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 25 free, full-text research articles on human participants. First 3 results:

UX007 for the treatment of long chain-fatty acid oxidation disorders: Safety and efficacy in children and adults following 24weeks of treatment.
 

Author(s): J Vockley, B Burton, G T Berry, N Longo, J Phillips, A Sanchez-Valle, P Tanpaiboon, S Grunewald, E Murphy, R Humphrey, J Mayhew, A Bowden, L Zhang, J Cataldo, D L Marsden, E Kakkis

Journal: Mol. Genet. Metab.. 2017 Apr;120(4):370-377.

 

Long-chain fatty acid oxidation disorders (LC-FAOD) lead to accumulation of high concentrations of potentially toxic fatty acid intermediates. Newborn screening and early intervention have reduced mortality, but most patients continue to experience frequent hospitalizations and significant ...

Last Updated: 31 Dec 1969

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Living on the edge: substrate competition explains loss of robustness in mitochondrial fatty-acid oxidation disorders.
 

Author(s): Karen van Eunen, Catharina M L Volker-Touw, Albert Gerding, Aycha Bleeker, Justina C Wolters, Willemijn J van Rijt, Anne-Claire M F Martines, Klary E Niezen-Koning, Rebecca M Heiner, Hjalmar Permentier, Albert K Groen, Dirk-Jan Reijngoud, Terry G J Derks, Barbara M Bakker

Journal:

 

Defects in genes involved in mitochondrial fatty-acid oxidation (mFAO) reduce the ability of patients to cope with metabolic challenges. mFAO enzymes accept multiple substrates of different chain length, leading to molecular competition among the substrates. Here, we combined computational ...

Last Updated: 31 Dec 1969

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Triheptanoin treatment in patients with pediatric cardiomyopathy associated with long chain-fatty acid oxidation disorders.
 

Author(s): J Vockley, J Charrow, J Ganesh, M Eswara, G A Diaz, E McCracken, R Conway, G M Enns, J Starr, R Wang, J E Abdenur, J Sanchez-de-Toledo, D L Marsden

Journal: Mol. Genet. Metab.. 2016 11;119(3):223-231.

 

Long-chain fatty acid oxidation disorders (LC-FAOD) can cause cardiac hypertrophy and cardiomyopathy, often presenting in infancy, typically leading to death or heart transplant despite ongoing treatment. Previous data on triheptanoin treatment of cardiomyopathy in LC-FAOD suggested ...

Last Updated: 31 Dec 1969

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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 7 free, full-text review articles on human participants. First 3 results:

Fatty Acid Oxidation and Its Relation with Insulin Resistance and Associated Disorders.
 

Author(s): Gary D Lopaschuk

Journal: Ann. Nutr. Metab.. 2016 ;68 Suppl 3():15-20.

 

Alterations in muscle fatty acid metabolism have been implicated in mediating the severity of insulin resistance. In the insulin resistant heart fatty acids are favored as an energy source over glucose, which is thus associated with increased fatty acid oxidation, and an overall decrease ...

Last Updated: 31 Dec 1969

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Mitochondrial dysfunction in fatty acid oxidation disorders: insights from human and animal studies.
 

Author(s): Moacir Wajner, Alexandre Umpierrez Amaral

Journal:

 

Mitochondrial fatty acid oxidation (FAO) plays a pivotal role in maintaining body energy homoeostasis mainly during catabolic states. Oxidation of fatty acids requires approximately 25 proteins. Inherited defects of FAO have been identified in the majority of these proteins and constitute ...

Last Updated: 31 Dec 1969

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Long-term major clinical outcomes in patients with long chain fatty acid oxidation disorders before and after transition to triheptanoin treatment--A retrospective chart review.
 

Author(s): Jerry Vockley, Deborah Marsden, Elizabeth McCracken, Stephanie DeWard, Amanda Barone, Kristen Hsu, Emil Kakkis

Journal: Mol. Genet. Metab.. ;116(1-2):53-60.

 

Long chain fatty acid oxidation disorders (LC-FAODs) are caused by defects in the metabolic pathway that converts stored long-chain fatty acids into energy, leading to a deficiency in mitochondrial energy production during times of physiologic stress and fasting. Severe and potentially ...

Last Updated: 31 Dec 1969

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Symptoms, Diagnosis, and Treatment

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Clinical Trial Information This information is provided by ClinicalTrials.gov

Last Updated: 6 Dec 2017

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