Long-chain acyl-CoA dehydrogenase deficiency

Also Known As:

Acyl-Coa Dehydrogenase, Long-Chain, Deficiency of

Networked: 14 relevant articles (0 outcomes, 0 trials/studies)

Disease Context: Research Results

Congenital, Hereditary, and Neonatal Diseases and Abnormalities: 933
- Inborn Genetic Diseases: 11939
  - Inborn Errors Metabolism: 1162
    - Inborn Errors Lipid Metabolism
      - Long-chain acyl-CoA dehydrogenase deficiency: 14
Nutritional and Metabolic Diseases: 15
- Metabolic Diseases: 10142
  - Inborn Errors Metabolism: 1162
    - Inborn Errors Lipid Metabolism
      - Long-chain acyl-CoA dehydrogenase deficiency: 14
  - Lipid Metabolism Disorders: 898
    - Inborn Errors Lipid Metabolism
      - Long-chain acyl-CoA dehydrogenase deficiency: 14
Enzymes and Coenzymes: 1
- Enzymes: 152586
  - Oxidoreductases: 13671
    - Oxidoreductases Acting on CH-CH Group Donors
      - Acyl-CoA Dehydrogenases: 26
        Long-Chain Acyl-CoA Dehydrogenase: 240
        Long-chain acyl-CoA dehydrogenase deficiency: 14
Amino Acids, Peptides, and Proteins: 1
- Proteins: 484843
  - Flavoproteins: 234
    - Long-Chain Acyl-CoA Dehydrogenase: 240
      - Long-chain acyl-CoA dehydrogenase deficiency: 14

Related Diseases

1.	Medium chain acyl CoA dehydrogenase deficiency
2.	Multiple Acyl Coenzyme A Dehydrogenase Deficiency
3.	Zellweger Syndrome (Zellweger's Syndrome)
4.	Argininosuccinic Aciduria
5.	Propionic Acidemia

Experts

1.	Buch, Astrid Emilie: 1 article (01/2019)
2.	Lund, Allan Meldgaard: 1 article (01/2019)
3.	Madsen, Karen Lindhardt: 1 article (01/2019)
4.	Poulsen, Nanna Scharff: 1 article (01/2019)
5.	Stemmerik, Mads Godtfeldt: 1 article (01/2019)
6.	Vissing, John: 1 article (01/2019)
7.	Alcorn, John F: 1 article (04/2014)
8.	Beck, Megan E: 1 article (04/2014)
9.	Bharathi, Sivakama S: 1 article (04/2014)
10.	Chen, Rimei: 1 article (04/2014)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Long-chain acyl-CoA dehydrogenase deficiency:

1.	Fatty Acids (Saturated Fatty Acids)IBA 03/15/1993 - "Detection and assessment by positron emission tomography of a genetically determined defect in myocardial fatty acid utilization (long-chain acyl-CoA dehydrogenase deficiency)." 03/01/1991 - "Complementation assays demonstrated that the patient's fibroblasts complemented fibroblast lines from all known defects in fatty acid oxidation except long-chain acyl-CoA dehydrogenase deficiency. " 12/01/2002 - "Questionnaire responses provided by dietitians specializing in metabolic disorders evaluated practices used for treatment of fatty acid oxidation disorders, medium-chain acyl-CoA dehydrogenase deficiency (MCAD), very-long-chain acyl-CoA dehydrogenase deficiency (VLCAD), long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency (LCHAD), long-chain acyl-CoA dehydrogenase deficiency (LCAD), and Trifunctional Protein deficiency (TFP). " 01/01/1996 - "To determine the importance of peroxisomes and mitochondria in hydroxyeicosatetraenoic acid (HETE) oxidation in vivo, urinary excretion of 12- and 15-HETE was measured in eight patients with a peroxisome deficiency disorder (Zellweger syndrome) showing normal mitochondrial beta-oxidation capacity, in three patients with a defect of mitochondrial long-chain fatty acid oxidation (long-chain acyl-CoA dehydrogenase deficiency), and in eight healthy subjects. "
2.	Long-Chain Acyl-CoA Dehydrogenase (Long-Chain-Acyl-Coenzyme A Dehydrogenase)IBA 09/01/1991 - "Immunochemical characterization of variant long-chain acyl-CoA dehydrogenase in cultured fibroblasts from nine patients with long-chain acyl-CoA dehydrogenase deficiency." 12/01/2002 - "Questionnaire responses provided by dietitians specializing in metabolic disorders evaluated practices used for treatment of fatty acid oxidation disorders, medium-chain acyl-CoA dehydrogenase deficiency (MCAD), very-long-chain acyl-CoA dehydrogenase deficiency (VLCAD), long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency (LCHAD), long-chain acyl-CoA dehydrogenase deficiency (LCAD), and Trifunctional Protein deficiency (TFP). "
3.	TransferasesIBA 04/01/2011 - "Sixty-four children (11.6%) were confirmed with IEM by the MS/MS, including 33 cases of methylmalonic acidemia or propionic acidemias, 2 cases of phenylketonuria, 3 cases of carnitine palmotoyl transferase I deficiency, 1 case of long-chain acyl-CoA dehydrogenase deficiency, 2 cases of medium-chain acyl-CoA dehydrogenase deficiency, 6 cases of maple syrup urine disease, 2 cases of short-chain acyl-CoA dehydrogenase deficiency, 2 cases of glutaric acidemia type I, 2 cases of isovaleric acidemia, 2 cases of homocystinuria, 4 cases of carnitine deficiency, 1 case of tyrosinemia, 1 case of argininosuccinic aciduria, 2 cases of citrullinemia and 1 case of argininemia. " 01/01/1987 - "Activities in the different disorders were (percent control): short-chain acyl-coenzyme A (CoA) dehydrogenase deficiency (115%), medium chain acyl-CoA dehydrogenase deficiency (18%), long-chain acyl-CoA dehydrogenase deficiency (28%), multiple acyl-CoA dehydrogenation disorder, mild and severe variants (49% and 7%), and palmityl-carnitine transferase deficiency (4%). "
4.	Carnitine (L-Carnitine)FDA LinkGeneric 04/01/2011 - "Sixty-four children (11.6%) were confirmed with IEM by the MS/MS, including 33 cases of methylmalonic acidemia or propionic acidemias, 2 cases of phenylketonuria, 3 cases of carnitine palmotoyl transferase I deficiency, 1 case of long-chain acyl-CoA dehydrogenase deficiency, 2 cases of medium-chain acyl-CoA dehydrogenase deficiency, 6 cases of maple syrup urine disease, 2 cases of short-chain acyl-CoA dehydrogenase deficiency, 2 cases of glutaric acidemia type I, 2 cases of isovaleric acidemia, 2 cases of homocystinuria, 4 cases of carnitine deficiency, 1 case of tyrosinemia, 1 case of argininosuccinic aciduria, 2 cases of citrullinemia and 1 case of argininemia. " 01/01/1987 - "Activities in the different disorders were (percent control): short-chain acyl-coenzyme A (CoA) dehydrogenase deficiency (115%), medium chain acyl-CoA dehydrogenase deficiency (18%), long-chain acyl-CoA dehydrogenase deficiency (28%), multiple acyl-CoA dehydrogenation disorder, mild and severe variants (49% and 7%), and palmityl-carnitine transferase deficiency (4%). "
5.	Acyl Coenzyme A (Acyl CoA)IBA 01/01/1993 - "Several syndromes of impaired FFA beta oxidation were taken into account and discarded successively after laboratory investigations: systemic carnitine deficiency, Medium and Long Chain Acyl-CoA Dehydrogenase deficiency and Multiple Acyl CoA Dehydrogenation deficiency (Glutaric aciduria, Ethylmalonic-adipic aciduria and riboflavin-responsive multiple acyl CoA dehydrogenation deficiency). " 01/01/1987 - "Activities in the different disorders were (percent control): short-chain acyl-coenzyme A (CoA) dehydrogenase deficiency (115%), medium chain acyl-CoA dehydrogenase deficiency (18%), long-chain acyl-CoA dehydrogenase deficiency (28%), multiple acyl-CoA dehydrogenation disorder, mild and severe variants (49% and 7%), and palmityl-carnitine transferase deficiency (4%). "
6.	Acyl-CoA DehydrogenasesIBA 02/14/1992 - "Fibroblasts from patients with long-chain acyl-CoA dehydrogenase deficiency were found to oxidize [1-14C]linoleate at an average rate of 60% of normal but [9,10(n)-3H]myristate at an average rate of only 37% of normal, a relationship reverse from that predicted by the chain-length specificities of the three known straight-chain mitochondrial acyl-CoA dehydrogenases. "
7.	Myristic Acid (Tetradecanoic Acid)IBA 02/14/1992 - "Fibroblasts from patients with long-chain acyl-CoA dehydrogenase deficiency were found to oxidize [1-14C]linoleate at an average rate of 60% of normal but [9,10(n)-3H]myristate at an average rate of only 37% of normal, a relationship reverse from that predicted by the chain-length specificities of the three known straight-chain mitochondrial acyl-CoA dehydrogenases. "
8.	Linoleic Acid (Linoleate)IBA 02/14/1992 - "Fibroblasts from patients with long-chain acyl-CoA dehydrogenase deficiency were found to oxidize [1-14C]linoleate at an average rate of 60% of normal but [9,10(n)-3H]myristate at an average rate of only 37% of normal, a relationship reverse from that predicted by the chain-length specificities of the three known straight-chain mitochondrial acyl-CoA dehydrogenases. "
9.	Riboflavin (Vitamin B2)FDA LinkGeneric 01/01/1993 - "Several syndromes of impaired FFA beta oxidation were taken into account and discarded successively after laboratory investigations: systemic carnitine deficiency, Medium and Long Chain Acyl-CoA Dehydrogenase deficiency and Multiple Acyl CoA Dehydrogenation deficiency (Glutaric aciduria, Ethylmalonic-adipic aciduria and riboflavin-responsive multiple acyl CoA dehydrogenation deficiency). "
10.	Pulmonary Surfactants (Pulmonary Surfactant)IBA 04/11/2014 - "Long-chain acyl-CoA dehydrogenase deficiency as a cause of pulmonary surfactant dysfunction."