PMID: 24949105

Cebotaru L, Guggino WB
Complement yourself: Transcomplementation rescues partially folded mutant proteins.
Biophys Rev. 2014 Mar 1;6(1):169-180., [PubMed]
Sentences
No. Mutations Sentence Comment
77 ABCC7 p.Arg553Gln
X
ABCC7 p.Arg553Gln 24949105:77:79
status: NEW
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ABCC7 p.Arg553Met
X
ABCC7 p.Arg553Met 24949105:77:69
status: NEW
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 Using a mutagenesis approach, they were able to isolate two mutants, R553M and R553Q, which in combination with ƊF508 restored mating. Login to comment 80 ABCC7 p.Ile539Thr
X
ABCC7 p.Ile539Thr 24949105:80:112
status: NEW
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 For example, Hoelen (43), again using a limited proteolysis approach to assess domain stability, showed that an I539T second-site suppressor mutation, in combination with ƊF508 CFTR, restored NBD1 stability to the wt domain pattern, suggesting that correcting NBD1 stability will be critical for the development of a therapeutic for CF. Login to comment 89 ABCC7 p.Arg1070Trp
X
ABCC7 p.Arg1070Trp 24949105:89:77
status: NEW
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 Recent experiments have shown that certain amino acid substitutions, namely R1070W in combination with ƊF508 CFTR, can rescue ƊF508 CFTR trafficking, again pointing to the importance of domain-domain interactions in the mis-processing of ƊF508 (62). Login to comment