exon 1 (non coding) |
intron 1 |
c.1-2481G > T |
|
formerly: IVS1+1G > T |
Liu et al., 2008 (Liu et al., 2008) |
1 |
1 |
|
|
|
|
|
|
|
3 |
20 |
exon 2 c.1-177 |
c.-3_7del |
p.Met1? |
|
Sullivan et al., 2013 (Sullivan et al., 2013); Kiser et al., 2019 (Kiser et al., 2019) |
2 |
|
2 |
|
|
|
|
|
|
10/17/29 |
10/58/62 |
c.1A > T |
p.Met1? |
|
Carss et al., 2017 (Carss et al., 2017) |
1 |
|
|
|
|
|
|
|
|
|
|
c.18G > C |
p.Glu6Asp |
|
van Huet et al., 2015 (van Huet et al., 2015) |
1 |
|
|
|
1 |
|
|
|
|
|
|
c.19_20insA |
p.Leu7Hisfs*4 |
|
Sullivan et al., 2013 (Sullivan et al., 2013) |
1 |
|
1 |
|
|
|
|
|
|
|
|
c.34G > T |
p.Glu12* |
|
Van Cauwenbergh et al., 2017 (Van Cauwenbergh et al., 2017) |
1 |
|
1 |
|
|
|
|
|
|
|
|
c.55del |
p.Glu19Lysfs*46 |
|
Martin-Merida et al., 2018 (Martin-Merida et al., 2018) |
1 |
|
1 |
|
|
|
|
|
|
|
|
c.59_65del7 |
p.Gly20Alafs*43 |
|
Saini et al., 2012 (Saini et al., 2012) |
1 |
|
1 |
|
|
|
|
|
|
|
17 |
c.79G > T |
p.Glu27X |
|
Waseem et al., 2007 (Waseem et al., 2007) |
1 |
|
|
1 |
|
|
|
|
|
15 |
43 |
c.121C > G |
p.Leu41Val |
reported as cause of disease, but no functional studies |
Ellingford et al., 2016 (Ellingford et al., 2016a) |
1 |
|
|
|
1 |
|
|
|
|
|
|
c.165G > A |
|
|
de la Cerda et al., 2019 (de la Cerda et al., 2019) |
1 |
|
|
|
|
|
|
|
|
|
|
c.172A > T |
p.Lys58X |
|
Zhang et al., 2016 (Zhang et al., 2016) |
1 |
|
|
|
|
|
|
|
|
|
13 |
c.196_197delAA |
p.Lys66Aspfs*2 |
|
Xu et al., 2012 (Xu et al., 2012) |
1 |
|
1 |
1 |
|
|
|
|
|
|
24 |
intron 2 |
c.177+1G > A |
|
formerly IVS2+1 G > A |
Sullivan et al., 2006a (Sullivan et al., 2006a) |
1 |
1 |
|
|
|
|
|
|
|
|
|
c.177+1delG |
|
|
Rivolta et al., 2006 (Rivolta et al., 2006) |
1 |
1 |
|
|
|
|
|
|
|
|
|
exon 3 c.178–238 (20aa) |
c.217A > T |
p.Lys73X |
|
Eisenberger et al., 2013 (Eisenberger et al., 2013) |
1 |
|
|
1 |
|
|
|
|
|
|
|
c.220C > T |
p.Gln74X |
|
Sullivan et al., 2006a (Sullivan et al., 2006a); Van Cauwenbergh et al., 2017 (Van Cauwenbergh et al., 2017); Kiser et al., 2019 (Kiser et al., 2019) |
3 |
|
|
3 |
|
|
|
|
|
7 |
9 |
intron 3 |
exon 4239-322 |
c.267delA |
p.Glu89Aspfs*11 |
|
Sullivan et al., 2013 (Sullivan et al., 2013) |
1 |
|
1 |
|
|
|
|
|
|
|
|
|
p.Leu107del4 ctGAGT |
|
Aleman et al., 2009 (Aleman et al., 2009) |
1 |
|
1 |
|
|
|
|
|
|
|
32 |
c.319C > G |
|
|
Rivolta et al., 2006 (Rivolta et al., 2006); Rio Frio et al., 2008 (Rio Frio et al., 2008b) |
2 |
2 |
|
|
|
|
|
|
|
|
|
intron 4 |
c.322 + 4_322+7del |
p.? |
2 families in MM paper, 3 families in Zhang paper |
Zhang et al., 2016 (Zhang et al., 2016); Martin-Merida et al., 2018 (Martin-Merida et al., 2018) |
5 |
5 |
|
|
|
|
|
|
|
|
19/27 |
c.322+1G > A |
|
|
Wu et al., 2018 (Wu et al., 2018); Kiser et al., 2019 (Kiser et al., 2019) |
2 |
2 |
|
|
|
|
|
|
|
|
|
c.323-2A > G |
|
|
Rivolta et al. (2006) (Rivolta et al., 2006) |
1 |
1 |
|
|
|
|
|
|
|
|
|
exon 5 c.323-421 |
c.328_330del |
p.Ile110del |
Reported as p.Ile109del in de Sousa Dias paper |
de Sousa Dias et al., 2013 (de Sousa Dias et al., 2013); Martin-Merida et al., 2018 (Martin-Merida et al., 2018) |
2 |
|
|
|
|
2 |
|
|
|
|
|
c.331_342del |
p.His111_Ile114del |
111 and 114 inc |
Wang et al. (2003) (Wang et al., 2003) |
1 |
|
|
|
|
1 |
|
|
|
|
|
c.341T > A |
Ile114Asn |
|
Wheway et al. (2019) (Wheway et al., 2019) |
1 |
|
|
|
1 |
|
|
|
|
|
|
c.357_358delAA |
p.Ser119Serfs*5 |
in 2 families and 1 sporadic case in Zheng paper |
Xiao et al., 2017b (Xiao et al., 2017); Zheng et al., 2018 (Zheng et al., 2018) |
2 |
|
2 |
|
|
|
|
|
|
6/10 |
|
c.358–359 del AA |
p.Lys120Glufs*122 |
|
Gandra et al., 2008 (Gandra et al., 2008); Yang et al., 2015 (Yang et al., 2015) |
2 |
|
2 |
|
|
|
|
|
|
|
|
c.359dupA |
|
|
Hariri et al., 2018 (Hariri et al., 2018) |
1 |
|
|
|
|
|
|
|
|
|
41 |
c.359delA |
p.Lys120Argfs*78 |
|
Carss et al., 2017 (Carss et al., 2017) |
1 |
|
1 |
|
|
|
|
|
|
|
|
c.360dupA |
p.K120fs*5 |
|
Glockle et al., 2014 (Glockle et al., 2014) |
1 |
|
1 |
|
|
|
|
|
|
|
|
c.390delC |
p.Asn131fs*67 |
|
Sullivan et al., 2006a (Sullivan et al., 2006a); Kiser et al., 2019 (Kiser et al., 2019) |
2 |
|
2 |
|
|
|
|
|
|
10/10/17 |
16/21/48 |
c.400delG |
p.Asp134Ilefs |
|
Ellingford et al., 2016 (Ellingford et al., 2016a) |
1 |
|
1 |
|
|
|
|
|
|
|
|
c.413C > A |
Thr138Lys |
|
Waseem et al., 2007 (Waseem et al., 2007) |
1 |
|
|
|
1 |
|
|
|
|
15,20 |
30 |
intron 5 |
c.421-2A > G |
|
|
Jespersgaard et al., 2019 (Jespersgaard et al., 2019) |
1 |
1 |
|
|
|
|
|
|
|
|
|
c.421-1G > A |
|
formerly IVS5-1G > A |
Xia et al., 2004 (Xia et al., 2004); Xi et al., 2005 (Xi et al., 2005) |
2 |
2 |
|
|
|
|
|
|
|
|
|
exon 6 c.421-527 |
c.421G > T |
Glu141X |
|
Sullivan et al., 2006a (Sullivan et al., 2006a) |
1 |
|
|
1 |
|
|
|
|
|
|
|
c.433_434del |
p.S145Pfs*8 |
|
Kurata et al., 2018 (Kurata et al., 2018) |
1 |
|
1 |
|
|
|
|
|
|
7 |
7 |
c.522_527 + 10del |
|
Same family in these 2 papers |
Ghazawy et al., 2007 (Ghazawy et al., 2007); Buskin et al., 2018 (Buskin et al., 2018) |
1 |
|
|
|
|
|
|
|
|
30s |
33 |
c.525_526insAG |
|
|
Kiser et al., 2019 (Kiser et al., 2019) |
1 |
|
1 |
|
|
|
|
|
|
16 |
47 |
intron 6 |
c.527+1G > A |
|
Described as p.IVS6+1G > T |
Chakarova et al., 2006 (Chakarova et al., 2006); Martin-Merida et al., 2018 (Martin-Merida et al., 2018); Abdulridha-Aboud et al., 2016 (Abdulridha-Aboud et al., 2016) |
3 |
3 |
|
|
|
|
|
|
|
13/48/21 |
13/48 |
c.527+1G > T |
|
|
Gandra et al., 2008 (Gandra et al., 2008); Kiser et al., 2019 (Kiser et al., 2019) |
2 |
2 |
|
|
|
|
|
|
|
|
|
c.527+2T > G |
|
|
Wu et al., 2018 (Wu et al., 2018) |
1 |
1 |
|
|
|
|
|
|
|
|
|
c.527+2T > C |
|
|
Audo et al., 2010 (Audo et al., 2010) |
1 |
1 |
|
|
|
|
|
|
|
|
|
c.527+3A > G |
|
In 2 families in Waseem paper. Reported as IVS 6 + 3 A > G in Ivings paper |
Vithana et al., 2001 (Vithana et al., 2001); Waseem et al., 2007 (Waseem et al., 2007); Ivings et al., 2008 (Ivings et al., 2008); Ellingford et al., 2016 (Ellingford et al., 2016a); Xie et al., 2018 (Xie et al., 2018); Kiser et al., 2019 (Kiser et al., 2019) |
7 |
7 |
|
|
|
|
|
|
|
20/29/28/27 |
52/30/70 |
c.528–3_45del |
|
|
Vithana et al., 2001 (Vithana et al., 2001); Sato et al., 2005 (Sato et al., 2005) |
2 |
2 |
|
|
|
|
|
|
|
|
|
c.528–39_531del |
|
|
Sullivan et al., 2013 (Sullivan et al., 2013) |
1 |
1 |
|
|
|
|
|
|
|
|
|
c.528-1G > A |
|
|
Waseem et al., 2007 (Waseem et al., 2007); Van Cauwenbergh et al., 2017 (Van Cauwenbergh et al., 2017) |
2 |
2 |
|
|
|
|
|
|
|
|
20 |
exon 7 c.528-697 |
c.541G > T |
p.Glu181X |
2 families in MM paper |
Pomares et al., 2010 (Pomares et al., 2010); Van Cauwenbergh et al., 2017 (Van Cauwenbergh et al., 2017); Martin-Merida et al., 2018 (Martin-Merida et al., 2018) |
4 |
|
|
4 |
|
|
|
|
|
|
19, 23 |
c.544_618del75bp |
E182_E206del |
|
Xu et al., 2012 (Xu et al., 2012) |
1 |
|
|
|
|
1 |
|
|
|
|
24 |
c.547delG |
p.E183fs |
|
Xiao et al., 2017 (Xiao et al., 2017) |
1 |
|
1 |
|
|
|
|
|
|
5,6,7,8,10 |
|
c.548_580dup |
p.Glu183_Met193dup |
|
Tiwari et al., 2016 (Tiwari et al., 2016) |
1 |
|
|
|
|
|
1 |
|
|
24 |
|
c.550_552del |
p.Leu184del |
|
Kiser et al., 2019 (Kiser et al., 2019) |
1 |
|
|
|
|
1 |
|
|
|
71 |
71 |
c.553G > T |
p.Glu185X |
de novo in Neveling paper |
Neveling et al., 2012 (Neveling et al., 2012); van Huet et al., 2015 (van Huet et al., 2015) |
2 |
|
|
1 |
|
|
|
|
|
|
|
c.562G > T |
p.Glu188X |
|
ClinVar (likely pathogenic) |
1 |
|
|
1 |
|
|
|
|
|
|
|
c.580_581delGC |
p.Leu195GlyFs |
|
ClinVar (likely pathogenic) |
1 |
|
1 |
|
|
|
|
|
|
|
|
c.580-581dup33bp |
|
|
Vithana et al., 2001 (Vithana et al., 2001) |
1 |
|
|
|
|
|
1 |
|
|
|
|
c.581C > A |
Ala194Glu |
|
Vithana et al., 2001 (Vithana et al., 2001) |
1 |
|
|
|
1 |
|
|
|
|
|
|
c.590T > C |
Leu197Pro |
|
Bryant et al., 2019 (Bryant et al., 2019); Wu et al., 2018 (Wu et al., 2018) |
2 |
|
|
|
2 |
|
|
|
|
|
|
c.615C > A |
p.Tyr205X |
|
ClinVar (pathogenic) |
1 |
|
|
1 |
|
|
|
|
|
|
|
c.615C > G |
p.Tyr205X |
|
ClinVar (likely pathogenic) |
1 |
|
|
1 |
|
|
|
|
|
|
|
c.615delC |
p.Y205X |
|
Xu et al., 2012 (Xu et al., 2012) |
1 |
|
|
1 |
|
|
|
|
|
|
27 |
c.616G > T |
p.Glu206X |
|
Wang et al., 2014 (Wang et al., 2014) |
1 |
|
|
1 |
|
|
|
|
|
|
|
c.629delC |
|
|
Huang et al., 2015 (Huang et al., 2015) |
1 |
|
1 |
|
|
|
|
|
|
|
|
c.636delG |
p.Met212fs*238 |
|
Sullivan et al., 2006a (Sullivan et al., 2006a); Bowne et al., 2011 (Bowne et al., 2011); Wang et al., 2014 (Wang et al., 2014) |
3 |
|
3 |
|
|
|
|
|
|
|
|
c.646G > C |
Ala216Pro |
|
Vithana et al., 2001 (Vithana et al., 2001) |
1 |
|
|
|
1 |
|
|
|
|
|
|
c.666_668del |
p.Ile223del |
|
Jespersgaard et al., 2019 (Jespersgaard et al., 2019) |
1 |
|
|
|
|
1 |
|
|
|
|
|
c.673del |
p.Ala225Hisfs*14 |
|
Jespersgaard et al., 2019 (Jespersgaard et al., 2019) |
1 |
|
1 |
|
|
|
|
|
|
|
|
intron 7 |
c.698-1G > A |
|
|
Roberts et al., 2016 (Roberts et al., 2016); Birtel et al., 2018 (Birtel et al., 2018a) |
2 |
2 |
|
|
|
|
|
|
|
|
|
exon 8 c.698 - 855 |
c.709-734dup |
|
|
Terray et al., 2017 (Terray et al., 2017) |
1 |
|
|
|
|
|
1 |
|
|
|
|
732-737delins20bp |
M244fsX248 |
|
Martinez-Gimeno et al., 2003 (Martinez-Gimeno et al., 2003) |
1 |
|
|
|
|
|
|
1 |
|
6–20 |
|
c.736G > A |
p.Ala246Thr |
|
Xu et al., 2014 (Xu et al., 2012); Martin-Merida et al., 2018 (Martin-Merida et al., 2018) |
2 |
|
|
|
2 |
|
|
|
|
|
|
c.741_742insA |
p.Asn248Lysfs |
|
ClinVar (likely pathogenic) |
1 |
|
1 |
|
|
|
|
|
|
|
|
c.758_767del |
p.Gly253fs*317 |
|
Sullivan et al., 2006a (Sullivan et al., 2006a); Kiser et al., 2019 (Kiser et al., 2019) |
2 |
|
2 |
|
|
|
|
|
|
19 |
31 |
c.763C > T |
p.Gln255X |
|
Wang et al., 2014 (Wang et al., 2014) |
1 |
|
|
1 |
|
|
|
|
|
|
|
769-770insA |
K257fs*277 |
|
Vithana et al., 2001 (Vithana et al., 2001); Martinez-Gimeno et al., 2003 (Martinez-Gimeno et al., 2003) |
2 |
|
2 |
|
|
|
|
|
|
10–18 |
|
c.770dup |
p.Thr258Aspfs*21 |
|
Vithana et al., 2001 (Vithana et al., 2001); Martin-Medira et al. (2018)
|
2 |
|
2 |
|
|
|
|
|
|
|
|
c.772_773del2insCAACATGCAACATCAT |
p.(Thr258Glnfs) |
|
Zhao et al., 2015 (Zhao et al., 2015) |
1 |
|
1 |
|
|
|
|
|
|
|
|
c.781G > C |
Gly261Arg |
|
Xiao et al., 2017 (Xiao et al., 2017) |
1 |
|
|
|
1 |
|
|
|
|
|
|
c.785delT |
p.Phe262Serfs*59 |
|
Lim et al., 2009 (Lim et al., 2009) |
1 |
|
1 |
|
|
|
|
|
|
<10 |
|
c.804delG |
p.L268fs |
|
Xiao et al., 2017 (Xiao et al., 2017) |
1 |
|
1 |
|
|
|
|
|
|
|
|
c.808_809insC |
p.His270Profs*8 |
|
Sullivan et al., 2013 (Sullivan et al., 2013) |
1 |
|
1 |
|
|
|
|
|
|
|
|
c.815G > T |
p.Gly272Val |
predicted by Sullivan to be benign |
Sullivan et al., 2006a (Sullivan et al., 2006a); Daiger et al., 2014 (Daiger et al., 2014) |
2 |
|
|
|
2 |
|
|
|
|
|
|
c.816_830delCTACATCTACCACAG |
p.Tyr273_Ser277del |
|
Birtel et al., 2018 (Birtel et al., 2018b) |
1 |
|
|
|
|
1 |
|
|
|
|
|
c.824_825insA |
p.Y275X |
|
Yang et al., 2013 (Yang et al., 2013) |
1 |
|
|
1 |
|
|
|
|
|
12 |
|
c.828_829del |
p.His276Glnfs*2 |
|
Martinez-Gimeno et al., 2003 (Martinez-Gimeno et al., 2003); Martin-Merida et al., 2018 (Martin-Merida et al., 2018) |
2 |
|
2 |
|
|
|
|
|
|
5–20 |
|
c.838_841dupGTGC |
p.Gln281Argfs*44 |
|
Carss et al., 2017 (Carss et al., 2017) |
1 |
|
1 |
|
|
|
|
|
|
|
|
c.839T > G |
p.Val280Gl |
|
Birtel et al., 2018 (Birtel et al., 2018b) |
1 |
|
|
|
1 |
|
|
|
|
|
|
intron 8 |
c.855+1G > C |
|
|
Lu et al., 2005 (Lu et al., 2005) |
1 |
1 |
|
|
|
|
|
|
|
|
|
c.855+1G > T |
|
|
Jespersgaard et al., 2019 (Jespersgaard et al., 2019) |
1 |
1 |
|
|
|
|
|
|
|
|
|
c.855+1G > A |
|
|
ClinVar (pathogenic) |
1 |
1 |
|
|
|
|
|
|
|
|
|
c.856-2A > G |
|
|
Rivolta et al., 2006 (Rivolta et al., 2006) |
1 |
1 |
|
|
|
|
|
|
|
|
|
exon 9 c.856 - 945 |
c.862C > T |
p.Arg288Trp |
|
Coussa et al., 2015 (Coussa et al., 2015) |
1 |
|
|
|
1 |
|
|
|
|
66 |
68 |
c.866_879delGGAAAGCGGCCCGG |
p.R289Pfs*30 |
|
Villanueva et al., 2014 (Villanueva et al., 2014); Zhang et al., 2016 (Zhang et al., 2016) |
2 |
|
2 |
|
|
|
|
|
|
2–16 |
7–63 |
c.871G > C |
Ala291Pro |
|
Sullivan et al., 2006a (Sullivan et al., 2006a) |
1 |
|
|
|
1 |
|
|
|
|
|
|
c.877_910del |
p.Arg293_Arg304>Valfs*17 |
|
Rivolta et al., 2006 (Rivolta et al., 2006) |
1 |
|
1 |
|
|
|
|
|
|
|
|
c.895T > C |
Cys299Arg |
|
Sullivan et al., 2006a (Sullivan et al., 2006a); Xu et al., 2012 (Xu et al., 2012); Martin-Merida et al., 2018 (Martin-Merida et al., 2018); Kiser et al., 2019 (Kiser et al., 2019) |
4 |
|
|
|
4 |
|
|
|
|
21/27/41/63 |
27/44/63/65 |
c.896G > A |
p.Cys299Tyr |
|
Bhatia et al., 2018 (Bhatia et al., 2018) |
1 |
|
|
|
1 |
|
|
|
|
|
|
c.910C > T |
p.Arg304Cys |
|
Huang et al., 2015 (Huang et al., 2015); Hariri et al., 2018 (Hariri et al., 2018) |
2 |
|
|
|
2 |
|
|
|
|
|
45 |
c.914_915insTGT |
p.Val305_Asp306insVal |
|
Utz et al., 2013 (Utz et al., 2013) |
1 |
|
|
|
|
|
1 |
|
|
30s |
40s |
c.915_916insTGT |
p.Val305_Asp306insCys |
|
Sullivan et al., 2013 (Sullivan et al., 2013) |
1 |
|
|
|
|
|
1 |
|
|
|
|
c.916G > A |
p.Asp306Asn |
reported as cause of disease, but functional studies not carried out |
Ellingford et al., 2016 (Ellingford et al., 2016a) |
1 |
|
|
|
1 |
|
|
|
|
|
|
|
p.307fs*15 |
|
Lu et al., 2013 (Lu et al., 2013) |
1 |
|
1 |
|
|
|
|
|
|
|
|
c.939dup |
p.Gly314Argfs*10 |
|
Fernandez-San Jose et al., 2015 (Fernandez-San Jose et al., 2015); Martin-Merida et al., 2018 (Martin-Merida et al., 2018) |
2 |
|
2 |
|
|
|
|
|
|
|
|
c.940delG |
p.Ala302Glnfs |
|
ClinVar (pathogenic/likely pathogenic) |
2 |
|
2 |
|
|
|
|
|
|
|
|
intron 9 |
c.946–1 G > C |
|
|
Bowne et al., 2011 (Bowne et al., 2011); Daiger et al. (2014) (Daiger et al., 2014) |
2 |
2 |
|
|
|
|
|
|
|
|
|
exon 10 c.946 - 1073 |
c.950delG |
p.Gly316Alafs*4 |
|
ClinVar (pathogenic) |
1 |
|
1 |
|
|
|
|
|
|
|
|
c.961A > T |
p.Lys321X |
|
Jespersgaard et al., 2019 (Jespersgaard et al., 2019) |
1 |
|
|
1 |
|
|
|
|
|
|
|
c.967G > T |
p.Glu323X |
|
Ellingford et al., 2016 (Ellingford et al., 2016a) |
1 |
|
|
1 |
|
|
|
|
|
|
|
c.973G > T |
Glu325X |
|
Sullivan et al., 2006a (Sullivan et al., 2006a) |
1 |
|
|
1 |
|
|
|
|
|
|
|
c.978_982del |
p.Lys327Argfs*146 |
|
Van Cauwenbergh et al., 2017 (Van Cauwenbergh et al., 2017) |
1 |
|
1 |
|
|
|
|
|
|
|
|
c.992G > A |
p.Trp331X |
|
ClinVar (pathogenic) |
1 |
|
|
1 |
|
|
|
|
|
|
|
c.994C > T |
p.Gln332X |
|
Ellingford et al., 2016 (Ellingford et al., 2016a) |
1 |
|
|
1 |
|
|
|
|
|
|
|
c.997del |
p.Glu333Serfs*5 |
|
Jespersgaard et al., 2019 (Jespersgaard et al., 2019) |
1 |
|
1 |
|
|
|
|
|
|
|
|
c.1015C > T |
p.Q339X |
|
Xie et al., 2018 (Xie et al., 2018) |
1 |
|
|
1 |
|
|
|
|
|
|
|
c.1035_1036insGC |
p.Pro346Argfs*18 |
|
Wu et al., 2018 (Wu et al., 2018) |
1 |
|
1 |
|
|
|
|
|
|
|
|
c.1048C > T |
p.Gln350X |
|
Eisenberger et al., 2013 (Eisenberger et al., 2013) |
1 |
|
|
1 |
|
|
|
|
|
|
|
c.1060C > T |
p.Arg354X |
|
Sullivan et al., 2013 (Sullivan et al., 2013); Ellingford et al., 2016 (Ellingford et al., 2016a); Xiao et al., 2017 (Xiao et al., 2017); Wu et al., 2018 (Wu et al., 2018); Kurata et al., 2018 (Kurata et al., 2018); Kiser et al., 2019 (Kiser et al., 2019), |
6 |
|
|
6 |
|
|
|
|
|
5/6/7/8/8/6/12 |
6/24 |
intron 10 |
c.1067_1073+8del |
|
|
Eisenberger et al., 2013 (Eisenberger et al., 2013) |
1 |
1 |
|
|
|
|
|
|
|
|
|
c.1073+1G > A |
|
|
Sullivan et al., 2006a (Sullivan et al., 2006a); Kiser et al., 2019 (Kiser et al., 2019) |
2 |
2 |
|
|
|
|
|
|
|
28/40 |
9/12/14/40 |
c.1074-2 A > T |
p.Tyr359Sfs*29 |
|
Yang et al., 2013 (Yang et al., 2013) |
1 |
1 |
|
|
|
|
|
|
|
2–8 |
|
c.1074–1G > T |
p.? |
|
Martin-Merida et al., 2018 (Martin-Merida et al., 2018) |
1 |
1 |
|
|
|
|
|
|
|
|
|
exon 11 c.1074–1146 (24aa) |
c.1077C > A |
p.Tyr359X |
|
Van Cauwenbergh et al., 2017 (Van Cauwenbergh et al., 2017) |
1 |
|
|
1 |
|
|
|
|
|
|
|
c.1084delA |
p.Met362X |
|
Sullivan et al., 2013 (Sullivan et al., 2013); Kiser et al., 2019 (Kiser et al., 2019) |
1 |
|
|
1 |
|
|
|
|
|
6 |
44 |
c.1098delG |
p.Leu366fs*1 |
|
Pan et al., 2014 (Pan et al., 2014) |
1 |
|
1 |
|
|
|
|
|
|
5 |
22 |
c.1115_1125del |
p.Arg372Glnfs*99 |
Same family in Ivings and Buskin paper |
Vithana et al., 2001 (Vithana et al., 2001); Ivings et al., 2008 (Ivings et al., 2008); Buskin et al., 2018 (Buskin et al., 2018) |
2 |
|
2 |
|
|
|
|
|
|
|
Severe at 47 |
c.1120C > T |
p.Gln374X |
|
Ellingford et al., 2016b (Ellingford et al., 2016b) |
1 |
|
|
1 |
|
|
|
|
|
|
|
c.1129delC |
p.Arg377Valfs*2 |
|
Carss et al., 2017 (Carss et al., 2017) |
1 |
|
1 |
|
|
|
|
|
|
|
|
c.1142delG |
p.Gly381fs*30 |
in more than 4 Japanese families in Koyanagi paper |
Sato et al., 2005 (Sato et al., 2005); Taira et al., 2007 (Taira et al., 2007); Koyanagi et al., 2019 (Koyanagi et al., 2019) |
6 |
|
6 |
|
|
|
|
|
|
|
30–45 |
intron 11 |
c.1146+2T > C |
p.? |
|
Waseem et al., 2007 (Waseem et al., 2007); Martin-Merida et al., 2018 (Martin-Merida et al., 2018) |
2 |
2 |
|
|
|
|
|
|
|
|
18, 20 |
c.1146+2T > A |
p.? |
|
Martin-Merida et al., 2018 (Martin-Merida et al., 2018) |
1 |
1 |
|
|
|
|
|
|
|
|
|
exon 12 c.1147 - 1275 |
c.1155-1159delGGACG/insAGGGATT |
p.Asp386Glyfs*28 |
|
Sato et al., 2005 (Sato et al., 2005); Sullivan et al., 2006a (Sullivan et al., 2006a) |
2 |
|
1 |
|
|
|
|
|
|
20 |
45 |
c.1190dup |
p.His398Profs*77 |
|
Jespersgaard et al., 2019 (Jespersgaard et al., 2019) |
1 |
|
1 |
|
|
|
|
|
|
|
|
c.1205C > A |
p.Ser402X |
|
McLenachan et al., 2019 (McLenachan et al., 2019) |
1 |
|
|
1 |
|
|
|
|
|
|
|
c.1215delG |
p.G405fs*7 |
|
Dong et al., 2013 (Dong et al., 2013) |
1 |
|
1 |
|
|
|
|
|
|
9 |
9,22,73 |
c.1222C > T |
p.Arg408Trp |
|
Xiao et al., 2017 (Xiao et al., 2017) |
1 |
|
|
|
1 |
|
|
|
|
|
|
c.1224dupG |
p.Gln409Alafs*66 |
|
Wu et al., 2018 (Wu et al., 2018) |
1 |
|
1 |
|
|
|
|
|
|
|
|
c.1226_1227insA |
p.Thr410Aspfs*65 |
|
Xie et al., 2018 (Xie et al., 2018) |
1 |
|
|
|
|
|
|
|
|
|
|
c.1234del |
p.Val412X |
|
Jespersgaard et al., 2019 (Jespersgaard et al., 2019) |
1 |
|
|
1 |
|
|
|
|
|
|
|
c.1261_1262delTC |
p.S421Qfs*53 |
|
Glockle et al., 2014 (Glockle et al., 2014) |
1 |
|
1 |
|
|
|
|
|
|
|
|
c.1273C > T |
p.Gln425X |
|
ClinVar (pathogenic) |
1 |
|
|
1 |
|
|
|
|
|
|
|
intron 12 |
exon 13 c.1276-1374 |
c.1291C > T |
p.Gln431X |
|
ClinVar (pathogenic) |
1 |
|
|
1 |
|
|
|
|
|
|
|
c.1305T > A |
p.Y435X |
|
Huang et al., 2015 (Huang et al., 2015) |
1 |
|
|
1 |
|
|
|
|
|
|
|
c.1373A > T |
p.Gln458Leu |
|
Xiao et al., 2017 (Xiao et al., 2017) |
1 |
|
|
|
1 |
|
|
|
|
|
|
intron 13 |
c.1374 + 654C > G |
|
deep intronic |
Rio Frio et al., 2009 (Rio Frio et al., 2009) |
1 |
1 |
|
|
|
|
|
|
|
|
|
exon 14 c.1375–1492 (39aa) |
c.1462_1472del |
p.Lys488Argfs*75 |
|
Martin-Merida et al., 2018 (Martin-Merida et al., 2018) |
1 |
|
1 |
|
|
|
|
|
|
|
|
Deletion |
upstream |
|
|
Jespersgaard et al., 2019 (Jespersgaard et al., 2019) |
1 |
|
|
|
|
|
|
|
1 |
|
|
Deletion |
exons 1–14 (whole gene) |
|
|
Ivings et al., 2008 (Ivings et al., 2008); Bowne et al., 2011 (Bowne et al., 2011); Eisenberger et al., 2013 (Eisenberger et al., 2013); Almoguera et al., 2015 (Almoguera et al., 2015) Hariri et al., 2018 (Hariri et al., 2018); Martin-Merida et al., 2018 (Martin-Merida et al., 2018) |
6 |
|
|
|
|
|
|
|
6 |
|
18, severe at 65 |
30 kb deletion including putative promoter region of a novel gene OSCAR, the entire genomic content of genes NDUFA3, TFPT and most of the PRPF31 gene except for its terminal exon exon 14. |
|
|
|
Abu-Safieh et al., 2006 (Abu-Safieh et al., 2006) |
1 |
|
|
|
|
|
|
|
1 |
6–30 |
|
112 kb deletion encompassing over 90% of PRPF31 and five upstream genes: TFPT, OSCAR, NDUFA3, TARM-1, and VSTM-1 |
|
|
|
Rose et al., 2011 (Rose et al., 2011) |
1 |
|
|
|
|
|
|
|
1 |
|
|
58.7 kb deletion including TFPT, NDUFA3, OSCAR genes and 11 exons of the PRPF31 |
|
2 families in Sweden |
|
Kohn et al., 2009 (Kohn et al., 2009); Golovleva et al., 2010 (Golovleva et al., 2010) |
2 |
|
|
|
|
|
|
|
2 |
|
50s |
Deletion |
exon 1 |
|
|
Martin-Merida et al., 2018 (Martin-Merida et al., 2018) |
1 |
|
|
|
|
|
|
|
1 |
|
|
Deletion |
exons 1–3 |
|
|
Birtel et al., 2018 (Birtel et al., 2018a) |
1 |
|
|
|
|
|
|
|
1 |
|
|
12 kb deletion including exons 1–3 of PRPF31 |
|
|
|
Dong et al., 2013 (Dong et al., 2013) |
1 |
|
|
|
|
|
|
|
1 |
|
|
Deletion |
exons 1–5 |
|
|
Eisenberger et al., 2013 (Eisenberger et al., 2013); Birtel et al., 2018 (Birtel et al., 2018a) |
2 |
|
|
|
|
|
|
|
2 |
|
|
Deletion |
Intron 1 |
|
|
Jespersgaard et al., 2019 (Jespersgaard et al., 2019) |
1 |
|
|
|
|
|
|
|
1 |
|
|
Deletion |
exons 2-3 |
|
|
Jespersgaard et al., 2019 (Jespersgaard et al., 2019) |
1 |
|
|
|
|
|
|
|
1 |
|
|
Duplication |
exons 2-5 |
|
|
Martin-Merida et al., 2018 (Martin-Merida et al., 2018) |
1 |
|
|
|
|
|
|
|
1 |
|
|
Deletion |
exons 2-5 |
|
|
Jespersgaard et al., 2019 (Jespersgaard et al., 2019) |
1 |
|
|
|
|
|
|
|
1 |
|
|
Deletion |
exons 2-14 |
|
|
Jespersgaard et al., 2019 (Jespersgaard et al., 2019) |
1 |
|
|
|
|
|
|
|
1 |
|
|
Duplication |
exons 4-5 |
|
|
Jespersgaard et al., 2019 (Jespersgaard et al., 2019) |
1 |
|
|
|
|
|
|
|
1 |
|
|
Deletion |
exons 4-13 |
|
|
Weisschuh et al., 2016 (Weisschuh et al., 2016) |
1 |
|
|
|
|
|
|
|
1 |
|
|
Deletion |
exon 9 |
|
|
Martin-Merida et al., 2018 (Martin-Merida et al., 2018) |
1 |
|
|
|
|
|
|
|
1 |
|
|
Promoter mutation |
|
|
|
Rose et al., 2012 (Rose et al., 2012) |
1 |
|
|
|
|
|
|
|
1 |
|
|
Insertion/deletion |
149 bp deleted/640 bp inserted |
hg17 Deletion of 59,310,880–59,311,028/insertion of 59,292,594–59,291,955 reverse comp. |
|
Sullivan et al., 2006b (Sullivan et al., 2006b) |
1 |
|
|
|
|
|
|
|
1 |
14/16/25/46 |
37/46/50/52/77 |
Deletion |
4.8 kb |
hg 17 59,315,842–59,320,684 |
|
Sullivan et al., 2006b (Sullivan et al., 2006b) |
1 |
|
|
|
|
|
|
|
1 |
|
|
Deletion |
11.3 kb |
hg17 59,314,340–59,325,633 |
|
Sullivan et al., 2006b (Sullivan et al., 2006b) |
1 |
|
|
|
|
|
|
|
1 |
|
|
Deletion |
32–42 kb |
hg17 5′ breakpoint: 59,290,949–59,295,848; ′ breakpoint: 59,328,550–59,333,288 |
|
Sullivan et al., 2006b (Sullivan et al., 2006b) |
1 |
|
|
|
|
|
|
|
1 |
|
|
Deletion |
>44.8 kb |
hg17 5′ breakpoint: <59,283,753; 3′ breakpoint: 59,328,550–59,333,288 |
|
Sullivan et al., 2006b (Sullivan et al., 2006b) |
1 |
|
|
|
|
|
|
|
1 |
|
|
Deletion |
19:54622548–54633842del11295 |
|
|
Carss et al., 2017 (Carss et al., 2017) |
1 |
|
|
|
|
|
|
|
1 |
|
|
Deletion |
19:54632279–54632481del203 |
|
|
Carss et al., 2017 (Carss et al., 2017) |
1 |
|
|
|
|
|
|
|
1 |
|
|