TMEM126A and 3-methylglutaconic aciduria type 3: Autosomal recessive optic atrophy is rare and usually a syndromic presentation that includes Wolfram syndrome (WFS1 gene),16ACO2 gene, RTN4IP1 (OPA10), TMEM126A gene (OPA7), Behr syndrome (OPA1), and Costeff syndrome (OPA3).17