Mitochondrial Trifunctional Protein

A mitochondrial protein consisting of four alpha-subunits and four beta-subunits. It contains enoyl-CoA hydratase, long-chain-3-hydroxyacyl-CoA dehydrogenase, and acetyl-CoA C-acyltransferase activities and plays an important role in the metabolism of long chain FATTY ACIDS.

Also Known As:

FA beta-OMC; Mitochondrial Fatty Acid beta-Oxidation Trifunctional Protein; Mitochondrial Long Chain Enoyl CoA Hydratase 3 Hydroxyacyl CoA Dehydrogenase 3 Ketoacyl CoA Thiolase Trifunctional Protein; Mitochondrial Fatty Acid beta Oxidation Trifunctional Protein; Trifunctional Protein, Mitochondrial

Networked: 61 relevant articles (0 outcomes, 1 trials/studies)

Bio-Agent Context: Research Results

Experts

1.	Spiekerkoetter, Ute: 7 articles (05/2022 - 01/2004)
2.	Ibdah, Jamal A: 7 articles (01/2018 - 09/2002)
3.	Wanders, Ronald J A: 6 articles (01/2013 - 06/2003)
4.	Bennett, Michael J: 5 articles (02/2004 - 05/2002)
5.	Waterham, Hans R: 4 articles (01/2013 - 06/2005)
6.	Yang, Zi: 4 articles (12/2007 - 09/2002)
7.	Strauss, Arnold W: 4 articles (02/2004 - 09/2002)
8.	Yamaguchi, Seiji: 3 articles (09/2017 - 05/2014)
9.	Wijburg, Frits A: 3 articles (01/2013 - 06/2003)
10.	Zhao, Yiwen: 3 articles (05/2005 - 09/2002)

Related Diseases

1.	Sudden Infant Death (SID) 12/01/2007 - "The goal of the present study was to assess the value of post-mortem molecular analysis for medium-chain acyl-coenzyme A dehydrogenase (MCAD) and mitochondrial trifunctional protein (MTP) defects in unexplained sudden infant death (SID) associated with fatty infiltration of the liver. "
2.	Trifunctional Protein Deficiency With Myopathy And Neuropathy 09/01/2022 - "Outcomes and genotype correlations in patients with mitochondrial trifunctional protein or isolated long chain 3-hydroxyacyl-CoA dehydrogenase deficiency enrolled in the IBEM-IS database." 01/01/2017 - "Diagnosis, Treatment, and Clinical Outcome of Patients with Mitochondrial Trifunctional Protein/Long-Chain 3-Hydroxy Acyl-CoA Dehydrogenase Deficiency." 05/01/2015 - "Here, we report a case of AFLP caused by fetal mitochondrial trifunctional protein (TFP) deficiency. " 10/01/2010 - "Before the newborn screening era, disorders of the mitochondrial trifunctional protein (TFP) complex including long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency (LCHADD) presented with high morbidity and mortality. " 06/01/2005 - "As tandem mass spectrometry (TMS) does not discriminate between the different defects of the mitochondrial trifunctional protein (MTP) screening for isolated LCHAD deficiency includes the detection of long-chain 3-ketoacyl-CoA thiolase and complete MTP deficiencies as well. "
3.	Rhabdomyolysis 10/01/2016 - "The pathogenesis of the muscular symptoms and recurrent rhabdomyolysis that are commonly manifested in patients with mitochondrial trifunctional protein (MTP) and long-chain 3-hydroxy-acyl-CoA dehydrogenase (LCHAD) deficiencies is still unknown. " 03/01/2001 - "Deficiency of mitochondrial trifunctional protein (TP), beta-oxidation enzyme, is characterized by recurrent rhabdomyolysis in adult patients. " 01/01/2021 - "Charcot-Marie-Tooth Disease With Episodic Rhabdomyolysis Due to Two Novel Mutations in the β Subunit of Mitochondrial Trifunctional Protein and Effective Response to Modified Diet Therapy." 11/01/2022 - "Recurrent Rhabdomyolysis, Acute Kidney Injury, and Foot Drop: A Rare Case with Mitochondrial Trifunctional Protein (MTP) Deficiency." 01/01/2005 - "General mitochondrial trifunctional protein (TFP) deficiency leads to a wide clinical spectrum of disease ranging from severe neonatal/infantile cardiomyopathy and early death to mild chronic progressive sensorimotor poly-neuropathy with episodic rhabdomyolysis. "
4.	Cardiomyopathies (Cardiomyopathy) 05/01/2022 - "Mitochondrial trifunctional protein (MTP) deficiency is an ultrarare hereditary recessive disorder causing a broad spectrum of phenotypes with lethal infantile cardiomyopathy at the most severe end. " 12/01/2015 - "Fetal left ventricular noncompaction cardiomyopathy and fatal outcome due to complete deficiency of mitochondrial trifunctional protein." 12/01/2021 - "Mitochondrial trifunctional protein (TFP) deficiency is an autosomal recessive disorder that causes a clinical spectrum of diseases ranging from severe infantile cardiomyopathy to mild chronic progressive neuromyopathy, however, parathyroid glands, hematologic system and kidney damage are not the common presentations of this disease. " 08/01/2008 - "Intrauterine cardiomyopathy and cardiac mitochondrial proliferation in mitochondrial trifunctional protein (TFP) deficiency." 01/01/2005 - "General mitochondrial trifunctional protein (TFP) deficiency leads to a wide clinical spectrum of disease ranging from severe neonatal/infantile cardiomyopathy and early death to mild chronic progressive sensorimotor poly-neuropathy with episodic rhabdomyolysis. "
5.	Peripheral Nervous System Diseases (PNS Diseases) 01/01/2021 - "The spectrum of peripheral neuropathy in disorders of the mitochondrial trifunctional protein." 05/01/2022 - "Axonal peripheral neuropathy is a common complication of mitochondrial trifunctional protein (MTP) deficiency and long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency that is usually considered progressive. " 01/01/2018 - "A combination of unexplained peripheral neuropathy, hypoparathyroidism, and the inability to cope with metabolic stress could point to a rare inborn error of metabolism, such as mitochondrial trifunctional protein (MTP) deficiency.Here" 01/01/2004 - "Peripheral neuropathy, episodic myoglobinuria, and respiratory failure in deficiency of the mitochondrial trifunctional protein."

Related Drugs and Biologics

1.	Acyl-CoA Dehydrogenase (Medium-Chain Acyl-Coenzyme A Dehydrogenase)
2.	Fatty Acids (Saturated Fatty Acids)
3.	3-Hydroxyacyl-CoA Dehydrogenase
4.	Long-Chain Acyl-CoA Dehydrogenase (Long-Chain-Acyl-Coenzyme A Dehydrogenase)
5.	Oxidoreductases (Dehydrogenase)
6.	Enzymes
7.	Coenzyme A (CoA)
8.	Butyryl-CoA Dehydrogenase (Short-Chain Acyl-CoA Dehydrogenase)
9.	Cytochrome P-450 CYP2E1 (CYP2E1)
10.	Triglycerides (Triacylglycerol)

Related Therapies and Procedures

1.	Diet Therapy (Therapy, Diet)