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

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

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

Propionyl-CoA carboxylase (PCC): A mitochondrial biotin-containing enzyme that catalyzes conversion of propionyl-CoA to D-methylmalonyl-CoA (see FIG. 1 ). A ...

ANAPLEROTIC THERAPY IN PROPIONIC ACIDEMIA - PMC - NCBI

... treatment with anaplerotic agents ... Treatment of cardiomyopathy and rhabdomyolysis in long-chain fat oxidation disorders using an anaplerotic ...

Anaplerotic therapy in propionic acidemia - ScienceDirect.com

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

WO2017184583A1 - Agents anaplérotiques pour le traitement de ...

This disclosure concerns the treatment of disorders of propionate metabolism and long chain fatty acid oxidation by administration of anaplerotic agents ...

WO 2017/184583 A1 - googleapis.com

(54) Title: ANAPLEROTIC AGENTS FOR TREATMENT OF DISORDERS OF PROPIONATE AND LONG CHAIN FAT. METABOLISM. H. H. I. H. I. FIG. 5. OH. O. OH оо. OH.

Biochemical and anaplerotic applications of in vitro models of ...

Analysis of TCA cycle intermediates by GC–MS/MS indicated that propionate may inhibit enzymes of the TCA cycle as shown in PA, but is also incorporated in the ...

Medium branched chain fatty acids improve the profile of ... - NCBI

Triheptanoin (commercially known as Dojolvi; C7G), has been approved as an anaplerotic drug treatment for long chain fatty acid β-oxidation (FAO) ...

Anaplerotic therapy in propionic acidemia | Request PDF

Request PDF | Anaplerotic therapy in propionic acidemia | Background: Propionic acidemia is a rare metabolic disorder caused by a deficiency of propionyl- ...

Al-Walid A. Mohsen, Ph.D. | Department of Pediatrics | University of ...

Anaplerotic Agents for Treatment of Disorders of Propionate and Long Chain Fat Metabolism, Pitt# 03786; PCT/US2017/028100, filing date April 04, 2017 (under ...

Anaplerotic reactions - Wikipedia

Anaplerosis is the act of replenishing TCA cycle intermediates that have been extracted for biosynthesis (in what are called anaplerotic reactions). The TCA ...

relevance to disorders of propionyl-CoA metabolism

... anaplerotic agent for the acute treatment of propionic acidemia. However, the present study shows that in livers perfused with propionate + 3HP, there is an ...

Biomarkers for drug development in propionic and methylmalonic ...

Changes in primary metabolites such as methylcitric acid (MCA), MCA:citric acid ratio, oxidation of 13C-propionate (exhaled 13CO2), and ...

WO-2020077094-A1 - Tca Cycle Intermediates and Method of Use ...

... treating metabolic disorders ... University of Pittsburgh. Title: Anaplerotic Agents for Treatment of Disorders of Propionate and Long Chain Fat Metabolism ...

Pyruvate-Carboxylase-Mediated Anaplerosis Promotes Antioxidant ...

This dual responsibility requires anaplerotic pathways, such as pyruvate carboxylase (PC), to generate TCA cycle intermediates necessary for ...

potential of anaplerotic triheptanoin for the treatment of long-chain ...

Long-chain fatty acid oxidation disorders are the genetic and metabolic diseases in which the body is unable to break down long-chain fatty acids to make energy ...

Cardiac Complications in Patients with Propionic Acidemia

The biological effect of the inherited deficiency of the anaplerotic propionate, metabolism is aggravated by synergistic secondary effects of ...

Anaplerotic diet therapy in inherited metabolic disease

Pyruvate carboxylase deficiency: clinical and biochemical response to anaplerotic diet therapy. · Treatment of cardiomyopathy and rhabdomyolysis in long-chain ...

Anaplerotic molecules: Current and future | Journal of Inherited ...

The main anaplerotic substrates are pyruvate, glutamine/glutamate and precursors of propionyl-CoA (odd-chain fatty acids, specific amino acids, C5-ketone bodies) ...

Biochemical and anaplerotic applications of in vitro models of ...

Analysis of TCA cycle intermediates by GC-MS/MS indicated that propionate may inhibit enzymes of the TCA cycle as shown in PA, but is also incorporated in the ...

Anaplerotic Therapy in Propionic Acidemia - ClinicalTrials.Veeva

The objective of this project is to define whether nutritional supplements (ornithine alpha-ketoglutarate, glutamine, or citrate) capable of ...