A homozygous variant in mitochondrial RNase P subunit PRORP is associated with Perrault syndrome characterized by hearing loss and primary ovarian insufficiency
Irit Hochberg, Leigh A. M. Demain, Jill E. Urquhart, Albert Amberger, Andrea J. Deutschmann, Sandra Demetz, Kyle Thompson, James O’Sullivan, Inna A. Belyantseva, Melanie Barzik, Simon G. Williams, Sanjeev S. Bhaskar, Emma M. Jenkinson, Nada Al-Sheqaih, Zeev Blumenfeld, , William G. Newman
The paper identifies a homozygous variant in KIAA0391 (PRORP) as a cause of Perrault syndrome, showing reduced mitochondrial RNase P activity and impaired tRNA processing.
bioRxiv · Jul 25, 2017 · PreprintGenetics