by University of Montreal Schematic representation of Pol III assembly defects caused by various mutated subunits. Representation of the 17-subunit Pol III (Pol III; reviewed in detail in 4) assembled in the presence of (A) the leukodystrophy-causative mutations encoding POLR3B-R103H, POLR1C-N32I and POLR1C-N74S and (B) the neurological disorder causative de novo mutations encoding POLR3B-A365V, POLR3B-D375V,...