Glutaryl-coenzyme A is an intermediate in the metabolism of lysine and tryptophan.[1]
Clinical Significance
Deficiency of glutaryl-CoA dehydrogenase causes glutaric acidemia type 1, an autosomal recessive metabolic disorder[2].In this disorder, impaired metabolism of glutaryl-CoA is associated with elevated levels of organic acids, including glutaric acid and 3-hydroxyglutaric acid [3].
See also
References
- ↑ Rao KS, Albro M, Dwyer TM, Frerman FE (Dec 2006). "Kinetic mechanism of glutaryl-CoA dehydrogenase". Biochemistry. 45 (51): 15853–61. doi:10.1021/bi0609016. PMID 17176108.
- ↑ Hedlund, Gary L.; Longo, Nicola; Pasquali, Marzia (2006-05-15). "Glutaric acidemia type 1". American Journal of Medical Genetics Part C: Seminars in Medical Genetics. 142C (2): 86–94. doi:10.1002/ajmg.c.30088. ISSN 1552-4868. PMC 2556991. PMID 16602100.
- ↑ Hedlund, Gary L.; Longo, Nicola; Pasquali, Marzia (2006-05-15). "Glutaric acidemia type 1". American Journal of Medical Genetics. Part C, Seminars in Medical Genetics. 142C (2): 86–94. doi:10.1002/ajmg.c.30088. ISSN 1552-4868. PMC 2556991. PMID 16602100.