title: The Effect of Mitochondrial Supplements on Mitochondrial Activity in Children with Autism Spectrum Disorder.
abstract: Treatment for mitochondrial dysfunction is typically guided by expert opinion with a paucity of empirical evidence of the effect of treatment on mitochondrial activity. We examined citrate synthase and Complex I and IV activities using a validated buccal swab method in 127 children with autism spectrum disorder with and without mitochondrial disease, a portion of which were on common mitochondrial supplements. Mixed-model linear regression determined whether specific supplements altered the absolute mitochondrial activity as well as the relationship between the activities of mitochondrial components. Complex I activity was increased by fatty acid and folate supplementation, but folate only effected those with mitochondrial disease. Citrate synthase activity was increased by antioxidant supplementation but only for the mitochondrial disease subgroup. The relationship between Complex I and IV was modulated by folate while the relationship between Complex I and Citrate Synthase was modulated by both folate and B12. This study provides empirical support for common mitochondrial treatments and demonstrates that the relationship between activities of mitochondrial components might be a marker to follow in addition to absolute activities. Measurements of mitochondrial activity that can be practically repeated over time may be very useful to monitor the biochemical effects of treatments.
authors.affiliation: Arkansas Children's Research Institute, Little Rock, AR 72202, USA. lmdelhey@uams.edu.|Department of Pediatrics, University of Arkansas for Medical Sciences, Little Rock, AR 72202, USA. lmdelhey@uams.edu.
authors.forename: Leanna M
authors.initials: LM
authors.lastname: Delhey
authors.affiliation: Arkansas Children's Research Institute, Little Rock, AR 72202, USA. ekimkilinc@gmail.com.
authors.forename: Ekim
authors.initials: E
authors.lastname: Nur Kilinc
authors.affiliation: Child and Adolescent Department, Mental Health Centre, West China Hospital of Sichuan University, Chengdu 610041, China. dr.yinli@hotmail.com.
authors.forename: Li
authors.initials: L
authors.lastname: Yin
authors.affiliation: Arkansas Children's Research Institute, Little Rock, AR 72202, USA. jcslattery@uams.edu.|Department of Pediatrics, University of Arkansas for Medical Sciences, Little Rock, AR 72202, USA. jcslattery@uams.edu.
authors.forename: John C
authors.initials: JC
authors.lastname: Slattery
authors.affiliation: Arkansas Children's Research Institute, Little Rock, AR 72202, USA. mltippett@uams.edu.|Department of Pediatrics, University of Arkansas for Medical Sciences, Little Rock, AR 72202, USA. mltippett@uams.edu.
authors.forename: Marie L
authors.initials: ML
authors.lastname: Tippett
authors.affiliation: Arkansas Children's Research Institute, Little Rock, AR 72202, USA. srose@uams.edu.|Department of Pediatrics, University of Arkansas for Medical Sciences, Little Rock, AR 72202, USA. srose@uams.edu.
authors.forename: Shannon
authors.initials: S
authors.lastname: Rose
authors.affiliation: Arkansas Children's Research Institute, Little Rock, AR 72202, USA. scbennutri@uams.edu.|Department of Pediatrics, University of Arkansas for Medical Sciences, Little Rock, AR 72202, USA. scbennutri@uams.edu.
authors.forename: Sirish C
authors.initials: SC
authors.lastname: Bennuri
authors.affiliation: Arkansas Children's Research Institute, Little Rock, AR 72202, USA. kahlerstepheng@uams.edu.|Department of Pediatrics, University of Arkansas for Medical Sciences, Little Rock, AR 72202, USA. kahlerstepheng@uams.edu.
authors.forename: Stephen G
authors.initials: SG
authors.lastname: Kahler
authors.affiliation: Department of Pediatrics, Drexel University College of Medicine, Neurology Section, St. Christopher's Hospital for Children, Philadelphia, PA 19134, USA. shirish.damle@drexelmed.edu.
authors.forename: Shirish
authors.initials: S
authors.lastname: Damle
authors.affiliation: Department of Pediatrics, Drexel University College of Medicine, Neurology Section, St. Christopher's Hospital for Children, Philadelphia, PA 19134, USA. agustin.legido@drexelmed.edu.
authors.forename: Agustin
authors.initials: A
authors.lastname: Legido
authors.affiliation: Department of Pediatrics, Drexel University College of Medicine, Neurology Section, St. Christopher's Hospital for Children, Philadelphia, PA 19134, USA. michael.goldenthal@drexelmed.edu.
authors.forename: Michael J
authors.initials: MJ
authors.lastname: Goldenthal
authors.affiliation: Arkansas Children's Research Institute, Little Rock, AR 72202, USA. refrye@uams.edu.|Department of Pediatrics, University of Arkansas for Medical Sciences, Little Rock, AR 72202, USA. refrye@uams.edu.
authors.forename: Richard E
authors.initials: RE
authors.lastname: Frye
keywords: B12; Complex I; Complex IV; antioxidants; autism spectrum disorder; electron transport chain; fatty acids; folate; mitochondrial disease; mitochondrial dysfunction