TABLE 1.

Summary information of CFTR point mutants analyzed in present study

CFTR variants	Clinical dataa	Band C/band Bb (±S.E., n = 5)	Processingc	Normalized processingd	Normalized iodide efflux functione (±S.E., n = 6)	Iodide efflux to processed proteinf
		%	%	%	% peak intensity	%
WT-CFTR	—g	83 ± 3	77	100	100 ± 8	—
Murine	—	86 ± 5	66	86	74 ± 4	86 ± 4
E527Q	Mild CF	64 ± 5	49	63	46 ± 4	73 ± 4
E528Q	—	86 ± 5	79	102	135 ± 16	132 ± 10
S531T	—	87 ± 6	81	105	71 ± 5	67 ± 5
K536Q	—	69 ± 3	42	54	51 ± 4	94 ± 3
I539T	Revertant	112 ± 5	81	105	49 ± 6	46 ± 5
L581F	—	118 ± 3	83	107	72 ± 5	67 ± 3
L581F/K584E	—	125 ± 2	77	100	100 ± 12	100 ± 8
T1263I	Mild CF	75 ± 3	76	98	31 ± 8	31 ± 5
P1290T	Asymptomatic	87 ± 3	82	106	92 ± 10	86 ± 6
K1302Q	—	72 ± 3	77	100	37 ± 2	37 ± 2
Y1307N	—	82 ± 2	76	98	70 ± 5	71 ± 3
Q1309K	—	79 ± 4	77	100	26 ± 2	26 ± 3
S1311K	—	73 ± 4	72	93	33 ± 7	35 ± 5
R1325K	—	64 ± 6	78	101	47 ± 2	46 ± 4
V1338T	—	88 ± 2	77	100	37 ± 11	37 ± 6
C1344Y	—	71 ± 4	76	98	86 ± 4	87 ± 4
L1367I	—	72 ± 5	80	103	36 ± 5	34 ± 5
D1394G	—	78 ± 4	86	111	93 ± 12	83 ± 8
E1409D	—	70 ± 3	70	90	43 ± 5	47 ± 4

^a Data from the CFTR mutation database.

^b Percentage of processing given by (band C/band B) × 100.

^c Percentage of processing given by (band C/(band B + band C) × 100).

^d Ratio of the “percent band C” value for each mutant (from previous column) over the same “percentage for WT-CFTR.”

^e Iodide efflux expressed as percentage of peak intensity relative to that of WT-CFTR.

^f Ratio of “Normalized iodide efflux function” values (from previous column) normalized to “Normalized processing” values (from column 5).

^g —, not applicable/no information.