Transmembrane Protein 126A (TMEM126A)

TMEM126 protein contains a conserved domain defined by homology between TMEM126A lys5 to gly191 and TMEM126B asn45 to glu229. TMEM126A colocalized with mitochondrial complex II subunit 70 kD Fp (SDHA), complex IV subunit 1 (MTCO), ATP synthase subunit beta (ATP5B), and ATP synthase subunit alpha (ATP5A), supporting the mitochondrial localization of the protein. The fusion protein did not significantly colocalize with the markers of the Golgi apparatus, lysosomes, early endosomes, cytoskeleton, microtubule, or centrosome. RT-PCR on total RNA from various adult and fetal human tissues showed that TMEM126A is strongly expressed in the brain (whole), cerebellum, fetal brain, skeletal muscle, testis, fetal retinal pigmentary epithelium, and fetal retina.