Article
Clinical Neurology
Dong-hua Chen, Wei Li, Hai-shan Jiang, Chao Yuan
Summary: This case report describes a 30-year-old female patient who presented with recurrent proximal weakness in the lower extremities. After a series of tests and examinations, she was diagnosed with mitochondrial disorder and showed significant improvement after the corresponding treatment.
FRONTIERS IN NEUROLOGY
(2023)
Article
Medicine, Research & Experimental
Nathaniel K. Mullin, Andrew P. Voigt, Miles J. Flamme-Wiese, Xiuying Liu, Megan J. Riker, Katayoun Varzavand, Edwin M. Stone, Budd A. Tucker, Robert F. Mullins
Summary: Variants in the high copy number mitochondrial genome can disrupt organelle function and lead to severe multisystem disease. Heteroplasmy, the uneven distribution of abnormal mtDNA molecules in different cells and tissues, contributes to the wide range of manifestations observed in patients with mitochondrial disease. This study reveals the nonrandom nature of mitochondrial variant distribution in human mitochondrial disease, highlighting its importance for understanding pathogenesis and developing treatments.
Article
Biochemistry & Molecular Biology
Hiroki Kobayashi, Hideyuki Hatakeyama, Haruna Nishimura, Mutsumi Yokota, Sadafumi Suzuki, Yuri Tomabechi, Mikako Shirouzu, Hiroyuki Osada, Masakazu Mimaki, Yu-ichi Goto, Minoru Yoshida
Summary: Mitochondrial DNA mutations cause mitochondrial diseases, leading to various phenotypic abnormalities in cells. A small-molecule compound called tryptolinamide (TLAM) was discovered to activate mitochondrial respiration and rescue defects in neuronal differentiation in iPSCs with a high ratio of mutant mtDNA by inhibiting PFK1 and promoting AMPK-mediated fatty-acid oxidation. TLAM redirects carbon flow from glycolysis to the pentose phosphate pathway to enhance anti-oxidative potential. These findings suggest that PFK1 could be a potential therapeutic target for mitochondrial diseases.
NATURE CHEMICAL BIOLOGY
(2021)
Article
Biochemistry & Molecular Biology
Werner J. Geldenhuys, Debbie Piktel, Javohn C. Moore, Stephanie L. Rellick, Ethan Meadows, Mark Pinti, John M. Hollander, Amanda G. Ammer, Karen H. Martin, Laura F. Gibson
Summary: B-cell acute lymphoblastic leukemia (ALL) affects both pediatric and adult patients. Resistant tumor cells contribute to minimal residual disease, leading to poor clinical outcomes in some patients. Targeting mitochondrial oxidative phosphorylation (OXPHOS) in the treatment of refractory leukemic cells is a potential novel approach. In this study, knockout of mitoNEET in REH leukemic cells led to changes in mitochondrial function and ultrastructure, decreased OXPHOS capacity, increased reactive oxygen species, and decreased proliferation when exposed to cytarabine.
FREE RADICAL BIOLOGY AND MEDICINE
(2021)
Review
Neurosciences
Qian Zhou, Meiqun Tian, Huan Yang, Yue-Bei Luo
Summary: Neuronal intranuclear inclusion disease (NIID) is a rare neurodegenerative disease with diverse manifestations. Curvilinear hyperintensity along the corticomedullary junction on diffusion-weighted images (DWI) is a crucial clue for diagnosing NIID. This study presents a NIID patient with mitochondrial encephalomyopathy, lactic acidosis and stroke-like episode, and reversible DWI hyperintensities.
Article
Radiology, Nuclear Medicine & Medical Imaging
Rong Wang, Xueling Liu, Chong Sun, Bin Hu, Liqin Yang, Yiru Liu, Daoying Geng, Jie Lin, Yuxin Li
Summary: This study investigated neurovascular coupling changes and potential neural basis in patients with MELAS using rs-fMRI and ASL. The results showed dynamic alterations in neurovascular coupling from acute to chronic stage in MELAS patients, providing a novel insight into the pathogenesis of MELAS.
JOURNAL OF MAGNETIC RESONANCE IMAGING
(2023)
Article
Biochemistry & Molecular Biology
Rafaela C. Cordeiro, Camila N. C. Lima, Gabriel R. Fries, Giovana Zunta-Soares, Jair C. Soares, Joao Quevedo, Giselli Scaini
Summary: This study investigates the impact of mitochondrial dysfunction on bipolar disorder (BD). The study finds that BD patients have a lower mitochondrial health index (MHI) compared to non-psychiatry controls, with a negative correlation between MHI and cell-free mtDNA levels. The study further reveals that MHI is related to the expression of mitochondria quality control (MQC) proteins, and a longer illness duration, worse functional status, and higher depressive symptoms are associated with lower MHI and higher cell-free mtDNA levels.
MOLECULAR PSYCHIATRY
(2023)
Article
Environmental Sciences
Weiqi Sun, Zhijing Ni, Rui Li, Xiuli Chang, Weihua Li, Mingjun Yang, Zhijun Zhou
Summary: The study showed that flurochloridone induces apoptosis in TM4 cells through the mitochondrial apoptotic pathway, accompanied by accumulation of ROS, depolarization of the mitochondrial membrane potential, etc. Pretreatment with the ROS scavenger NAC significantly alleviated flurochloridone-induced cell apoptosis and MMP depolarization.
ECOTOXICOLOGY AND ENVIRONMENTAL SAFETY
(2021)
Article
Environmental Sciences
Kuan Liu, Zhiqi Liu, Zhuofan Liu, Zhuo Ma, Yunfei Jia, Yu Deng, Wei Liu, Bin Xu
Summary: This study found that manganese poisoning can cause impairment of mitochondrial biogenesis. Further investigation revealed that S-nitrosylation of PINK1 and subsequent ZNF746 protein degradation are crucial signaling processes involved in the impairment of mitochondrial biogenesis caused by manganese poisoning.
SCIENCE OF THE TOTAL ENVIRONMENT
(2023)
Article
Cell Biology
Kumarie Latchman, Mario Saporta, Carlos T. Moraes
Summary: Human induced pluripotent stem cells have been used to study MELAS syndrome, and through investigation of cell type disease models, the impact of m.3243G mtDNA mutation on mitochondrial function in different tissues has been revealed, enhancing the understanding of hiPSC as a mitochondrial disease model.
Article
Immunology
Junbo Li, Jingzeng Wang, Tianhui Pan, Xi Zhou, Huifang Yang, Lu Wang, Guobin Huang, Chen Dai, Bo Yang, Bo Zhang, Yuanyuan Zhao, Peixiang Lan, Zhishui Chen
Summary: Recent research has shown that dysfunctional T cells can improve acceptance of organ transplants. In this study, mice lacking ubiquitin-specific protease 25 (USP25) had dysfunctional T cells, leading to prolonged survival of skin and heart grafts. Further investigation revealed that USP25 may reverse T cell dysfunction by regulating mitochondrial stability.
INTERNATIONAL IMMUNOPHARMACOLOGY
(2023)
Article
Biochemistry & Molecular Biology
Peng Wang, Shi-Yi Zheng, Ruo-Lin Jiang, Hao-Di Wu, Yong-Ang Li, Jiang-Long Lu, Ye Xiong, Bo Han, Li Lin
Summary: The natural compound Chelerythrine (CHE) induces necroptosis in glioma cells through the generation of mitochondrial reactive oxygen species (ROS) and mitochondrial dysfunction. CHE also promotes mitophagy to clear damaged mitochondria and enhance necroptosis. These findings suggest that CHE may be a potential novel therapeutic strategy for glioma treatment.
FREE RADICAL BIOLOGY AND MEDICINE
(2023)
Article
Biology
Aine Fairbrother-Browne, Aminah T. Ali, Regina H. Reynolds, Sonia Garcia-Ruiz, David Zhang, Zhongbo Chen, Mina Ryten, Alan Hodgkinson
Summary: Fairbrother-Browne and collaborators analyzed the relationship between nuclear and mitochondrial gene expression in the human brain, finding correlations governed by regional patterns that are disrupted in neurodegenerative diseases. The study shows that interactions between mitochondrial and nuclear genes are vital for normal brain function, and disruptions in these relationships may contribute to the pathogenesis of diseases like AD. The research presents a tool called MitoNuclearCOEXPlorer to explore crucial mitochondria-nuclear relationships in multi-dimensional brain data.
COMMUNICATIONS BIOLOGY
(2021)
Article
Oncology
Tianyu Cao, Li Liu, Kelvin Kai-Wang To, Chun-Yu Lim, Runhong Zhou, Yue Ming, Ka-Yi Kwan, Sulan Yu, Chun-Yin Chan, Biao Zhou, Haode Huang, Yufei Mo, Zhenglong Du, Ruomei Gong, Luk-Tsz Yat, Ivan Fan-Ngai Hung, Anthony Raymond Tam, Wing-Kin To, Wai-Shing Leung, Thomas Shiu-Hong Chik, Owen Tak-Yin Tsang, Xiang Lin, You-qiang Song, Kwok-Yung Yuen, Zhiwei Chen
Summary: Background: Patients with COVID-19 display a wide range of disease severity and immune responses, and the mechanisms are unclear. Methods: We studied 137 patients infected with SARS-CoV-2, divided into mild and severe groups, and analyzed their immune and mitochondrial functions. Results: Severe patients showed increased activation of extrafollicular B cells and inflammation, while mild patients countered the disease through modulation of B cell mitochondrial function. Patients who received prior vaccination showed reduced B cell responses and milder disease. Conclusion: We identified an immune mechanism that controls disease severity, with implications for virus research, treatment, and vaccine development.
CLINICAL AND TRANSLATIONAL MEDICINE
(2022)
Review
Biochemistry & Molecular Biology
Giovanni Pagano, Luca Tiano, Federico Pallardo, Alex Lyakhovich, Sudit S. Mukhopadhyay, Paolo Di Bartolomeo, Adriana Zatterale, Marco Trifuoggi
Summary: Fanconi anemia (FA) has been linked to defective DNA repair and proinflammatory condition in early studies, but recent research suggests a potential re-definition of FA as a mitochondrial disease. Studies have shown that FA proteins affect mitochondrial function, indicating a central role of mitochondrial dysfunction in FA. Mitochondrial cofactors may have beneficial effects on FA patients, opening new avenues for potential mitoprotective strategies.
Editorial Material
Radiology, Nuclear Medicine & Medical Imaging
Hidehiko Okazawa
JOURNAL OF NUCLEAR MEDICINE
(2022)
Article
Oncology
Shun Seto, Tetsuya Tsujikawa, Katsuji Sawai, Hidetaka Kurebayashi, Mitsuhiro Morikawa, Hidehiko Okazawa, Takanori Goi
Summary: This study aimed to evaluate the diagnostic accuracy of [F-18]FDG PET/MRI for preoperative staging and the usefulness of EMVI detection for predicting metastasis in rectal cancer. The results showed that [F-18]FDG PET/MRI had high sensitivity and specificity in T and N staging, and pmrEMVI positivity was a significant predictor for metastasis.
Article
Rehabilitation
Hideaki Matsuo, Masafumi Kubota, Mayumi Matsumura, Mami Takayama, Yuri Mae, Yuki Kitazaki, Soichi Enomoto, Asako Ueno, Masamichi Ikawa, Tadanori Hamano, Ai Takahashi, Misao Tsubokawa, Seiichiro Shimada
Summary: The study examined the time-course changes in center of pressure parameters in three patients with Wallenberg's syndrome presenting with body lateropulsion. The results showed that the COP position shifted to the BL side in initial standing but gradually returned to the center over time. Some patients exhibited faster COP velocities to the BL side compared to the non-BL side, which decreased over time.
PHYSIOTHERAPY THEORY AND PRACTICE
(2022)
Article
Clinical Neurology
Tomohisa Yamaguchi, Masamichi Ikawa, Souichi Enomoto, Norimichi Shirafuji, Osamu Yamamura, Tetsuya Tsujikawa, Hidehiko Okazawa, Hirohiko Kimura, Yasunari Nakamoto, Tadanori Hamano
Summary: ASL imaging can be used as a promising tool for the diagnosis of CBS by detecting CBF asymmetry, which shows a wider range of brain regions in patients with CBS compared to patients with PD.
Article
Spectroscopy
Hiroshi Yoshii, Kodai Takamura, Tetsuaki Uwatoko, Hiroaki Takahashi, Yasuhiro Sakai
Summary: X-ray fluorescence analysis is useful for detecting long half-life radionuclides, especially uranium isotopes that are difficult to detect by radiation measurements. This study proposes an energy-dispersive X-ray fluorescence analysis method for rapid screening of uranium-contaminated substances, and verifies the linear relationship between uranium concentration and signal intensity.
SPECTROCHIMICA ACTA PART B-ATOMIC SPECTROSCOPY
(2022)
Article
Biochemistry & Molecular Biology
Hidehiko Okazawa, Masamichi Ikawa, Tetsuya Tsujikawa, Tetsuya Mori, Akira Makino, Yasushi Kiyono, Yasunari Nakamoto, Hirotaka Kosaka, Makoto Yoneda
Summary: This study applied oxidative stress imaging using Cu-ATSM PET/MRI to evaluate patients with early Alzheimer's disease. The results showed that oxidative stress was particularly evident in the cingulate cortex and hippocampus of the patients. This study is important for understanding the pathogenesis and diagnosis of Alzheimer's disease.
Article
Spectroscopy
Hiroshi Yoshii, Tetsuaki Uwatoko, Hiroaki Takahashi, Yasuhiro Sakai
Summary: A rapid XRF-based screening method for trace uranium in solutions was proposed, with a minimum detection limit of 0.37 ng ml(-1) and a simultaneous analysis time of approximately 2 hours for six samples. The method is suitable for screening small volumes of uranium-contaminated water samples.
X-RAY SPECTROMETRY
(2022)
Article
Clinical Neurology
Yuki Kitazaki, Masamichi Ikawa, Tomohisa Yamaguchi, Soichi Enomoto, Norimichi Shirafuji, Osamu Yamamura, Tetsuya Tsujikawa, Hidehiko Okazawa, Hirohiko Kimura, Yasunari Nakamoto, Tadanori Hamano
Summary: This study investigated the relationship between striatal dopamine transporter density and changes in gray matter volume and cerebral perfusion in patients with Parkinson's disease. The findings showed that there was a significant positive correlation between the density of dopamine transporter and gray matter volume in the inferior frontal gyrus. In addition, a positive correlation was found between the density of dopamine transporter and cerebral blood flow in various brain regions, including the inferior frontal gyrus. These results suggest that the progression of dopaminergic neurodegeneration in Parkinson's disease may lead to regional cortical dysfunction in specific brain areas.
Article
Geriatrics & Gerontology
Tadanori Hamano, Miwako Nagata, Rokuro Matsubara, Yukihiko Ikebata, Tatsuhiko Ito, Akihiro Ibe, Youshi Fujita, Yukinori Kusaka, Takahiro Tokunaga, Soichi Enomoto, Yoshinori Endo, Asako Ueno, Norimichi Shirafuji, Masamichi Ikawa, Kouji Hayashi, Osamu Yamamura, Yasunari Nakamoto
Summary: Early intervention is crucial for dementia prevention, but the effective screening tool for early detection of dementia remains unclear. This study found that individuals who couldn't make a call by looking up phone numbers and manage their own deposits had an increased risk of low MMSE scores, suggesting that the self-reporting yes/no survey can effectively screen for dementia.
FRONTIERS IN AGING NEUROSCIENCE
(2023)
Article
Medicine, General & Internal
Makoto Kitade, Hideaki Nakajima, Tetsuya Tsujikawa, Sakon Noriki, Tetsuya Mori, Yasushi Kiyono, Hidehiko Okazawa, Akihiko Matsumine
Summary: Activated microglia play a role in secondary injury after acute spinal cord injury (SCI) and the development of spinal cord-related neuropathic pain (NeP). This study assessed the expression of translocator protein 18 kDa (TSPO) as an indicator of microglial activation and visualized the dynamics of activated microglia in the injured spinal cord using PET imaging with (R)-[C-11]PK11195. Results showed that TSPO was mainly expressed in activated microglia in SCI chimeric animal models.
JOURNAL OF CLINICAL MEDICINE
(2023)
Article
Multidisciplinary Sciences
Kai Nakagawa, Yongjeon Cheong, Seonkyoung Lee, Kaie Habata, Taku Kamiya, Daichi Shiotsu, Ichiro M. Omori, Hidehiko Okazawa, Hirotaka Kosaka, Minyoung Jung
Summary: The caudate nucleus, traditionally associated with motor control, may also play a role in attachment behavior, cognition, emotion, and mental functions. This study explores the relationship between white matter structure in the caudate nucleus and sensory processing using diffusion tensor imaging (DTI). The results suggest that diffusion parameters in the caudate nucleus are correlated with the Adolescent/Adult Sensory Profile (AASP) scores, specifically in relation to tactile sensation and avoidance.
Article
Medicine, General & Internal
Kenji Takata, Hirohiko Kimura, Shota Ishida, Makoto Isozaki, Yoshifumi Higashino, Ken-Ichiro Kikuta, Hidehiko Okazawa, Tetsuya Tsujikawa
Summary: The relationship between MRI-arterial spin labeling (ASL) parameters and PET-cerebral blood flow (CBF)/cerebrovascular reactivity (CVR) was investigated in Moyamoya disease. The results showed significant correlations between ASL parameters and PET-CBF/PET-CVR. Furthermore, ASL-ATT in the MCA territories was found to be related to CVR.
Article
Medicine, General & Internal
Akinobu Kita, Hidehiko Okazawa, Katsuya Sugimoto, Nobuyuki Kosaka, Eiji Kidoya, Tetsuya Tsujikawa
Summary: This study developed a new CNN method to estimate the specific binding ratio (SBR) using only frontal projection images in single-photon emission-computed tomography. Different datasets were created to train two CNNs, and the accuracy of SBR estimation was evaluated. The new CNN method showed feasibility and small error rate in estimating SBR from frontal projection images.
Article
Biochemistry & Molecular Biology
Hiroshi Yoshii, Kodai Takamura, Tetsuaki Uwatoko, Yasuhiro Sakai
Summary: In this study, a method based on X-ray fluorescence (XRF) was developed for the screening of uranium in brackish water samples in the event of an accident in a coastal area.
Meeting Abstract
Endocrinology & Metabolism
H. Okazawa, M. Ikawa, T. Tsujikawa, T. Mori, A. Makino, Y. Kiyono, H. Kosaka
JOURNAL OF CEREBRAL BLOOD FLOW AND METABOLISM
(2022)