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Our later studies on the Shank3 -/- and Cntnap2 -/- knockout mice, the human SH-SY5Y cell line, and the human induced pluripotent stem cellsderived cortical neurons isolated from patients carrying a SHANK3 mutation confirmed the increased levels of NO and protein SNO [172,173,174,175,176]

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Debattisti V, Horn A, Singh R, Seifert EL, Hogarth MW, Mazala DA, Huang KT, Horvath R, Jaiswal JK, Hajnczky G (2019) Dysregulation of mitochondrial Ca 2+ Uptake and sarcolemma repair underlie muscle weakness and wasting in patients and mice lacking MICU1

In contrast to increased FAs uptake, mitochondrial FAO was downregulated in obesity, as evidenced by the remarkable decrease of AMPK phosphorylation, and PGC1 expression in the atria of HFD mice compared to STD mice (Figures 3HL)

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