Table 2. Definite pathogenic mutations in PKD1 and PKD2 found in this study.
Family No. | Exon/intron | cDNA change | Protein change | Mutation Type | Proband No. | Family history | Previous description |
---|---|---|---|---|---|---|---|
PKD1 | |||||||
28 | 1 | c.90C > A | p.Cys30Ter | Nonsense | G0586 | Yes | Novel |
17 | 3 | c.338-341delATTT | p.Asn113AsnfsX3 | Frameshift | G0211 | Yes | Novel |
13 | 4 | c.412C > T | p.Arg138Ter | Nonsense | G1739 | Yes | PKDB |
20 | 5 | c.937G > T | p.Glu313Ter | Nonsense | G0214 | Yes | PKDB |
51 | 11 | c.2711-2712delAG | p.Glu904GlyfsX195 | Frameshift | G1604 | Yes | Novel |
42 | 13 | c.3068delA | p.Gln1023ArgfsX14 | Frameshift | G0463 | Yes | Novel |
1 | 15 | c.4306C > T | p.Arg1436Ter | Nonsense | G0256 | Yes | PKDB |
9 | 15 | c.4369-4370delTC | p.Ser1457SerfsX64 | Frameshift | G0315 | Yes | PKDB |
41 | 15 | c.5014-5015delAG | p.Arg1672GlyfsX97 | Frameshift | G0262 | Yes | PKDB |
32 | 15 | c.5863delC | p.His1955ThrfsX1 | Frameshift | G0679 | Yes | Novel |
48 | 15 | c.5912-5913delTG | p.Val1971GlufsX18 | Frameshift | G1052 | Yes | PKDB |
37 | 15 | c.6465-6466delGC | p.Leu2155LeufsX18 | Frameshift | G1103 | Yes | Novel |
33 | 15 | c.6635delG | p.Ser2212ThrfsX29 | Frameshift | G0680 | Yes | Novel |
5 | 19 | c.7566C > A | p.Cys2522Ter | Nonsense | G0246 | Noa | Novel |
49 | 21 | c.7867G > T | p.Glu2623Ter | Nonsense | G1436 | Noa | Novel |
27 | IVS21 | c.8017-2-1delAG | p.Gly2673fs | Splice | G0576 | Yes | PKDB |
11 | 23 | c.8606-8607delAG | p.Gln2869AlafsX66 | Frameshift | G0111 | Yes | PKDB |
19 | 24 | c.8495-8496insA | p.His2932GlnfsX4 | Frameshift | G0213 | Yes | Novel |
39 | 40 | c.11313delG | p.Ser3771SerfsX54 | Frameshift | G0017 | Noa | Novel |
14 | 40 | c.11343C > A | p.Tyr3781Ter | Nonsense | G0054 | Yes | 16 |
21 | 41 | c.11433-11439dupCGTGTAT | p.Ala3812ValfsX4 | Frameshift | G0224 | Noa | Novel |
15 | 43 | c.11719-11720dupCT | p.Leu3907LeufsX37 | Frameshift | G0092 | Yes | Novel |
46 | 43 | c.11944C > T | p.Gln3982Ter | Nonsense | G0863 | Yes | PKDB |
45 | 43 | c.11977-11978insT | p.Ser3993PhefsX163 | Frameshift | G0858 | Yes | Novel |
34 | 45 | c.12169delG | p.Val4057TrpfsX139 | Frameshift | G0865 | Yes | Novel |
3 | 45 | c.12391delG | p.Glu4131SerfsX67 | Frameshift | G0251 | Yes | Novel |
50 | 46 | c.12682C > T | p.Arg4228Ter | Nonsense | G1543 | Yes | PKDB |
36 | 46 | c.12712C > T | p.Gln4238Ter | Nonsense | G1021 | Yes | PKDB |
30 | 1 | EX1del | Large deletion | G0677 | Yes | 25 | |
52 | 21 | EX21del | Large deletion | G0121 | Yes | Novel | |
40 | 1–30 | EX1-30del | Large deletion | G0018 | Noa | Novel | |
PKD2 | |||||||
44 | IVS1 | c.595_595 + 14delGGTAAGAGCGCGCGA | Splice | G0904 | Yes | Novel |
PKDB, Polycystic Kidney Mutation Database.
ade novo mutation.