BACK HOME

The 4 Thioredoxin Repeats and their Mutations

S.NOUniprot IDProtein Name Sequence LengthThioredoxin RegionMutationInvolvement of Repeats in Mutations
1 Q15084 Protein disulfide-isomerase A6 44055 55 C->S: 50% decrease in enzyme activity
when associated with S-58
when associated with S-58
S-190 and S-193
58 58 C->A: Accelerates dephosphorylation of ERN1
when associated with A-193
58 58 C->S: 50% decrease in enzyme activity
when associated with S-55
when associated with S-193
when associated with S-55
S-190 and S-193
190 190 C->S: 25% decrease in enzyme activity
when associated with S-193
when associated with S-55
S-58 and S-193
193 193 C->A: Accelerates dephosphorylation of ERN1
when associated with A-58
193 193 C->S: 90% decrease in enzyme activity
when associated with S-58
when associated with S-190
when associated with S-55
S-58 and S-190
Y
2 P30101 Protein disulfide-isomerase A3 50557 57 C->A: No loss of activity
when associated with A-406
57 57 C->S: Activity changed to serine protease
60 60 C->S: Activity changed to serine protease
when associated with S-409
406 406 C->A: No loss of activity
when associated with A-57
406 406 C->S: Activity changed to serine protease
409 409 C->S: Activity changed to serine protease
when associated with S-60
Y
3 Q13087 Protein disulfide-isomerase A2 52518 18 C->A: Impairs interchain disulfide bridge formation
284 284 N->Q: Increases formation of a highly stable disulfide-bonded PDIA2 dimer
364 364 C->A: No effect on interchain disulfide bridge formation
N
4 Q8IXB1 DnaJ homolog subfamily C member 10 79363 63 H->Q: Prevents interaction with HSPA5, leading to prolonged interaction with substrate proteins
161 161 C->A: Abolishes disulfide reductase activity
when associated with A-483
A-591 and A-703
483 483 C->A: Abolishes disulfide reductase activity
when associated with A-161
A-591 and A-703
591 591 C->A: Abolishes disulfide reductase activity
when associated with A-161
A-483 and A-703
703 703 C->A: Abolishes disulfide reductase activity
when associated with A-161
A-483 and A-591
Y