Clinical Outcome of Triheptanoin Treatment in Patients With Long-chain Fatty Acid Oxidation Disorders (LC-FAOD) Treated Under Expanded Access Program

2018-12-13 02:49:11 | BioPortfolio

Published on BioPortfolio: 2018-12-13T02:49:11-0500

Clinical Trials [2835 Associated Clinical Trials listed on BioPortfolio]

Study of Triheptanoin for Treatment of Long-Chain Fatty Acid Oxidation Disorder

Humans eat long-chain fat in their diet and use it for energy during exercise and during periods of fasting. Patients with long-chain fatty acid oxidation disorders cannot use dietary fat ...

Fatty Acid Oxidation Defects and Insulin Sensitivity

The purpose of this study is to learn more about what causes insulin resistance. It has been suggested that proper breakdown of fat into energy (oxidation) in the body is important to all...

Individual Patient Compassionate Use of Triheptanoin

Individual patient expanded access requests may be considered for patients who have no other treatment options

Nutritional Therapy of the Deficits of Oxidation Mitochondrial of the Fatty Acids

Usual dietary therapies of mitochondrial fatty acid oxidation disorders (FAO) are based on 3 strategies: - limitation of lipid intake in the diet; - supplementation of the di...

Experiment 2: Understanding Liver Fat Metabolism

There is a largely unsubstantiated thought in the literature that dietary unsaturated enter oxidation pathways preferentially compared to saturated fatty acids. This study is undertaking a...

PubMed Articles [26865 Associated PubMed Articles listed on BioPortfolio]

Fatty Acid Oxidation in Macrophages and T Cells: Time for Reassessment?

Previous immunometabolism research using the CPT1 inhibitor etomoxir suggests that long-chain fatty acid oxidation (LC-FAO) supports IL-4-driven alternative macrophage activation (M(IL-4)) and regulat...

β-Oxidation in ghrelin-producing cells is important for ghrelin acyl-modification.

Ghrelin is a unique fatty acid-modified peptide hormone produced in the stomach and has important roles in energy homeostasis and gastrointestinal motility. However, the medium-chain fatty acid source...

Non-enzymatic conversion of primary oxidation products of Docosahexaenoic acid into less toxic acid molecules.

Docosahexaenoic acid (DHA) is long chain omega-3 fatty acid with known health benefits and clinical significance. However, 4-hydroxy hexenal (HHE), an enzymatic oxidation product of DHA has recently b...

Fatty acid activation in thermogenic adipose tissue.

Channeling carbohydrates and fatty acids to thermogenic tissues, including brown and beige adipocytes, have garnered interest as an approach for the management of obesity-related metabolic disorders. ...

Biomarker of Long-Chain n-3 Fatty Acid Intake and Breast Cancer: Accumulative Evidence from an Updated Meta-Analysis of Epidemiological Studies.

We aimed to summarize the up-to-date epidemiology evidence on biomarkers of long-chain (LC) n-3 fatty acid (FA) intake in relation to breast cancer (BC).

Medical and Biotech [MESH] Definitions

A long-chain fatty acid ester of carnitine which facilitates the transfer of long-chain fatty acids from cytoplasm into mitochondria during the oxidation of fatty acids.

A perilipin that functions in LIPOGENESIS; LIPOLYSIS; and fatty acid oxidation in BROWN ADIPOSE TISSUE; heart, liver, and skeletal muscle. It recruits MITOCHONDRIA to the surface of LIPID DROPLETS where it functions in both the storage of fatty acids as TRIGLYCERIDES, and their release for mitochondrial fatty acid oxidation in response to metabolic needs.

A flavoprotein oxidoreductase that has specificity for long-chain fatty acids. It forms a complex with ELECTRON-TRANSFERRING FLAVOPROTEINS and conveys reducing equivalents to UBIQUINONE.

A heterogeneous group of inherited metabolic disorders marked by absent or dysfunctional PEROXISOMES. Peroxisomal enzymatic abnormalities may be single or multiple. Biosynthetic peroxisomal pathways are compromised, including the ability to synthesize ether lipids and to oxidize long-chain fatty acid precursors. Diseases in this category include ZELLWEGER SYNDROME; INFANTILE REFSUM DISEASE; rhizomelic chondrodysplasia (CHONDRODYSPLASIA PUNCTATA, RHIZOMELIC); hyperpipecolic acidemia; neonatal adrenoleukodystrophy; and ADRENOLEUKODYSTROPHY (X-linked). Neurologic dysfunction is a prominent feature of most peroxisomal disorders.

Enzymes that reversibly catalyze the oxidation of a 3-hydroxyacyl CoA to 3-ketoacyl CoA in the presence of NAD. They are key enzymes in the oxidation of fatty acids and in mitochondrial fatty acid synthesis. EC

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