a GAA expansion in the first intron of the gene, resulting in decreased frataxin expression. By introducing a specific mutation into the murine Fxn gene, researchers have analyzed the consequences of this mutation in vivo. They found that mice with this mutation displayed low frataxin levels in all tissues, neurological deficits resembling those in FA patients, and mitochondrial alterations. The researchers concluded that the primary pathological mechanism underlying this mutation is frataxin deficiency, suggesting frataxin replacement therapies may benefit patients with this mutation.
CELLULAR AND MOLECULAR LIFE SCIENCES
(2022)
Article
Neurosciences
Elisabetta Indelicato, Klaus Faserl, Matthias Amprosi, Wolfgang Nachbauer, Rainer Schneider, Julia Wanschitz, Bettina Sarg, Sylvia Boesch
Summary: This study used proteomics analysis to compare muscle samples from FRDA patients and controls, and found significant differences in mitochondrial protein profiles and metabolic pathways. These findings have important implications for the design of future therapeutic strategies.
FRONTIERS IN NEUROSCIENCE
(2023)
Article
Multidisciplinary Sciences
Wing-Hang Tong, Hayden Ollivierre, Audrey Noguchi, Manik C. Ghosh, Danielle A. Springer, Tracey A. Rouault
Summary: Cardiomyopathy is a primary cause of death in Friedreich ataxia patients with defective iron-sulfur cluster biogenesis. The mTOR and AKT signaling cascades play crucial roles in regulating cardiovascular growth and homeostasis, with mTOR and AKT hyperactivations possibly as part of the metabolic stress response to ISC deficiencies.
Article
Pharmacology & Pharmacy
Joseph Johnson, Elizabeth Mercado-Ayon, Elisia Clark, David Lynch, Hong Lin
Summary: Friedreich ataxia is a neurodegenerative disease characterized by the lack of frataxin protein in mitochondria. Deficiency of frataxin leads to excessive mitochondrial fragmentation, partially dependent on Drp1 activity. Inhibiting Drp1 can reverse mitochondrial fragmentation.
PHARMACOLOGY RESEARCH & PERSPECTIVES
(2021)
Article
Oncology
Riccardo Ballaro, Patrizia Lopalco, Valentina Audrito, Marc Beltra, Fabrizio Pin, Roberto Angelini, Paola Costelli, Angela Corcelli, Andrea Bonetto, Hazel H. Szeto, Thomas M. O'Connell, Fabio Penna
Summary: Cancer cachexia is a debilitating syndrome caused by tumor growth and chemotherapy, affecting skeletal muscle metabolism and function. The study focused on using the mitochondria-targeted compound SS-31 to counteract cachexia by improving metabolic function, showing effectiveness in early disease stages but limited effects in late refractory cachexia. Targeting mitochondrial function may be important in preventing cancer cachexia, with potential for multi-modal therapies including SS-31.
Article
Neurosciences
Chiara Pane, Alberto Maria Marra, Ludovica Aliberti, Mario Campanile, Federica Coscetta, Giulia Crisci, Roberta D'Assante, Angela Marsili, Giorgia Puorro, Andrea Salzano, Antonio Cittadini, Francesco Sacca
Summary: This study aims to investigate the potential of DMF, an already available treatment for MS and psoriasis, in correcting the biological deficits of FRDA and improving mitochondrial respiration in-vivo. The study consists of a screening visit and two sequential 12-week phases: a core phase and an extension phase. The primary endpoint is the change in FXN gene expression level after 12 weeks of treatment, and secondary endpoints include measures of frataxin protein level, cardiopulmonary exercise test outputs, echocardiographic measures, Nrf2 pathway and mitochondrial biogenesis gene expression, safety, clinical scales, and quality of life scales.
FRONTIERS IN NEUROSCIENCE
(2023)
Article
Biotechnology & Applied Microbiology
Lin Lin, Chuanhui Song, Zheng Wei, Huihui Zou, Shengwei Han, Zichen Cao, Xinyu Zhang, Guorong Zhang, Jianchuan Ran, Yu Cai, Wei Han
Summary: This study developed a novel organic photosensitive nanoparticle for photodynamic/photothermal therapy of oral leukoplakia. The results showed that the nanoparticles effectively treated the condition without apparent toxicity.
JOURNAL OF NANOBIOTECHNOLOGY
(2022)
Article
Nanoscience & Nanotechnology
Z. Wei, H. Zhang, H. Zou, C. Song, S. Zhao, Z. Cao, X. Zhang, G. Zhang, Y. Cai, W. Han
Summary: In this study, a novel nanoagent was constructed for imaging tumor vessel co-option and tumor micrometastasis. The nanoagent demonstrated high tissue penetration and low tissue autofluorescence, allowing real-time tumor imaging and control of vascular targeting therapy using laser.
MATERIALS TODAY NANO
(2022)
Article
Engineering, Biomedical
Jingyang Shan, Xiaoxuan Zhang, Yi Cheng, Chuanhui Song, Guopu Chen, Zhuxiao Gu, Yuanjin Zhao
Summary: This study developed a catalytic microneedle patch using near-infrared-II responsive and dual-nanozyme active Au-Cu2MoS4 nanosheets for treating diabetic wound infection. The patch achieves glucose consumption, oxygen generation, and bacterial elimination, providing an effective treatment for diabetic patients with wound infection.
ACTA BIOMATERIALIA
(2023)
Article
Engineering, Environmental
Junyi Che, Xiangyi Wu, Yong Shi, Chuanhui Song, Yuanjin Zhao, Lingyun Sun
Summary: Chronic hard-healing diabetic wounds are a significant burden on global health, and this study presents a novel hierarchically structured delivery system for promoting diabetic wound healing. The system utilizes macrophage-like nanovesicles encapsulated in hydrogel microspheres, which can protect and release the nanovesicles in the diabetic wound environment. The delivery system enhances the migration and tube formation abilities of endothelial cells and exhibits desirable wound healing effects in diabetic rats.
CHEMICAL ENGINEERING JOURNAL
(2023)
Article
Engineering, Biomedical
Jianchuan Ran, Tao Liu, Chuanhui Song, Zheng Wei, Chuanchao Tang, Zichen Cao, Huihui Zou, Xinyu Zhang, Yu Cai, Wei Han
Summary: This study demonstrates a rhythmically controlled mild-temperature photothermal therapy (mPTT) using organic photothermal nanoparticles. The rhythm mPTT shows improved antitumor efficacy and induction of tumor-specific immune responses. The temperature-dependent release of damage-associated molecular patterns (DAMPs) during mPTT-induced immunogenic cancer cell death (ICD) enhances immune activation. The results suggest that rhythm mPTT has significant potential for clinical applications in tumor treatment.
ADVANCED HEALTHCARE MATERIALS
(2023)
Article
Engineering, Environmental
Chuanhui Song, Xinyu Zhang, Zichen Cao, Zheng Wei, Meng Zhou, Yufeng Wang, Shengwei Han, Yu Cai, Wei Han
Summary: Immunotherapy is hindered by the immune suppression caused by high cholesterol concentration in the tumor microenvironment. To address this issue, researchers have developed a cholesterol-regulate nanoplatform that can reduce cholesterol levels and activate the immune system to enhance the effectiveness of tumor immunotherapy. Both in vitro and in vivo experiments have demonstrated that this nanoplatform can effectively kill tumor cells, regulate cholesterol levels, and stimulate the immune system, providing a new strategy for overcoming immune suppression in the tumor microenvironment and paving the way for adjuvant immunotherapy.
CHEMICAL ENGINEERING JOURNAL
(2023)
Article
Chemistry, Physical
Siyu Shen, Rui Liu, Chuanhui Song, Tao Shen, Yiwen Zhou, Junxia Guo, Bin Kong, Qing Jiang
Summary: This study proposes a novel fish-derived scaffold combined with photothermal therapy and mesenchymal stem cells (MSCs) to promote bone regeneration. The fish-derived scaffold, composed of decellularized fish scale and gelatin methacrylate synthesized from fish gelatin (fGelMA), can promote the proliferation and osteogenesis of MSCs without immunological rejection. Incorporating black phosphorus (BP) nanosheets into the fGelMA hydrogel network endows the hydrogel with the capacity of photothermal conversion stimulated by near-infrared (NIR) light. In vitro experiments showed that the fish-derived scaffold can enhance the osteogenesis process of MSCs with higher expression of osteogenic markers and mineralization assisted by NIR light. In addition, mice calvarial defects treated with the scaffold and photothermal therapy with MSCs exhibited accelerated regeneration. These findings suggest that the fish-derived scaffold, combined with photothermal therapy and MSCs, holds promise as a clinical strategy for bone regeneration.
Article
Engineering, Biomedical
Xinyu Zhang, Zichen Cao, Chuanhui Song, Zheng Wei, Meng Zhou, Si Chen, Jianchuan Ran, Hongbo Zhang, Huihui Zou, Shengwei Han, Yu Cai, Wei Han
Summary: Impressive results have been achieved in cancer treatment through immunotherapy. However, the abnormally high cholesterol metabolism in the tumor microenvironment (TME) hinders immunotherapy's effectiveness in patients with oral squamous cell carcinoma (OSCC). A cholesterol-modulating nanoplatform (PYT NP) was developed in this study to restore the normal immune microenvironment by reducing cholesterol in the TME, inhibiting tumor cell proliferation, and triggering immunogenic cell death. Equipped with a near-infrared photosensitizer (Y8), the nanoplatform promotes immune activation and intra-tumor infiltration for photoimmunotherapy. PYT NPs show great potential in sensitized OSCC immunotherapy.
ADVANCED HEALTHCARE MATERIALS
(2023)
Article
Chemistry, Multidisciplinary
Baojie Wen, Danqing Huang, Chuanhui Song, Jingyang Shan, Yuanjin Zhao
Summary: This study presents a novel ultrasound responsive natural pollen delivery system for synergistic treatment of triple negative breast cancer (TNBC) using simultaneous loading of chemotherapeutics and gaseous drugs. The hollow structure of pollen grains carries oxygen-enriched perfluorocarbon (PFC), and the porous spinous process structure adsorbs the chemotherapeutic drug doxorubicin (DOX) (PO/D-PGs). Ultrasound triggers oxygen release from PFC and excites DOX to realize chemo-sonodynamic therapy. The PO/D-PGs are demonstrated to effectively enhance oxygen concentration and increase the production of reactive oxygen species in the presence of low-intensity ultrasound, synergistically enhancing tumor killing ability. Based on ultrasound-facilitated PO/D-PGs, this synergistic therapy significantly enhances the antitumor effect in a mouse TNBC model, suggesting its potential as an effective strategy to enhance chemo-sonodynamic therapy for TNBC.
Article
Chemistry, Physical
Pengcheng Sun, Yuankai Jin, Yingying Yin, Chenyang Wu, Chuanhui Song, Yawei Feng, Peiyang Zhou, Xuezhi Qin, Yusheng Niu, Qiankai Liu, Jie Zhang, Zuankai Wang, Xiuqing Hao
Summary: Static repellency and pressure resistance are essential for high-performance super-omniphobic surfaces. However, these two properties are usually mutually exclusive. Inspired by the Springtail, researchers have developed a surface with micrometric and nanometric reentrant structures that can simultaneously provide static liquid repellency and pressure resistance. This design avoids the structural conflicts and achieves the highest-pressure resistance ever reported.
Review
Multidisciplinary Sciences
Danqing Huang, Jinglin Wang, Chuanhui Song, Yuanjin Zhao
Summary: Ultrasound is a biofavorable mechanical wave that has shown significant practical implications in the field of biomedicine. This review discusses the current developments in ultrasound-responsive matters, including breakable intermolecular conjugations, catalytic sonosensitizers, fluorocarbon compounds, microbubbles, and ultrasound-propelled micro- and nanorobots. The interactions between ultrasound and advanced matters create biochemical products and enhanced mechanical effects, leading to the exploration of potential biomedical applications and clinical translations. The review also highlights the current challenges and proposes future perspectives in the field of ultrasound-responsive matters in biomedicine.
Article
Chemistry, Multidisciplinary
Chuanhui Song, Xiangyi Wu, Jinglin Wang, Rui Liu, Yuanjin Zhao
Summary: In this study, a novel photosensitizer-immunotherapy integrated microneedles (MNs) were developed for preventing breast cancer recurrence and metastasis after surgery. The MNs had the capability to penetrate and deliver nanophotosensitizers to deep residual tumor tissue, which could be released through infrared ray (NIR) irradiation. The generated reactive oxygen species and local hyperthermia from nanophotosensitizers induced immunogenic death of cancer cells and activated dendritic cells (DCs) for system immunity. The results in a murine model demonstrated the effectiveness of the proposed MNs in protecting against local tumor relapse and distant metastasis in synergy with immune checkpoint inhibitors.
Article
Multidisciplinary Sciences
Chuanhui Song, Xiaoxuan Zhang, Minhui Lu, Yuanjin Zhao
Summary: In this study, a novel reactive oxygen species (ROS)-responsive detachable microneedle (MN) system was developed for controllable periodontal drug delivery and treatment. The bioinspired MNs can penetrate healthy gingival tissue and release the antibiotic metronidazole (Met) directly around the pathogen under high ROS levels in the periodontitis sulcus, resulting in improved therapeutic effects. The experimental results obtained from a rat model with periodontitis demonstrated the therapeutic potential of the bioinspired MNs in periodontal diseases.
Article
Chemistry, Multidisciplinary
Chuanhui Song, Xiangyi Wu, Yu Wang, Jinglin Wang, Yuanjin Zhao
Summary: Inspired by the behavior of cuttlefish expelling ink, this study developed thermal-responsive melanin-integrated porous microparticles for periodontitis treatment. These microparticles can release melanin nanoparticles under near-infrared irradiation, exhibiting antibacterial activity and anti-inflammatory properties, demonstrating promising therapeutic effects.
Article
Chemistry, Multidisciplinary
Xiangyi Wu, Haofang Zhu, Chuanhui Song, Qian Tan, Yuanjin Zhao, Luoran Shang
Summary: Inspired by the breadmaking process, yeast microcarriers (YMC) were developed as vehicles for stem cells in the treatment of diabetic wounds. The use of YMC increased the survival rate and retained functionality of transplanted adipose-derived stem cells (ADSCs). Additionally, YMC exhibited antioxidant activity, protecting ADSCs from oxidative stress and resulting in improved tissue regeneration in vivo.
ADVANCED MATERIALS
(2023)
