Fatty Oxidation Disorders

Common Name(s)

Fatty Oxidation Disorders

Description for this condition is not yet available.
 

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Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Fatty Oxidation Disorders" for support, advocacy or research.

There are currently no organizations listed in Disease InfoSearch that support this condition. Create a listing.

 

 

General Support Organizations

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Scientific Literature

Articles from the PubMed Database

Research articles describe the outcome of a single study. They are the published results of original research.
The terms "Fatty Oxidation Disorders" returned 21 free, full-text research articles on human participants. First 3 results:

Bezafibrate in skeletal muscle fatty acid oxidation disorders: a randomized clinical trial.
 

Author(s): Mette Cathrine Ørngreen, Karen Lindhardt Madsen, Nicolai Preisler, Grete Andersen, John Vissing, Pascal Laforêt

Journal: Neurology. 2014 Feb;82(7):607-13.

 

To assess whether bezafibrate increases fatty acid oxidation (FAO) and lowers heart rate (HR) during exercise in patients with carnitine palmitoyltransferase (CPT) II and very long-chain acyl-CoA dehydrogenase (VLCAD) deficiencies.

Last Updated: 18 Feb 2014

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The effect of valinomycin in fibroblasts from patients with fatty acid oxidation disorders.
 

Author(s): Uzochi Chimdinma Ndukwe Erlingsson, Francesco Iacobazzi, Aiping Liu, Orly Ardon, Marzia Pasquali, Nicola Longo

Journal: Biochem. Biophys. Res. Commun.. 2013 Aug;437(4):637-41.

 

Disorders of the carnitine cycle and of the beta oxidation spiral impair the ability to obtain energy from fats at time of fasting and stress. This can result in hypoketotic hypoglycemia, cardiomyopathy, cardiac arrhythmia and other chronic medical problems. The in vitro study of ...

Last Updated: 12 Aug 2013

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Neuropsychological outcomes in fatty acid oxidation disorders: 85 cases detected by newborn screening.
 

Author(s): Susan E Waisbren, Yuval Landau, Jenna Wilson, Jerry Vockley

Journal: Dev Disabil Res Rev. 2013 ;17(3):260-8.

 

Mitochondrial fatty acid oxidation disorders include conditions in which the transport of activated acyl-Coenzyme A (CoA) into the mitochondria or utilization of these substrates is disrupted or blocked. This results in a deficit in the conversion of fat into energy. Most patients ...

Last Updated: 26 Jun 2013

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Reviews from the PubMed Database

Review articles summarize what is currently known about a disease. They discuss research previously published by others.
The terms "Fatty Oxidation Disorders" returned 4 free, full-text review articles on human participants. First 3 results:

Fatty acid omega-oxidation as a rescue pathway for fatty acid oxidation disorders in humans.
 

Author(s): Ronald J A Wanders, Jasper Komen, Stephan Kemp

Journal: FEBS J.. 2011 Jan;278(2):182-94.

 

Fatty acids (FAs) can be degraded via different mechanisms including α-, β- and ω-oxidation. In humans, a range of different genetic diseases has been identified in which either mitochondrial FA β-oxidation, peroxisomal FA β-oxidation or FA α-oxidation is impaired. Treatment ...

Last Updated: 5 Jan 2011

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Mitochondrial fatty acid oxidation disorders: pathophysiological studies in mouse models.
 

Author(s): Ute Spiekerkoetter, Philip A Wood

Journal: J. Inherit. Metab. Dis.. 2010 Oct;33(5):539-46.

 

Mouse models have been designed for a number of fatty acid oxidation defects. Studies in these mouse models have demonstrated that different pathogenetic mechanisms play a role in the pathophysiology of defects of fatty acid oxidation. Supplementation with L-carnitine does not prevent ...

Last Updated: 29 Sep 2010

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Fetal fatty acid oxidation disorders, their effect on maternal health and neonatal outcome: impact of expanded newborn screening on their diagnosis and management.
 

Author(s): Prem S Shekhawat, Dietrich Matern, Arnold W Strauss

Journal: Pediatr. Res.. 2005 May;57(5 Pt 2):78R-86R.

 

Mitochondrial fatty acid oxidation disorders (FAOD) are recessively inherited errors of metabolism. Newborns with FAOD typically present with hypoketotic hypoglycemia, metabolic acidosis, hepatic failure, and cardiomyopathy. Late presentations include episodic myopathy, neuropathy, ...

Last Updated: 29 Apr 2005

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Symptoms, Diagnosis, and Treatment

There are currently no related results available in GeneReviews.

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Clinical Trial Information This information is provided by ClinicalTrials.gov

Compassionate Use of Triheptanoin (C7) for Fatty Acid Oxidation Disorders and Glycogen Storage Disease
 

Status: Available

Condition Summary: Very Long-chain acylCoA Dehydrogenase (VLCAD) Deficiency; Carnitine Palmitoyltransferase Deficiencies (CPT1, CPT2); Mitochondrial Trifunctional Protein Deficiency; Long-chain Hydroxyacyl-CoA Dehydrogenase Deficiency; Glycogen Storage Disorders; Pyruvate Carboxylase Deficiency Disease; ACYL-CoA DEHYDROGENASE FAMILY, MEMBER 9, DEFICIENCY of

 

Last Updated: 26 May 2015

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Fat Induced Insulin Resistance and Atherosclerosis
 

Status: Recruiting

Condition Summary: Other Fatty Acid Oxidation Disorders; Diabetes Mellitus; Insulin Resistance

 

Last Updated: 27 Jan 2015

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Longitudinal Study of Mitochondrial Hepatopathies
 

Status: Recruiting

Condition Summary: Acute Liver Failure,; Mitochondrial Diseases; End Stage Liver Disease,; Respiratory Chain Deficiencies, Mitochondrial; Disorder of Fatty Acid Oxidation

 

Last Updated: 15 Sep 2014

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