Long-Chain Acyl-CoA Dehydrogenase (Long-Chain-Acyl-Coenzyme A Dehydrogenase)

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.

Also Known As:

Long-Chain-Acyl-Coenzyme A Dehydrogenase; VLCAD; Acyl-CoA Dehydrogenase, Very-Long-Chain; Acyl-CoA Dehydrogenase, Long-Chain; Decanoyl CoA Dehydrogenase; Decanoyl CoA-Dehydrogenase; Very-Long-Chain Acyl-CoA Dehydrogenase; Acyl CoA Dehydrogenase, Long Chain; CoA Dehydrogenase, Decanoyl; CoA-Dehydrogenase, Decanoyl; Dehydrogenase, Decanoyl CoA; Dehydrogenase, Long-Chain Acyl-CoA; Dehydrogenase, Long-Chain-Acyl-CoA; Dehydrogenase, Long-Chain-Acyl-Coenzyme A; Dehydrogenase, Very-Long-Chain Acyl-CoA; Long Chain Acyl CoA Dehydrogenase; Long Chain Acyl Coenzyme A Dehydrogenase; Very Long Chain Acyl CoA Dehydrogenase; Long-Chain-Acyl-CoA Dehydrogenase

Networked: 240 relevant articles (5 outcomes, 12 trials/studies)

Relationship Network

Bio-Agent Context: Research Results

Experts

1.	Spiekerkoetter, Ute: 16 articles (01/2020 - 09/2003)
2.	Tucci, Sara: 12 articles (01/2020 - 11/2010)
3.	Yamaguchi, Seiji: 10 articles (01/2022 - 02/2002)
4.	Vockley, Jerry: 8 articles (01/2022 - 08/2006)
5.	Mohsen, Al-Walid: 7 articles (01/2022 - 05/2013)
6.	Shigematsu, Yosuke: 6 articles (11/2022 - 01/2004)
7.	Yamada, Kenji: 6 articles (01/2022 - 06/2018)
8.	Wanders, Ronald J A: 6 articles (01/2020 - 07/2004)
9.	Strauss, Arnold W: 6 articles (02/2014 - 09/2003)
10.	Spiekerkoetter, U: 6 articles (01/2012 - 05/2003)

Related Diseases

1.	VLCAD deficiency 08/01/2006 - "The development of a safe, durable, and effective VLCAD gene delivery system for use at the time of diagnosis could result in a significant improvement in the quality and duration of life for patients with VLCAD deficiency. " 01/01/2017 - "Very long-chain acyl-CoA dehydrogenase (VLCAD-) deficiency-studies on treatment effects and long-term outcomes in mouse models." 06/01/2005 - "The VLCAD-deficient mouse exhibits similar clinical and biochemical phenotypes to those observed in humans and, therefore, represents an excellent model to study VLCAD deficiency. " 11/01/2023 - "Very long-chain acyl-CoA dehydrogenase (VLCAD) deficiency (VLCADD) is a relatively common inborn error of metabolism, but due to difficulty in accurately predicting affected status through newborn screening, molecular confirmation of the causative variants by sequencing of the ACADVL gene is necessary. " 10/25/2023 - "With the patient's informed consent, acyl-CoA dehydrogenase very long-chain (ACADVL) gene analysis revealed compound heterozygous mutations of c.1332G>A in exon 13 and c.1349G>A (p.R450H) in exon 14. "
2.	Insulin Resistance 01/01/2017 - "In contrast, male VLCAD-/- mice seemed to be protected and displayed only signs of insulin resistance. " 07/01/2015 - "These findings are striking since the first signs of insulin resistance already occur in female VLCAD-/- mice during their reproductive period. " 07/01/2015 - "However, only VLCAD-/- female mice developed a severe metabolic syndrome characterized by marked insulin resistance, dyslipidemia, severe hepatic and visceral steatosis, whereas VLCAD-/- males seemed to be protected and only presented with milder insulin resistance. " 10/23/2007 - "To determine whether primary defects in mitochondrial beta-oxidation can cause insulin resistance, we studied mice with a deficiency of long-chain acyl-CoA dehydrogenase (LCAD), a key enzyme in mitochondrial fatty acid oxidation. " 10/15/2019 - "Liraglutide treatment improved insulin sensitivity, accompanied with reduced expression of the phosphorylated Acetyl-CoA carboxylase-2 (ACC2) and upregulation of long chain acyl CoA dehydrogenase (LCAD) in insulin sensitive tissues. "
3.	Fatty Liver 07/01/2015 - "However, only VLCAD-/- female mice developed a severe metabolic syndrome characterized by marked insulin resistance, dyslipidemia, severe hepatic and visceral steatosis, whereas VLCAD-/- males seemed to be protected and only presented with milder insulin resistance. " 10/20/2017 - "Adenovirus-mediated expression of SIRT3 or an acetylation-defective SIRT3-K57R mutant in diet-induced obese mice decreased acetylation of mitochondrial long-chain acyl-CoA dehydrogenase, a known SIRT3 deacetylation target; improved fatty acid β-oxidation; and ameliorated liver steatosis and glucose intolerance. " 12/01/2012 - "Herein, we observed that (a) Se treatment induced insulin-like effects by lowering the serum glucose level in rats; (b) Se-treated rats showed significance values decreases in abdominal fat mass, adipocyte size, and adiponectin, which are associated with lipid metabolism; (c) Se treatment led to reduced levels of cholesterol, triglycerides, low-density lipoprotein (LDL), and high-density lipoprotein (HDL) cholesterol; (d) fat tissue in Se-treated rats displayed significantly lower expression of adipocyte marker genes along with increased expression of VLCAD and MCAD; and (e) fatty liver formation and β-oxidation gene expression were both significantly reduced in liver tissue of Se-treated rats. "
4.	Respiratory Insufficiency (Respiratory Failure) 01/01/2019 - "In summary, stress with exercise and fasting induces respiratory insufficiency in VLCAD-/- mice and a single injection with AAV9-VLCAD gene therapy ameliorates breathing." 01/01/2019 - "During hypercapnia, VLCAD -/- mice had a significantly lower frequency, tidal volume, minute ventilation, and peak inspiratory and expiratory flow, all of which indicate respiratory insufficiency. " 01/01/2019 - "Fatty acids are a major source of energy during catabolic stress, so the absence of VLCAD can result in a metabolic crises and respiratory insufficiency. " 02/01/2008 - "We report the case of a 7-month-old infant with long-chain acyl-coenzyme A dehydrogenase deficiency who was admitted to the hospital because of respiratory failure and in whom radiographs revealed simultaneous pneumomediastinum and pneumoperitoneum. "
5.	Glucose Intolerance 10/20/2017 - "Adenovirus-mediated expression of SIRT3 or an acetylation-defective SIRT3-K57R mutant in diet-induced obese mice decreased acetylation of mitochondrial long-chain acyl-CoA dehydrogenase, a known SIRT3 deacetylation target; improved fatty acid β-oxidation; and ameliorated liver steatosis and glucose intolerance. "

Related Drugs and Biologics

1.	Fatty Acids (Saturated Fatty Acids)
2.	Triglycerides (Triacylglycerol)
3.	Insulin (Novolin)
4.	Sirtuin 3
5.	Acetyl Coenzyme A (Acetyl-CoA)
6.	Liraglutide
7.	Carnitine O-Palmitoyltransferase (Carnitine Palmitoyltransferase II)
8.	Carnitine (L-Carnitine)
9.	Transferases
10.	Bezafibrate

Related Therapies and Procedures

1.	Therapeutics
2.	Cell- and Tissue-Based Therapy (Cell Therapy)
3.	Intravenous Administration
4.	Extracorporeal Membrane Oxygenation
5.	Aftercare (After-Treatment)