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Anaplerotic agents for treatment of disorders of propionate and long ...

Anaplerotic agents for treatment of disorders of propionate and long ...

Therapeutic Effects of L-Carnitine and Propionyl-L ... - Caldic Magistral

larly important because propionate can be used by mitochondria as an anaplerotic ... Long-term carnitine treatment of chronic anemia of patients with end-stage ...

Secondary mitochondrial dysfunction in propionic aciduria

Biochemically, these disorders are characterized by accumulation of propionyl-CoA and metabolites of alternative propionate oxidation. In the present study, we ...

April 2019 US Patents by Issue Date - Justia Patents Search

ANAPLEROTIC AGENTS FOR TREATMENT OF DISORDERS OF PROPIONATE AND LONG CHAIN FAT METABOLISM. Publication number: 20190117611. Abstract: Methods of treating ...

Shortcomings of Currently Applied - Erasmus University Rotterdam

... disorders as a potential cause of growth retardation: Options for treatment. ... propionic acidemia patients and its negative association with long-term outcome.

Efficacy and Safety of Elamipretide in Individuals With Primary ...

Elamipretide is an investigational mitochondrial-targeting agent in development for treating patients with a variety of mitochondrial diseases. Elamipretide is ...

Citations to Inhibition by propionyl-coenzyme A of N ... - JCI

Long-term N-carbamylglutamate treatment of ... propionate metabolism in rat liver: relevance to disorders of propionyl-CoA metabolism ... Reye's and Reye-Like ...

Systemic Metabolic Abnormalities in Adult-onset Acid Maltase ...

... anaplerotic therapy in this disorder. In conclusion, these observations in patients with adult-onset GSD II suggest that there is a ...

Discovery of Novel Pyruvate Dehydrogenase Kinase 4 Inhibitors for ...

For this reason, we investigated small-molecule PDK4 inhibitors for the treatment of various diseases such as diabetes, allergies, and cancer.

Cellular metabolism regulates the differentiation and function of T ...

... long-chain fatty acids, and very long-chain fatty acids ... treating autoimmune diseases, such as multiple sclerosis and psoriasis.

Propionic Acidemia - MalaCards

Propionic acidemia is an autosomal recessive metabolic disorder caused by defective functioning in the mitochondrial enzyme propionyl CoA carboxylase (PCC), ...

Unsuccessful treatment of severe pyruvate carboxylase deficiency ...

Because the enzyme is involved in gluconeogenesis and anaplerosis of the Krebs cycle, therapeutic strategies have included avoiding fasting and ...

2-hydrazinoquinoline as a novel derivatization agent for

long-term alcohol abuse. For example, alcoholic lactic ... drug treatment, nutrition intervention, and environmental challenge, but also provides a ...

Secondary mitochondrial dysfunction in propionic aciduria - HAL

pathomechanism in inherited disorders of propionate metabolism, i.e. propionic and methylmalonic ... agent propionate. Eur. J. Biochem. 271 ...

Anaplerotic Reactions - Definition, Citric Acid Cycle, Mechanism and ...

Anaplerosis can be defined as the reaction that can replenish the intermediates of the pathway. In simpler terms, anaplerotic reaction maintains the dynamic ...

Anaplerotic therapy in propionic acidemia. - Read by QxMD

However, supplementation with any anaplerotic agents normalized the physiological buffering of ammonia by glutamate, with plasma glutamate and alanine ...

Anaplerotic reactions - wikidoc

Anaplerotic reactions are those that form intermediates of the TCA or citric acid cycle. The malate is created by PEP carboxylase and malate dehydrogenase in ...

Shortcomings of Currently Applied —Dietary— Treatment for ...

UCD are caused by deficiencies of the enzymes and transporter needed for the urea-cycle (figure 1). While all AOA disorders affect protein metabolism, ...