Tetratricopeptide Repeat Protein 19, Mitochondrial (TTC19) Reagents

Tetratricopeptide repeat protein 19, mitochondrial

Required for the preservation of the structural and functional integrity of mitochondrial respiratory complex III by allowing the physiological turnover of the Rieske protein UQCRFS1. Involved in the clearance of UQCRFS1 N-terminal fragments, which are produced upon incorporation of UQCRFS1 into the complex III and whose presence is detrimental for its catalytic activity.

Mitochondrial complex III deficiency, nuclear 2 (MC3DN2)

Mitochondrion inner membrane.

TTC19 family

2010204O13Rik; FLJ20343; MGC138312; MGC19520; mitochondrial; RIKEN cDNA 2810460C24; Tetratricopeptide repeat protein 19; TPR repeat protein 19; Ttc 19; TTC19; TTC19_HUMAN