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Fig. 1 | Molecular Brain

Fig. 1

From: Riluzole partially restores RNA polymerase III complex assembly in cells expressing the leukodystrophy-causative variant POLR3B R103H

Fig. 1

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, POLR3B-L426S, POLR3B-T462R and POLR3B-R1046H. Asterisks (*) indicate the bait protein used in AP-MS. Light gray subunits indicate statistically significant reduction, black subunits indicate statistically significant increase and the white subunits were not detected in the AP-MS experiments. Dark gray subunits were detected but did not show statistically significant differences between the WT and the mutant

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Molecular Brain

ISSN: 1756-6606