Clinical Trials About "footprints mitochondrial impairment cellular energy crisis pathogenesis xenobiotics" RSS

20:14 EDT 26th June 2019 | BioPortfolio

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Showing "footprints mitochondrial impairment cellular energy crisis pathogenesis xenobiotics" Clinical Trials 1–25 of 7,100+

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Mitochondrial Complex I Dysfunction in PWS

Prader-Willi Syndrome (PWS) is characterized by profound infantile hypotonia, growth delay, cognitive impairment, muscle weakness and exercise intolerance. Studies have suggested that a defect in energy metabolism, yet to be clarified, may be involved in its pathogenesis. Many PWS patients have received Coenzyme Q10, but the rationale for this and objective impact on cellular metabolism has not been clarified.

Mitochondrial DNA as a Biomarker of Sepsis Severity

Mitochondria are organelles (a specialized subunit of a cell) responsible for providing cells with energy. For reasons not yet understood, mitochondria will release their DNA into blood in response to cellular injury or cell death. With a simple blood draw, investigators can measure the amount of mitochondrial DNA in a patient's blood. The investigators' hypothesis, is that mitochondrial DNA can be used as a surrogate marker of cellular injury to predict patient outcome...

Mitochondrial Bioenergetics and Role in Cellular Damage in Ischemic Myocardium

Cardiac ischemia is a common pathological condition, known to elicit multiple pathological processes at the cellular level. One of the most affected is thought to be cellular metabolism, key for the adequate cardiac function. The aim is to study mitochondrial bioenergetic function, interaction with other cellular systems and influence of several co-morbidities in myocardium of the affected patients.

Oxygen Therapy in Schizophrenia

Due to intense ATP-consuming processes in the brain, a high level of brain energy supply is required. A popular hypothesis regarding the pathogenesis and pathophysiology of schizophrenia postulates hypofunction of neuronal circuits in the prefrontal and limbic-temporal areas. An emerging body of data suggests that impaired energy metabolism due to mitochondrial dysfunction plays a role in the pathophysiology of schizophrenia. Under normal conditions cellular metabolic rate, i...


Mitochondrial Energy Metabolism in Obese Women

Considering that the failure of the treatment of obesity is justified by the multifactorial pathophysiology of this morbidity, the present project has the following hypotheses: 1. The occurrence of obesity is due to the derange,ent of mitochondrial energy metabolism ; 2. The unbalance is therapeutically modified through physical training ; 3. Obesity courses with the break-down in energy metabolism mitochondrial disease associated with systemic inflamma...

Nicotinamide Riboside and Mitochondrial Biogenesis

Mitochondria are important parts of the cell that are responsible for producing energy. The amount of energy they produce depends on how much energy the body needs to function and this energy production can be severely impaired in people with mitochondrial disease. Symptoms of mitochondrial disease vary widely but usually involve the brain, nerves and muscles, as these are tissues that need a lot of energy. Mitochondrial disorders affect 1 in 5000 of the UK population and there...

Phase III Trial of Coenzyme Q10 in Mitochondrial Disease

Our central hypothesis is that oral CoQ10 is a safe and effective treatment for children with inborn errors of mitochondrial energy metabolism due to defects in specific respiratory chain (RC) complexes or mitochondrial DNA (mtDNA) mutations, and that this beneficial action is reflected in improved motor and neurobehavioral function and in quality of life.

Mitochondria in HIV and Aging (MITO+)

Older adults with human immunodeficiency virus (HIV) and a long history of antiretroviral therapy have more mitochondrial dysfunction- the cells that help them make energy. This dysfunction in mitochondria may lead to symptoms of muscle fatigue, physical function impairment, and impaired exercise tolerance compared to HIV-uninfected controls of a similar age and body mass index (BMI). Furthermore, the investigators hypothesize that the older antiretroviral therapy (ART) of teno...

Executive Function Disorders and Anxio-depressive Symptomatology in Children and Adolescents With Mitochondrial Pathologies

The major steps forward of the neurosciences in recent years have linked psychiatric diseases, neuropsychological symptoms and brain dysfunctions. The cerebral functioning requiring a big quantity of energy, mitochondria, essential organelles in the cellular energy processes, are at present considered as a way of research for big interest in neurology and in psychiatry. Thus, an increasing number of studies describe potential links between mitochondrial dysfunction and psychiat...

Skeletal Muscle and Platelet Mitochondrial Dysfunction During Sepsis

The primary aim of the study is to demonstrate that mitochondrial dysfunction occurs in both skeletal muscle and circulating platelets of severely septic and septic shock ICU-admitted patients. Secondary aims are to clarify the pathogenesis and the clinical relevance of mitochondrial damage during sepsis.

The Effects of Exercise Versus Inactivity on People With Mitochondrial Muscle Disease

Mitochondrial myopathies include various inherited diseases that are caused by damage to the mitochondria, energy-producing structures that fuel the body's processes. The main symptoms are muscle weakness, reduced muscle mass, and difficulty with exercising. The purpose of this study is to determine the effects of exercise training versus inactivity on mitochondrial function in muscle and muscle performance in people with mitochondrial myopathies.

mtDNA as Novel Biomarker for Intra-amniotic Infection

Finding a predictive biomarker for IAI could improve the clinical outcome for the mother and the neonate. The aim of this study is to quantify the copy number of circulating cell-free mitochondrial DNA in maternal serum and the placenta compared to controls.the investigators hypothesise that circulating cell-free mitochondrial DNA levels could help predict the likelihood of early inflammation in IAI. In addition, mitochondrial DNA could be a promotor triggering the pathogenesis...

Calf Muscle Strength in Mitochondrial Diseases

Mitochondrial disorders are a group of inherited disorders causing malfunctional mitochondria. Mitochondria are found in every cell of the body, and the disorders therefore give symptoms from every tissue, especially those with high energy needs as the brain, heart and muscles. The disorders are highly disabling. The aim of the study is to investigate the relation between muscle strength and contractile cross sectional area (CCSA) in the leg of patients affected by mitochondri...

Nicotinamide Riboside and Mitochondrial Metabolism

Vitamin B3 has recently been found to be a potent modifier of energy metabolism, especially the function of mitochondria. Mitochondria power up all cells in our bodies, by generating fuel, ATP, for cellular functions. In previous studies, it has been discovered that mitochondrial biogenesis and oxidative metabolism in adipose tissue is severely impaired in obesity, already at a young adult age. Here the investigators describe a proposal where they use nicotinamide riboside (NR)...

Relative Mitochondrial Toxicity of Tenofovir Alafenamide (TAF) vs. Tenofovir Disoproxil Fumarate (TDF)

Increased comorbidities such as cardiovascular disease (CVD), are emerging problems in HIV infection but the mechanisms are unclear. Understanding how antiretrovirals can minimize morbidity in treated HIV infection is a research priority. Nucleoside/nucleotide reverse transcriptase inhibitors (NRTIs) are included in all HIV treatment regimens. Tenofovir (TFV) disoproxil fumarate (TDF) has been associated with an increased risk of nephrotoxicity and bone disease compared with ot...

Mitochondrial Impairment in Muscle Insulin Resistance

This investigation is being carried out to learn more about research findings from a study that was completed last year. Those findings revealed that within the skeletal muscle cells of individuals with type 2 diabetes, there was often damage to the mitochondria (the muscle cell's power source or the machinery of the muscle cell that produces energy). In individuals with type 2 diabetes, the liver continues to release sugar even when sugar levels are normal; the pancreas is n...

GDF-15 as a Biomarker for Mitochondrial Disease

Mitochondrial disorders are a group of inherited disorders causing malfunctional mitochondria. Mitochondria are found in every cell of the body, and the disorders therefore give symptoms from every tissue, especially those with high energy needs as the brain, heart and muscles. The symptoms are often unspecific in terms of muscle weakness and fatigue, which delays the first contact to the doctor and further delays the diagnosis. The aim of this study is to investigate if it is...

A Pilot Trial of Triheptanoin for People With Amyotrophic Lateral Sclerosis (PALS)

The causes of ALS are largely unknown. However, mitochondrial dysfunction, resulting in impaired energy production, oxidative stress and apoptosis, may play a key role in ALS progression. Triheptanoin can improve mitochondrial function and energy production and therefore has potential for slowing ALS progression. Indeed, triheptanoin slowed motor neuron loss and delayed the onset of weakness in a mutant SOD1 model of ALS. This pilot trial will determine if Triheptanoin is safe ...

Pathophysiology Analysis of "Costello Syndrome" on Cellular Models

The objective is to collect skin biopsies rom patients with Costello syndrome to analyze the molecular mechanisms responsible for this syndrome caused by a mutation in the HRAS gene and the effects of this mutation on energy metabolism and mitochondrial physiology.

A Dose-escalating Clinical Trial With KH176

Mitochondrial Diseases are rare progressive, multi-system, often early fatal disorders affecting both children and adults. KH176 is a novel chemical entity currently under development for the treatment of inherited mitochondrial diseases, including MELAS (Mitochondrial Encephalomyopathy, Lactic acidosis, and Stroke-like episodes), Leigh's Disease and LHON (Leber's Hereditary Optic Neuropathy). KH176 is a potent intracellular redox modulating agent targeting the reactive oxygen ...

Trial of Nicotinamide Riboside and Co-enzyme Q10 in Chronic Kidney Disease

Chronic kidney disease is associated with the loss of skeletal muscle mass and function. This process detrimentally impacts mobility, functional independence, and quality of life. Mounting evidence suggests that chronic kidney disease impairs skeletal muscle functioning by injuring mitochondria, the central energy producing units of cells. Potential treatment options to restore mitochondrial function include aerobic and weight bearing exercise and medications that directly imp...

Tecfidera and MRI for Brain Energy in MS

The primary objective of this study is to test the hypothesis that DMF can improve mitochondrial function in the brain of people with MS. The investigators will assess mitochondrial function in the cerebral grey matter by measuring PCr and ATP by 31P magnetic resonance spectroscopy (MRS) and NAA in NAWM by 1H MRS.

Study On the Role of Mitochondrial Dysfunction in the Pathogenesis of Metformin-associated Lactic Acidosis

Metformin is the first line drug of choice for the treatment of type II diabetes. Lactic acidosis can develop as a side effect, especially when renal failure leads to drug accumulation. Lactic acidosis is usually attributed to an abnormal inhibition of hepatic lactate clearance. Growing evidence suggest that metformin can dose-dependently inhibit hepatocyte mitochondrial function. Whether a similar effect occurs in extra-hepatic human tissues remains unknown. The ...

Mitochondrial Functions and Oxidative Stress in ALS Patients

In Amyotrophic Lateral Sclerosis (ALS), malnutrition is frequent (16 to 50 % of the patients) and is an independent prognostic factor. One of the implicated factors is the increase of resting energy expenditure (REE) which can be found in about 50 % of ALS patients. The origin of this hypermetabolism is currently unknown but could be located in the mitochondria. In fact, some studies have found mitochondrial abnormalities and the existence of an oxidative stress. Thus, the aim ...

A Case Control Study to Identify the Role of Epigenetic Regulation of Genes Responsible for Energy Metabolism and Mitochondrial Function in the Obesity Paradox in Cardiac Surgery

This is a prospective, single-center case control comparison of expression of target genes responsible for energy utilization, mitochondrial function and oxidative stress and levels of histone acetylation/ DNA methylation in obese and non-obese adult cardiac surgery patients.

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