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Published online 2017 Feb 21. doi: 10.3389/fnmol.2017.00034
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Table 2

Studies showing association between ASD and mitochondrial dysfunction in animal models of ASD.

ReferencesEvidence of mitochondrial dysfunction
LINKAGE BETWEEN MITOCHONDRIA-RELATED GENES AND AUTISTIC PHENOTYPE
Hullinger et al., 2016Increased expression of AT-1/SLC33A1 caused an autistic-like phenotype in mice.
Inan et al., 2016Progressive decline in oxidative phosphorylation led to circuit dysfunction, impaired sensory gating and social disability when the cox10 gene was selectively deleted in parvalbumin neurons in mouse.
Sakurai et al., 2010Loss of SLC25A12 gene resulted in hypomyelination. Myelin deficits in slice cultures from knockout mice were reversed by administration of pyruvate.
Xie et al., 2016Cell-autonomous insufficiencies in the activity of TMLHE reduced neural stem cell pools in the embryonic mouse brain.
Zhao et al., 2010ASD-like features observed in neuronal glucose transporter isoform 3-deficient mice.
ALTERATIONS IN MITOCHONDRIAL FUNCTION IN ANIMAL OR CELLULAR MODELS OF ASD BASED ON GENETIC FACTORS
De Filippis et al., 2015The rate of hydrogen peroxide generation was increased and the function of complex ii impaired in the brain of MeCP2-308 heterozygous female mice.
Jin et al., 2015Mecp2, whose mutations cause Rett syndrome, was observed to regulate mitochondrial bioenergetics through a glutamine transporter in microglia.
Kriaucionis et al., 2006Mitochondrial abnormalities observed in Mecp2-null mouse, a model of Rett syndrome.
Nie et al., 2015Mitochondrial uncoupling protein-2 was highly induced in Tsc2-deficient neurons, and also in a neuron-specific Tsc1 conditional knock-out mouse model.
Norkett et al., 2016DISC1 protein regulated mitochondrial dynamics in neurites of neurons.
Pancrazi et al., 2015A fraction of the protein Foxg1, which is implicated in autism, was found to localize to mitochondria and coordinate cell differentiation and bioenergetics.
Santini et al., 2015A mouse model of Angelman syndrome displayed elevated levels of mitochondria-derived reactive oxygen species in pyramidal neurons in hippocampal CA1 area, and administration of MitoQ, a mitochondria-specific antioxidant, to this model normalized synaptic plasticity and restored memory.
Su et al., 2011Mitochondrial dysfunction observed in hippocampal neurons of the UBE3A-deficient mouse model of Angelman syndrome.
MITOCHONDRIAL DYSFUNCTION IN ANIMAL MODELS OF ASD BASED ON ENVIRONMENTAL FACTORS
Bhandari and Kuhad, 2015Propanoic acid exposure induced autism-like behavior in rats and activities of complex I and II were reduced.
Kumar and Sharma, 2016Prenatal exposure to valproic acid decreased the activity of mitochondrial complex I, II, and IV in rats.
Macfabe, 2012Mitochondrial dysfunction observed in a rat ASD model in which propionic acid, an enteric bacterial fermentation product, is infused intracerebroventricularly.
TREATMENT RELATED TO METABOLISM AND MITOCHONDRIAL FUNCTION IN ANIMAL MODELS OF ASD*
Ciarlone et al., 2016Ketone ester supplementation improved motor coordination, learning and memory, and synaptic plasticity in a mouse model of Angelman syndrome. The treatment also attenuated seizure activity and altered brain amino acid metabolism in this model.
Currais et al., 2016Dietary glycemic index was found to modulate behavioral and biochemical phenotype of the BTBR mouse model of ASD.
Naviaux et al., 2013, 2014, 2015Anti-purinergic therapy improved autism-like features in the maternal immune activation mouse model and the Fragile X mouse model.
Park et al., 2014Dietary therapy with triheptanoin enhanced mitochondrial substrate use and improved metabolism and behaviors of Mecp2-null mouse model of Rett syndrome.
Sakurai et al., 2010Loss of the SLC25A12 gene resulted in hypomyelination. Myelin deficits in slice cultures from knockout mice are reversed by administration of pyruvate.
Santini et al., 2015A mouse model of Angelman syndrome displayed elevated levels of mitochondria-derived reactive oxygen species in pyramidal neurons in CA1 hippocampus, and administration of MitoQ, a mitochondria-specific antioxidant, in this model normalized synaptic plasticity and restored memory.
*Studies using the ketogenic diet are described in more detail in the main text.