Mitochondrial dysfunction in drug-induced hepatic steatosis: recent findings and current concept

Clinics and Research in Hepatology and Gastroenterology, Год журнала: 2025, Номер 49(3), С. 102529 - 102529

Опубликована: Янв. 9, 2025

Язык: Английский

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Targeting Mitochondrial Dysfunction in Cerebral Ischemia: Advances in Pharmacological Interventions

Antioxidants, Год журнала: 2025, Номер 14(1), С. 108 - 108

Опубликована: Янв. 18, 2025

The study of mitochondrial dysfunction has become increasingly pivotal in elucidating the pathophysiology various cerebral pathologies, particularly neurodegenerative disorders. Mitochondria are essential for cellular energy metabolism, regulation reactive oxygen species (ROS), calcium homeostasis, and execution apoptotic processes. Disruptions function, driven by factors such as oxidative stress, excitotoxicity, altered ion balance, lead to neuronal death contribute cognitive impairments several brain diseases. Mitochondrial can arise from genetic mutations, ischemic events, hypoxia, other environmental factors. This article highlights critical role progression diseases discusses need targeted therapeutic strategies attenuate damage, restore enhance neuroprotection.

Язык: Английский

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Mitochondria: An overview of their origin, genome, architecture, and dynamics

Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease, Год журнала: 2025, Номер unknown, С. 167803 - 167803

Опубликована: Март 1, 2025

Язык: Английский

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Mitochondrial Dysfunction in Endothelial Cells: A Key Driver of Organ Disorders and Aging

Antioxidants, Год журнала: 2025, Номер 14(4), С. 372 - 372

Опубликована: Март 21, 2025

Mitochondria are of great importance in cell biology since they major sites adenosine triphosphate (ATP) production and widely involved different cellular pathways the response to stress. During ATP production, reactive oxygen species (ROS) can be produced. While a small amount ROS may important for regulation physiological processes, at elevated levels turn into harmful agents leading damage. From pathological perspective, it could particularly interesting focus on mitochondrial function endothelial cells development aging onset diseases, including renal, cardio-metabolic, liver neurodegenerative ones. However, date, there no surveys which address above issues. To fill this gap, valuable collect recent findings about role mitochondria function, not only increase knowledge but also clinical applications. Here, we overview most issues view characterizing as an innovative potential target prevention aging, well treatment conditions.

Язык: Английский

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Ndufs4 knockout mouse models of Leigh syndrome: pathophysiology and intervention

Brain, Год журнала: 2021, Номер 145(1), С. 45 - 63

Опубликована: Ноя. 22, 2021

Abstract Mitochondria are small cellular constituents that generate energy (ATP) by oxidative phosphorylation (OXPHOS). Dysfunction of these organelles is linked to a heterogeneous group multisystemic disorders, including diabetes, cancer, ageing-related pathologies and rare mitochondrial diseases. With respect the latter, mutations in subunit-encoding genes assembly factors first OXPHOS complex (complex I) induce isolated I deficiency Leigh syndrome. This syndrome an early-onset, often fatal, encephalopathy with variable clinical presentation poor prognosis due lack effective intervention strategies. Mutations nuclear DNA-encoded NDUFS4 gene, encoding NADH:ubiquinone oxidoreductase subunit S4 (NDUFS4) I, ‘mitochondrial deficiency, type 1’ (MC1DN1) paediatric patients. A variety (tissue-specific) Ndufs4 knockout mouse models were developed study pathomechanism testing. Here, we review discuss role human disease, how analysis has generated new insights into MC1ND1/Leigh its therapeutic targeting.

Язык: Английский

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Pearson syndrome: a multisystem mitochondrial disease with bone marrow failure

Orphanet Journal of Rare Diseases, Год журнала: 2022, Номер 17(1)

Опубликована: Окт. 17, 2022

Abstract Pearson syndrome (PS) is a rare fatal mitochondrial disorder caused by single large-scale DNA deletions (SLSMDs). Most patients present with anemia in infancy. Bone marrow cytology vacuolization erythroid and myeloid precursors ring-sideroblasts guides to the correct diagnosis, which established detection of SLSMDs. Non hematological symptoms suggesting disease are often lacking at initial presentation, thus PS an important differential diagnosis isolated hypogenerative Spontaneous resolution occurs two-third age 1–3 years, while multisystem non-hematological complications such as failure thrive, muscle hypotonia, exocrine pancreas insufficiency, renal tubulopathy cardiac dysfunction develop during clinical course. Some experience phenotypical change Kearns-Sayre syndrome. In absence curative therapy, prognosis dismal. die acute lactic acidosis multi-organ early childhood. There great need for development novel therapies alter natural history PS.

Язык: Английский

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Mitochondria and sensory processing in inflammatory and neuropathic pain

Frontiers in Pain Research, Год журнала: 2022, Номер 3

Опубликована: Окт. 17, 2022

Rheumatic diseases, such as osteoarthritis and rheumatoid arthritis, affect over 750 million people worldwide contribute to approximately 40% of chronic pain cases. Inflammation tissue damage in rheumatic but often persists even when inflammation/damage is resolved. Mechanisms that cause this persistent are still unclear. Mitochondria essential for a myriad cellular processes regulate neuronal functions. Mitochondrial dysfunction has been implicated multiple neurological disorders, its role sensory processing diseases relatively unexplored. This review provides comprehensive understanding how mitochondrial connects inflammation damage-associated pathways sensitization pain. To provide an overall framework on mitochondria control pain, we explored recent evidence inflammatory neuropathic conditions. have intrinsic quality mechanisms prevent functional deficits damage. We will discuss the link between activity, Lastly, pharmacological strategies aimed at reestablishing functions or boosting dynamics therapeutic interventions discussed. The presented shows may play not restricted cells peripheral central nervous system, also includes blood joint level indirectly. Pre-clinical clinical data suggest modulation can be used attenuate eliminate which could beneficial diseases.

Язык: Английский

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Mechanisms Governing Oligodendrocyte Viability in Multiple Sclerosis and Its Animal Models

Cells, Год журнала: 2024, Номер 13(2), С. 116 - 116

Опубликована: Янв. 9, 2024

Multiple sclerosis (MS) is a chronic autoimmune inflammatory demyelinating disease of the central nervous system (CNS), which triggered by an assault targeting oligodendrocytes and myelin. Recent research indicates that demise due to attack contributes significantly pathogenesis MS its animal model experimental encephalomyelitis (EAE). A key challenge in lies comprehending mechanisms governing oligodendrocyte viability devising therapeutic approaches enhance survival. Here, we provide overview recent findings highlight contributions death development EAE summarize current literature on these diseases.

Язык: Английский

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Epilepsy in Mitochondrial Diseases—Current State of Knowledge on Aetiology and Treatment

Children, Год журнала: 2021, Номер 8(7), С. 532 - 532

Опубликована: Июнь 22, 2021

Mitochondrial diseases are a heterogeneous group of resulting from energy deficit and reduced adenosine triphosphate (ATP) production due to impaired oxidative phosphorylation. The manifestation mitochondrial disease is usually multi-organ. Epilepsy one the most common manifestations dysfunction, especially in children. onset epilepsy associated with poor prognosis, while its treatment very challenging, which further adversely affects course these disorders. Fortunately, our knowledge still growing, gives hope for patients improve their condition future. paper presents pathophysiology, clinical picture options disease.

Язык: Английский

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Treatment and prevention of pathological mitochondrial dysfunction in retinal degeneration and in photoreceptor injury

Biochemical Pharmacology, Год журнала: 2022, Номер 203, С. 115168 - 115168

Опубликована: Июль 12, 2022

Pathological deterioration of mitochondrial function is increasingly linked with multiple degenerative illnesses as a mediator wide range neurologic and age-related chronic diseases, including those genetic origin. Several these diseases are rare, typically defined in the United States an illness affecting fewer than 200,000 people U.S. population, or about one 1600 individuals. Vision impairment due to dysfunction eye prominent feature evident numerous primary common pathophysiology many familiar ophthalmic disorders, macular degeneration, diabetic retinopathy, glaucoma retinopathy prematurity — collection syndromes, disorders significant unmet medical needs. Focusing on metabolic pathway mechanisms, possible roles cuproptosis ferroptosis retinal dysfunction, we shed light potential α-lipoyl-L-carnitine treating diseases. α-Lipoyl-L-carnitine bioavailable mitochondria-targeting lipoic acid prodrug that has shown protecting against degeneration photoreceptor cell loss indications.

Язык: Английский

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