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PMID: 15923638
Farinha CM, Amaral MD
Most F508del-CFTR is targeted to degradation at an early folding checkpoint and independently of calnexin.
Mol Cell Biol. 2005 Jun;25(12):5242-52.,
[PubMed]
Sentences
No.
Mutations
Sentence
Comment
208
ABCC7 p.Asn894Ala
X
ABCC7 p.Asn894Ala 15923638:208:160
status:
NEW
view ABCC7 p.Asn894Ala details
ABCC7 p.Asn894Gln
X
ABCC7 p.Asn894Gln 15923638:208:131
status:
NEW
view ABCC7 p.Asn894Gln details
ABCC7 p.Asn900Gln
X
ABCC7 p.Asn900Gln 15923638:208:141
status:
NEW
view ABCC7 p.Asn900Gln details
ABCC7 p.Asn900Ala
X
ABCC7 p.Asn900Ala 15923638:208:170
status:
NEW
view ABCC7 p.Asn900Ala details
As the N-glyconjugate is attached to asparagine residues 894 and 900 of CFTR, we replaced both these residues by either glutamine (
N894Q
and
N900Q
) or alanine (
N894A
and
N900A
) residues and studied the CFTR turnover of the resulting proteins on wt and F508del backgrounds when stably expressed in BHK cells as above.
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219
ABCC7 p.Asn894Ala
X
ABCC7 p.Asn894Ala 15923638:219:55
status:
NEW
view ABCC7 p.Asn894Ala details
ABCC7 p.Asn894Ala
X
ABCC7 p.Asn894Ala 15923638:219:172
status:
NEW
view ABCC7 p.Asn894Ala details
ABCC7 p.Asn894Gln
X
ABCC7 p.Asn894Gln 15923638:219:93
status:
NEW
view ABCC7 p.Asn894Gln details
ABCC7 p.Asn894Gln
X
ABCC7 p.Asn894Gln 15923638:219:221
status:
NEW
view ABCC7 p.Asn894Gln details
ABCC7 p.Asn900Gln
X
ABCC7 p.Asn900Gln 15923638:219:99
status:
NEW
view ABCC7 p.Asn900Gln details
ABCC7 p.Asn900Gln
X
ABCC7 p.Asn900Gln 15923638:219:227
status:
NEW
view ABCC7 p.Asn900Gln details
ABCC7 p.Asn900Ala
X
ABCC7 p.Asn900Ala 15923638:219:61
status:
NEW
view ABCC7 p.Asn900Ala details
ABCC7 p.Asn900Ala
X
ABCC7 p.Asn900Ala 15923638:219:178
status:
NEW
view ABCC7 p.Asn900Ala details
BHK cells stably expressing wt CFTR (A, lanes 1 to 5),
N894A
-
N900A
(WAA) (A, lanes 6 to 10),
N894Q
-
N900Q
(WQQ) (A, lanes 11 to 15), F508del-CFTR (B, lanes 1 to 5), F508del-
N894A
-
N900A
(FAA) (B, lanes 6 to 10), or F508del-
N894Q
-
N900Q
CFTR (FQQ) (B, lanes 11 to 15) were pulse-labeled and chased as before (Fig. 2) for 0 h (lanes 1, 6, and 11), 0.5 h (lanes 2, 7, and 12), 1 h (lanes 3, 8, and 13), 2 h (lanes 4, 9, and 14), and 3 h (lanes 5, 10, and 15).
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284
ABCC7 p.Asn894Ala
X
ABCC7 p.Asn894Ala 15923638:284:83
status:
NEW
view ABCC7 p.Asn894Ala details
ABCC7 p.Asn894Gln
X
ABCC7 p.Asn894Gln 15923638:284:119
status:
NEW
view ABCC7 p.Asn894Gln details
ABCC7 p.Asn900Gln
X
ABCC7 p.Asn900Gln 15923638:284:129
status:
NEW
view ABCC7 p.Asn900Gln details
ABCC7 p.Asn900Ala
X
ABCC7 p.Asn900Ala 15923638:284:93
status:
NEW
view ABCC7 p.Asn900Ala details
Asparagine residues at positions 894 and 900 were replaced by either two alanines (
N894A
and
N900A
) or two glutamines (
N894Q
and
N900Q
).
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