... abnormal mitochondria in the myocardium we have not yet succeeded in obtaining convincing evidence of any functional impairment of the heart ...

... Inborn errors of metabolism consist of numerous infiltrative storage diseases, abnormal energy production, biochemical deficiencies and disorders related to toxic ...

HIV Infections; Lipid Metabolism; Glucose Metabolism; Metabolic Abnormality; Lipodystrophy; Cardiovascular Disease

This book contains eight parts, each consisting of several papers. The part titles are: The Heritage of Sir Archibald Garrod; New Approaches to the Diagnosis and Treatment of Genetic Disease; Achievements, New Trends, and Policies in the Detection of Inborn Errors of Metabolism; Disorders of Amino Acid Metabolism; Diseases of Energy ...

The prevalence of obesity and related metabolic abnormalities in children and adolescents has escalated. Energy expenditure has been physiologically associated with thyroid hormones and adiposity-related hormones such as leptin and insulin. Genetic factors contribute to the regulation of energy expe...

HIV infection is associated with abnormal lipid metabolism, body fat redistribution, and altered energy expenditure. The pathogenesis of these complex abnormalities is unclear. Viral protein R (Vpr), an HIV-1 accessory protein, can regulate gene transcription mediated by the glucocorticoid receptor ...

Nuclear receptors (NR) are a superfamily of ligand-activated transcription factors that regulate development, reproduction, and metabolism of lipids, drugs and energy. The importance of this family of proteins in metabolic disease is exemplified by NR ligands used in the clinic or under exploratory development for the treatment of ...

Interest in the control feeding and has increased as a result of the obesity epidemic and rising incidence of metabolic diseases. The brain detects alterations in energy stores and triggers metabolic and behavioral responses designed to maintain energy balance. Energy homeostasis is controlled ...

Ophthalmologic manifestations occur in various inborn errors of metabolism (IEM), including small molecule disorders and organelle disorders. In a minority of diseases the occurrence of eye abnormalities could be attributed to direct toxic mechanisms of abnormal metabolic products or accumulation of normal ...

The presence of abnormalities in fibromyalgia muscle using current methodological approaches is well established. The more serious abnormalities are demonstrated by histologic studies particularly on electron microscopy: disorganisation of Z bands and abnormalities in the number and shape of mitochondria. Biochemical studies and P 31 ...

Resuscitation from hemorrhagic shock with fluid (Ringer's Lactate) and blood may not correct all of the metabolic abnormalities associated with shock. Patients who are apparently fully resuscitated may yet develop organ failure syndromes such as respirato...

Mitochondria are subcellular organelles present in cells of all tissues (except sperm) that carry out the oxidative-phosphorylation (OXPHOS) metabolic reactions that are essential for and central to cellular energy production. Abnormal mitochondrial funct...

Autophagy is a lysosomal pathway by which intracellular organelles and proteins are degraded to supply the cell with energy and to maintain cellular homeostasis. Recently, lipid droplets (LDs) have been identified as a substrate for macroautophagy. In addition to the classic pathway of lipid metabolism by cytosolic lipases, LDs are sequestered in ...

1. Lesions in the lateral hypothalamus of rats always produced an immediate increase in total energy expenditure. The increase was maintained for 24 hr or longer only in rats that became and remained aphagic. Rats that showed no recovery from the aphagia and were maintained by tube feeding showed a second, larger increase in metabolic rate after about 7 ...

The diagnosis of inborn errors of metabolism is challenging for most physicians. Improvements in medical technology and greater knowledge of the human genome are resulting in significant changes in the diagnosis, classification, and treatment of inherited metabolic disorders (IMDs). Many known inborn errors of metabolism will be ...

Over the last decades, the negative health consequences of obesity have triggered massive searches for the cause of --and potential solutions for--the excessive mismatch between calorie consumption and expenditure. The concepts of malfunctioning hypothalamic neurotransmission and impaired related neuromodulatory networks in other brain regions have attracted considerable attention as they may ...

SUMMARYAge-related neurodegenerative diseases are associated with mild impairment of oxidative metabolism and accumulation of abnormal proteins. Within the cell, the mitochondria appears to be a dominant site for initiation and propagation of disease processes. Shifts in metabolism in response to mild metabolic ...

Many behavioral and physiological processes, including locomotor activity, blood pressure, body temperature, sleep (fasting)/wake (feeding) cycles, and metabolic regulation display diurnal rhythms. The biological clock ensures proper metabolic alignment of energy substrate availability and processing. Studies in animals and humans ...

Many behavioral and physiological processes, including locomotor activity, blood pressure, body temperature, sleep(fasting)/wake(feeding) cycles as well as metabolic regulation display diurnal rhythms. The biological clock ensures proper metabolic alignment of energy substrate availability and processing. Studies in animals and humans ...

Abnormalities of the skeletal and urogenital system are discussed which result from an interference with the metabolism of calcium in cadmium workers.

The possibility of modifying cardiac metabolism by switching the fuel used by the myocardium could become increasingly important. Inhibitors of free fatty acid (FFA) oxidation could have an important role in therapeutic strategy for patients with heart failure, and shifting the energy substrate preference away from FFA metabolism and ...

Resistance to insulin metabolic signaling in adipose tissue contributes to the lipid abnormalities in obese, hyperinsulinemic, insulin-resistant patients who develop the cardiorenal syndrome. These same metabolic dyslipidemic abnormalities can be found in conditions of caloric energy ...

Brain ischemia resulting from multiple disease states including cardiac arrest, stroke and traumatic brain injury, is a leading cause of death and disability. Despite significant resources dedicated to developing pharmacological interventions, few effective therapeutic options are currently available. The basic consequence of cerebral ischemia, characterized by energy failure ...