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The 2 RNase III Repeats and their Mutations

S.NOUniprot IDProtein Name Sequence LengthRNase III RegionMutationInvolvement of Repeats in Mutations
1 Q9UPY3 Endoribonuclease Dicer 1922960 960 F->A: 2-fold decrease in activity
971 971 Y->A: 10-fold decrease in activity
when associated with Y-972
972 972 Y->A: 10-fold decrease in activity
when associated with Y-971
1036 1036 E->A: 5-fold decrease in activity
1313 1313 E->A: No effect on activity
1320 1320 D->A: Decreased activity
when associated with D-1709
1340 1340 E->A: No effect on activity
1444 1444 E->A: Decreased activity
when associated with E-1813
1702 1702 Q->A: No effect on activity
1709 1709 D->A: Decreased activity
when associated with D-1320
1729 1729 P->E: No effect on activity
1813 1813 E->A: Decreased activity
when associated with E-1444
Y
2 Q9NRR4 Ribonuclease 3 1374536 536 C->A: Impairs protein folding and stability
when associated with A-538
538 538 C->A: Impairs protein folding and stability
when associated with A-536
561 561 C->A: Impairs protein folding and stability
622 623 622 623 RF->AA: Abolishes RNase activity
676 676 C->A: Impairs protein folding and stability
835 836 835 836 RR->AA: Abolishes RNase activity
914 914 R->M: Impairs RNase activity
923 923 R->A: Abolishes RNase activity
when associated with A-927
927 927 Y->A: Abolishes RNase activity
when associated with A-923
938 940 938 940 RKK->QQQ: Abolishes RNase activity
993 993 E->A,Q: No effect on pri-miRNA processing activity
1045 1045 E->Q: Impairs pri-miRNA processing activity
when associated with Q-1222
1077 1077 V->E: Loss of one DGCR8 interaction site
no effect on the second DGCR8 interaction site
1171 1171 E->A,Q: No effect on pri-miRNA processing activity
1194 1194 L->R: Abolishes interaction with DGCR8
1222 1222 E->Q: Impairs pri-miRNA processing activity
when associated with Q-1045
1243 1243 V->D: Abolishes interaction with DGCR8
Y