Unique variants in the POLR3A gene

Information The variants shown are described using the NM_007055.3 transcript reference sequence.

179 entries on 2 pages. Showing entries 1 - 100.
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Effect     

Reported     

Exon     

AscendingDNA change (cDNA)     

RNA change     

Protein     

Classification method     

Clinical classification     

DNA change (genomic) (hg19)     

DNA change (hg38)     

Published as     

ISCN     

DB-ID     

Variant remarks     

Reference     

ClinVar ID     

dbSNP ID     

Origin     

Segregation     

Frequency     

Re-site     

VIP     

Methylation     

Owner     
?/. 1 - c.-4482T>G r.(?) p.(=) - VUS g.79793647A>C - RPS24(NM_033022.4):c.-13A>C - POLR3A_000099 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Groningen
+?/. 2 1 c.-35C>G r.? p.? - likely pathogenic (recessive) g.79789200G>C g.78029442G>C - - POLR3A_000121 - PubMed: La Piana 2016 - - Germline - - - - - Johan den Dunnen
+/. 1 - c.3G>T r.(?) p.0? - pathogenic g.79789163C>A g.78029405C>A Met1? - POLR3A_000027 - PubMed: Paolacci 2018 - - Germline - - - - - Johan den Dunnen
+?/. 1 1i c.44+5G>C r.44_45ins[GTAAC;44+6_44+10] p.Ile15Metfs*2 ACMG VUS g.79789117C>G g.78029359C>G - - POLR3A_000132 - - - - Germline yes - - - - Xiaomei Luo
-?/. 1 - c.45-3C>T r.spl? p.? - likely benign g.79785990G>A - POLR3A(NM_007055.4):c.45-3C>T - POLR3A_000143 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
+/. 1 - c.103C>T r.(?) p.(Gln35Ter) - pathogenic g.79785929G>A g.78026171G>A POLR3A(NM_007055.4):c.103C>T (p.Q35*) - POLR3A_000069 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_VUmc
-?/. 1 - c.156A>G r.(?) p.(Leu52=) - likely benign g.79785876T>C g.78026118T>C POLR3A(NM_007055.3):c.156A>G (p.L52=) - POLR3A_000068 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+/., ?/. 3 2 c.169G>A r.(?) p.(Asp57Asn) - pathogenic (recessive), VUS g.79785863C>T g.78026105C>T POLR3A(NM_007055.3):c.169G>A (p.D57N) - POLR3A_000067 VKGL data sharing initiative Nederland PubMed: Timmons 2006, PubMed: Wolf 2014 - - CLASSIFICATION record, Germline - - - - - Johan den Dunnen, VKGL-NL_Rotterdam
?/. 1 - c.212C>G r.(?) p.(Thr71Ser) - VUS g.79785486G>C g.78025728G>C POLR3A(NM_007055.3):c.212C>G (p.T71S) - POLR3A_000066 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-?/. 1 - c.229G>T r.(?) p.(Ala77Ser) - likely benign g.79785469C>A g.78025711C>A POLR3A(NM_007055.3):c.229G>T (p.A77S) - POLR3A_000065 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+/. 2 3 c.272C>T r.(?) p.(Pro91Leu) - pathogenic (recessive) g.79785426G>A g.78025668G>A - - POLR3A_000077 - PubMed: Daoud 2013, PubMed: Wolf 2014 - - Germline - - - - - Johan den Dunnen
?/. 1 - c.295A>G r.(?) p.(Arg99Gly) - VUS g.79785403T>C - POLR3A(NM_007055.4):c.295A>G (p.R99G) - POLR3A_000129 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Groningen
-?/. 1 - c.318+7A>C r.(=) p.(=) - likely benign g.79785373T>G - POLR3A(NM_007055.4):c.318+7A>C - POLR3A_000126 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
?/. 1 - c.322A>G r.(?) p.(Ile108Val) - VUS g.79784897T>C - POLR3A(NM_007055.3):c.322A>G (p.I108V) - POLR3A_000098 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-?/. 1 - c.346A>G r.(?) p.(Met116Val) - likely benign g.79784873T>C g.78025115T>C - - POLR3A_000076 - PubMed: Khalifa 2015 - - Germline - - - - - Johan den Dunnen
+/. 2 4 c.367_369del r.(?) p.(Lys123del) - pathogenic (recessive) g.79784855_79784857del g.78025097_78025099del 364_366delAAG - POLR3A_000076 - PubMed: Wolf 2014 - - Germline - - - - - Johan den Dunnen
+/. 1 4 c.418C>T r.(?) p.(Arg140*) - pathogenic (recessive) g.79784801G>A g.78025043G>A - - POLR3A_000043 - PubMed: Bernard 2011 - - Germline - - - - - Johan den Dunnen
-?/. 1 - c.438C>T r.(?) p.(Ile146=) - likely benign g.79784781G>A g.78025023G>A POLR3A(NM_007055.3):c.438C>T (p.I146=) - POLR3A_000064 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+/. 2 4 c.441dup r.(?) p.(Asp148*), p.(Asp148Ter) - pathogenic (recessive) g.79784778dup g.78025020dup 441dupT - POLR3A_000075 - PubMed: Wolf 2014 - - Germline - - - - - Johan den Dunnen
-?/. 1 - c.491-9C>T r.(=) p.(=) - likely benign g.79784470G>A - POLR3A(NM_007055.3):c.491-9C>T - POLR3A_000088 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+/. 4 5 c.496G>A r.(?) p.(Val166Ile) - pathogenic (recessive) g.79784456C>T g.78024698C>T - - POLR3A_000074 - PubMed: Daoud 2013, PubMed: Wolf 2014 - - Germline - - - - - Johan den Dunnen
+/. 2 5 c.552_553delinsT r.(?) p.(Lys184Asnfs*35), p.(Lys184AsnfsTer35) - pathogenic (recessive) g.79784399_79784400delinsA g.78024641_78024642delinsA 550_553delinsAAT, c.550_553delinsAAT - POLR3A_000073 - PubMed: Wolf 2014 - - Germline - - - - - Johan den Dunnen
+/. 1 - c.601del r.(?) p.(Ile201LeufsTer18) - pathogenic (recessive) g.79784351del g.78024593del 601delA - POLR3A_000120 - PubMed: Perrier 2020 - - Germline - - - - - Johan den Dunnen
+?/. 1 5i_8i c.(645+1_646-1)_(1185+1_1186-1)del r.? p.? - likely pathogenic (recessive) g.(79779024_79781303)_(79782143_79784306)del g.(78019266_78021545)_(78022385_78024548)del del ex6-8 - POLR3A_000102 variants in POLR3A are disease causing, either solely or in combination with the POLR3B variants PubMed: Mirchi 2023 - - Germline - - - - - Johan den Dunnen
?/. 2 - c.740C>T r.(?) p.(Pro247Leu) - VUS g.79782048G>A g.78022290G>A POLR3A(NM_007055.3):c.740C>T (p.(Pro247Leu)) - POLR3A_000013 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden, VKGL-NL_Nijmegen
-?/. 1 - c.741G>A r.(?) p.(=) - likely benign g.79782047C>T - POLR3A(NM_007055.4):c.741G>A (p.(Pro247=)) - POLR3A_000125 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
+/., +?/. 2 - c.760C>T r.(?) p.(Arg254Ter) - likely pathogenic, pathogenic g.79782028G>A g.78022270G>A POLR3A(NM_007055.3):c.760C>T (p.R254*) - POLR3A_000016 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam, VKGL-NL_Nijmegen
-?/. 1 - c.927C>T r.(?) p.(Asp309=) - likely benign g.79781739G>A g.78021981G>A POLR3A(NM_007055.3):c.927C>T (p.D309=) - POLR3A_000012 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
?/. 1 - c.970G>A r.(?) p.(Glu324Lys) - VUS g.79781696C>T - POLR3A(NM_007055.3):c.970G>A (p.E324K) - POLR3A_000092 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
?/. 1 - c.973C>T r.(?) p.(Leu325Phe) - VUS g.79781693G>A - POLR3A(NM_007055.4):c.973C>T (p.L325F) - POLR3A_000091 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Groningen
+/. 1 7i c.1048+1G>A r.spl p.? - pathogenic (recessive) g.79781617C>T g.78021859C>T - - POLR3A_000119 - PubMed: La Piana 2016 - - Germline - - - - - Johan den Dunnen
+/. 2 - c.1048+5G>T r.1048_1049ins[guagu;1048+6_1049-1], r.spl p.(Glu350Glufs*27), p.Arg353Profs*24 - pathogenic, pathogenic (recessive) g.79781613C>A g.78021855C>A - - POLR3A_000019 - PubMed: Harting 2020, PubMed: Paolacci 2018 - rs890755853 Germline - - - - - Johan den Dunnen
+/., +?/. 3 - c.1051C>T r.(?) p.(Arg351Ter) - likely pathogenic (recessive), pathogenic (recessive) g.79781438G>A g.78021680G>A - - POLR3A_000118 - PubMed: Mirchi 2023, PubMed: Perrier 2020 - - Germline - - - - - Johan den Dunnen
+?/. 3 - c.1078A>G r.(?) p.(Arg360Gly) - likely pathogenic (dominant) g.79781411T>C g.78021653T>C - - POLR3A_000150 ACMG PP2, PP3(moderate), PM2, PS3, ACMG PP2, PP3(moderate), PM2, PS4, 1 more item Journal: Ramos 2025 - - Germline, Germline/De novo (untested) - - - - - Johan den Dunnen
-?/. 1 - c.1113C>T r.(?) p.(Pro371=) - likely benign g.79781376G>A g.78021618G>A POLR3A(NM_007055.3):c.1113C>T (p.P371=) - POLR3A_000073 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+/. 3 8 c.1114G>A r.(?) p.(Asp372Asn) - pathogenic (recessive) g.79781375C>T g.78021617C>T - - POLR3A_000042 - PubMed: Bernard 2011, PubMed: Wolf 2014, PubMed: Yazidi 2019 - - Germline - - - - - Johan den Dunnen
+/. 2 8 c.1160C>G r.(?) p.(Ala387Gly) - pathogenic (recessive) g.79781329G>C g.78021571G>C - - POLR3A_000072 - PubMed: Daoud 2013, PubMed: Wolf 2014 - - Germline - - - - - Johan den Dunnen
?/. 1 - c.1163A>G r.(?) p.(Lys388Arg) - VUS g.79781326T>C - POLR3A(NM_007055.4):c.1163A>G (p.(Lys388Arg)) - POLR3A_000124 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
+/., +?/., ?/. 5 9 c.1186G>T r.(?) p.(Val396Leu) - likely pathogenic (recessive), pathogenic (recessive), VUS g.79779023C>A g.78019265C>A POLR3A(NM_007055.4):c.1186G>T (p.V396L) - POLR3A_000071 VKGL data sharing initiative Nederland PubMed: Mirchi 2023, PubMed: Wolf 2014, PubMed: Yazidi 2019 - - CLASSIFICATION record, Germline - - - - - Johan den Dunnen, VKGL-NL_Groningen
+/. 1 9i c.1289+3A>C r.spl p.? - pathogenic (recessive) g.79778917T>G g.78019159T>G - - POLR3A_000117 - PubMed: La Piana 2016 - - Germline - - - - - Johan den Dunnen
?/. 1 - c.1290-5T>C r.spl? p.? - VUS g.79777479A>G - POLR3A(NM_007055.4):c.1290-5T>C - POLR3A_000142 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
+/. 2 10 c.1301dup r.(?) p.(Tyr434*), p.(Tyr434Ter) - pathogenic (recessive) g.79777463dup g.78017705dup 1302insA - POLR3A_000070 - PubMed: Daoud 2013, PubMed: Wolf 2014 - - Germline - - - - - Johan den Dunnen
-?/. 1 - c.1302C>T r.(?) p.(Tyr434=) - likely benign g.79777462G>A g.78017704G>A POLR3A(NM_007055.3):c.1302C>T (p.Y434=) - POLR3A_000063 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-?/. 1 - c.1329G>C r.(?) p.(Glu443Asp) - likely benign g.79777435C>G g.78017677C>G POLR3A(NM_007055.3):c.1329G>C (p.E443D) - POLR3A_000062 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-?/. 1 - c.1345A>G r.(?) p.(Ile449Val) - likely benign g.79777419T>C g.78017661T>C POLR3A(NM_007055.3):c.1345A>G (p.I449V) - POLR3A_000061 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+/., +?/. 2 - c.1369G>A r.(?) p.(Gly457Arg) - likely pathogenic (recessive), pathogenic (recessive) g.79777395C>T g.78017637C>T - - POLR3A_000116 - PubMed: Mirchi 2023, PubMed: Yazidi 2019 - - Germline - - - - - Johan den Dunnen
-?/. 1 - c.1380G>A r.(?) p.(Val460=) - likely benign g.79777384C>T g.78017626C>T POLR3A(NM_007055.3):c.1380G>A (p.V460=) - POLR3A_000060 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+/. 2 11 c.1433C>G r.(?) p.(Ala478Gly) - pathogenic (recessive) g.79773547G>C g.78013789G>C - - POLR3A_000069 - PubMed: Wolf 2005, PubMed: Wolf 2014 - - Germline - - - - - Johan den Dunnen
?/. 1 - c.1450C>T r.(?) p.(Arg484Trp) - VUS g.79773530G>A - POLR3A(NM_007055.3):c.1450C>T (p.R484W) - POLR3A_000097 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-?/. 1 - c.1475T>C r.(?) p.(Val492Ala) - likely benign g.79773505A>G - POLR3A(NM_007055.3):c.1475T>C (p.V492A) - POLR3A_000081 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+/. 1 11i_15i c.(1572+1_1573-1)_(2074+1_2075-1)del r.? p.? - pathogenic (recessive) g.(79764647_79767459)_(79770299_79773407)del - del ex12-15 - POLR3A_000031 - PubMed: Paolacci 2018 - - Germline - - - - - Johan den Dunnen
?/. 1 - c.1624A>G r.(?) p.(Ile542Val) - VUS g.79770247T>C g.78010489T>C POLR3A(NM_007055.3):c.1624A>G (p.I542V) - POLR3A_000059 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+/. 2 13 c.1658C>T r.(?) p.(Thr553Ile) - pathogenic (recessive) g.79769734G>A g.78009976G>A - - POLR3A_000068 - PubMed: Wolf 2014 - - Germline - - - - - Johan den Dunnen
+/., +?/. 8 13 c.1674C>G r.(?) p.(Phe558Leu) - likely pathogenic (recessive), pathogenic (recessive) g.79769718G>C g.78009960G>C - - POLR3A_000041 - PubMed: Bernard 2011, PubMed: Mirchi 2023, PubMed: Wolf 2014, PubMed: Yazidi 2019 - - Germline - - - - - Johan den Dunnen
+/. 1 - c.1681C>T r.1681c>u p.Arg561Ter - pathogenic (recessive) g.79769711G>A g.78009953G>A - - POLR3A_000115 - PubMed: Perrier 2020 - - Germline - - - - - Johan den Dunnen
+?/., -?/. 2 - c.1724A>T r.(?) p.(Lys575Met) - likely benign, likely pathogenic (recessive) g.79769668T>A g.78009910T>A POLR3A(NM_007055.3):c.1724A>T (p.(Lys575Met)) - POLR3A_000077 VKGL data sharing initiative Nederland PubMed: Khalifa 2015 - - CLASSIFICATION record, Germline - - - - - Johan den Dunnen, VKGL-NL_Leiden
+/. 2 13 c.1740dup r.(?) p.(Val581Serfs*28), p.(Val581SerfsTer28) - pathogenic (recessive) g.79769654dup g.78009896dup 1741insA - POLR3A_000067 - PubMed: Daoud 2013, PubMed: Wolf 2014 - - Germline - - - - - Johan den Dunnen
+?/., -/. 2 - c.1745G>A r.(?) p.(Arg582His) - benign, likely pathogenic (recessive) g.79769647C>T g.78009889C>T POLR3A(NM_007055.3):c.1745G>A (p.R582H) - POLR3A_000011 VKGL data sharing initiative Nederland PubMed: Khalifa 2015 - - CLASSIFICATION record, Germline - - - - - Johan den Dunnen, VKGL-NL_Rotterdam
-?/. 2 - c.1745G>T r.(?) p.(Arg582Leu) - likely benign g.79769647C>A - POLR3A(NM_007055.3):c.1745G>T (p.(Arg582Leu)), POLR3A(NM_007055.4):c.1745G>T (p.R582L) - POLR3A_000096 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden, VKGL-NL_Utrecht
+/., +?/. 16 - c.1771-7C>G r.(1771_1909del), r.(1771_1909del,1643_1909del), r.1771_1909del,1643_1909del, r.spl, 1 more item p.(Pro591Metfs*9), p.(Pro591Metfs*9,Gly548_Tyr637del), p.Pro591Metfs*9,Gly548_Tyr637del, 1 more item - likely pathogenic, likely pathogenic (recessive), pathogenic (recessive) g.79769440G>C g.78009682G>C - - POLR3A_000010 - PubMed: Harting 2020, PubMed: Mirchi 2023, PubMed: Perrier 2020 - - Germline - - - - - Johan den Dunnen, IMGAG
+?/., ?/. 2 - c.1771-6C>A r.(=) p.(=) - likely pathogenic, VUS g.79769439G>T g.78009681G>T POLR3A(NM_007055.3):c.1771-6C>A, POLR3A(NM_007055.4):c.1771-6C>A - POLR3A_000058 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam, VKGL-NL_VUmc
+/., +?/. 8 13i c.1771-6C>G r.(1771_1909del), r.spl p.(Pro591Metfs*9), p.? - likely pathogenic, likely pathogenic (recessive), pathogenic (recessive) g.79769439G>C g.78009681G>C - - POLR3A_000003 - PubMed: Harting 2020, PubMed: La Piana 2016, PubMed: Mirchi 2023 - - Germline, Germline/De novo (untested) - - - - - Johan den Dunnen, IMGAG
-/. 1 - c.1771-5del r.spl? p.? - benign g.79769444del g.78009686del POLR3A(NM_007055.3):c.1771-5delC - POLR3A_000072 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-?/. 1 - c.1771-5dup r.spl? p.? - likely benign g.79769444dup - POLR3A(NM_007055.4):c.1771-5dup - POLR3A_000123 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
?/. 1 - c.1787C>T r.(?) p.(Thr596Met) - VUS g.79769417G>A - POLR3A(NM_007055.4):c.1787C>T (p.T596M) - POLR3A_000087 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_VUmc
+/. 2 14 c.1795C>A r.(?) p.(Gln599Lys) - pathogenic (recessive) g.79769409G>T g.78009651G>T - - POLR3A_000066 - PubMed: Wolf 2014 - - Germline - - - - - Johan den Dunnen
+/. 4 14 c.1797G>C r.(?) p.(Gln599His) - pathogenic (recessive) g.79769407C>G g.78009649C>G - - POLR3A_000065 - PubMed: Bekiesinska-Figatowska 2010, PubMed: Wolf 2014 - - Germline - - - - - Johan den Dunnen
+/. 1 - c.1800C>T r.[1771_1909del,=] p.[Pro591Metfs*9,=] - pathogenic (recessive) g.79769404G>A g.78009646G>A - - POLR3A_000023 - PubMed: Paolacci 2018 - - Germline - - - - - Johan den Dunnen
+/. 2 14 c.1804A>C r.(?) p.(Ser602Arg) - pathogenic (recessive) g.79769400T>G g.78009642T>G - - POLR3A_000064 - PubMed: Daoud 2013, PubMed: Wolf 2014 - - Germline - - - - - Johan den Dunnen
+/. 1 - c.1896_1897del r.(?) p.(Ala633GlnfsTer2) - pathogenic g.79769307_79769308del - - - POLR3A_000078 - - - - Unknown - - - - - IMGAG
+/. 1 14 c.1907C>A r.(?) p.(Ser636Tyr) - pathogenic (recessive) g.79769297G>T g.78009539G>T - - POLR3A_000040 - PubMed: Bernard 2011 - - Germline - - - - - Johan den Dunnen
+/., +?/. 10 14i c.1909+18G>A r.(=), r.spl, r.[=,1909_1910ins[1909+1_1909+17;ag]] p.(=), p.(Tyr637Cysfs*14), p.[=,Tyr637Cysfs*14] - likely pathogenic (recessive), pathogenic, pathogenic (recessive) g.79769277C>T g.78009519C>T 2003+18G>A - POLR3A_000018 VKGL data sharing initiative Nederland PubMed: Bernard 2011, PubMed: Mirchi 2023, PubMed: Paolacci 2018, PubMed: Yazidi 2019 - rs267608677 CLASSIFICATION record, Germline yes - - - - Johan den Dunnen, VKGL-NL_Nijmegen
+/., +?/., ?/. 12 14i c.1909+22G>A r.(=), r.(?), r.spl, r.[(=,1771_1909del)], r.[=,1771_1909del] p.(=), p.(?), p.?, p.[(=,Pro591Metfs*9)], p.[=,Pro59fs], p.[=,Pro59Metfs*9] - likely pathogenic, pathogenic, pathogenic (recessive), VUS g.79769273C>T g.78009515C>T POLR3A(NM_007055.3):c.1909+22G>A, POLR3A(NM_007055.4):c.1909+22G>A - POLR3A_000021 VKGL data sharing initiative Nederland PubMed: La Piana 2016, PubMed: Paolacci 2018 - rs191875469 CLASSIFICATION record, Germline, Unknown - - - - - Johan den Dunnen, VKGL-NL_Leiden, IMGAG, VKGL-NL_Rotterdam, VKGL-NL_Utrecht, VKGL-NL_Nijmegen, MobiDetails
+/. 7 15 c.1930G>A r.(?) p.(Glu644Lys) - pathogenic (recessive) g.79767604C>T g.78007846C>T - - POLR3A_000063 - PubMed: Wolf 2014, PubMed: Yazidi 2019 - - Germline - - - - - Johan den Dunnen
+/. 3 15 c.1935G>C r.(?) p.(Leu645Phe) - pathogenic (recessive) g.79767599C>G g.78007841C>G - - POLR3A_000062 - PubMed: Wolf 2014, PubMed: Yazidi 2019 - - Germline - - - - - Johan den Dunnen
+/. 2 15 c.2005C>G r.(?) p.(Arg669Gly) - pathogenic (recessive) g.79767529G>C g.78007771G>C - - POLR3A_000061 - PubMed: Wolf 2007, PubMed: Wolf 2014 - - Germline - - - - - Johan den Dunnen
+?/. 1 - c.2010C>G r.(?) p.(Asp670Glu) - likely pathogenic g.79767524G>C g.78007766G>C POLR3A(NM_007055.4):c.2010C>G (p.D670E) - POLR3A_000057 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Utrecht
+/. 6 15 c.2011T>C r.(?) p.(Trp671Arg) - pathogenic (recessive) g.79767523A>G g.78007765A>G - - POLR3A_000060 - PubMed: Daoud 2013, PubMed: Wolf 2014 - - Germline - - - - - Johan den Dunnen
+/., +?/. 29 15 c.2015G>A r.(?) p.(Gly672Glu) - likely pathogenic (recessive), pathogenic, pathogenic (recessive) g.79767519C>T g.78007761C>T - - POLR3A_000039 no variant 2nd chromosome PubMed: Bernard 2011, PubMed: Mirchi 2023, PubMed: Wolf 2014, PubMed: Yazidi 2019 - - Germline yes - - - - Johan den Dunnen
+/. 4 15 c.2039T>C r.(?) p.(Met680Thr) - pathogenic (recessive) g.79767495A>G g.78007737A>G - - POLR3A_000059 - PubMed: Wolf 2014 - - Germline - - - - - Johan den Dunnen
+/. 3 15 c.2045G>A r.(?) p.(Arg682Gln) - pathogenic (recessive) g.79767489C>T g.78007731C>T - - POLR3A_000056 - PubMed: Harting 2020, PubMed: Wolf 2005, PubMed: Wolf 2014 - - Germline - - - - - Johan den Dunnen
+/. 2 16 c.2098A>T r.(?) p.(Ile700Phe) - pathogenic (recessive) g.79764623T>A g.78004865T>A - - POLR3A_000058 - PubMed: Timmons 2006, PubMed: Wolf 2014 - - Germline - - - - - Johan den Dunnen
+/. 2 - c.2119C>T r.(?) p.(Gln707Ter) - pathogenic, pathogenic (recessive) g.79764602G>A g.78004844G>A - - POLR3A_000055 VKGL data sharing initiative Nederland PubMed: Perrier 2020 - - CLASSIFICATION record, Germline - - - - - Johan den Dunnen, VKGL-NL_Nijmegen
?/. 1 - c.2165A>G r.(?) p.(Lys722Arg) - VUS g.79764556T>C - POLR3A(NM_007055.4):c.2165A>G (p.K722R) - POLR3A_000086 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Groningen
+/. 1 16 c.2171G>A r.(?) p.(Cys724Tyr) - pathogenic (recessive) g.79764550C>T g.78004792C>T - - POLR3A_000038 - PubMed: Bernard 2011 - - Germline - - - - - Johan den Dunnen
+/. 1 - c.2270C>T r.(?) p.(Ser757Phe) - pathogenic (dominant) g.79762044G>A g.78002286G>A - - POLR3A_000149 ACMG PP2, PP3(moderate), PM2, PS2, PS3 Journal: Ramos 2025 - - De novo - - - - - Johan den Dunnen
+?/. 2 - c.2278C>T r.(?) p.(Arg760Cys) - likely pathogenic (dominant) g.79762036G>A g.78002278G>A - - POLR3A_000148 ACMG PP2, PP3(moderate), PM2, PS3 Journal: Ramos 2025 - - De novo - - - - - Johan den Dunnen
+/. 3 17 c.2324A>T r.(?) p.(Asn775Ile) - pathogenic (recessive) g.79761990T>A g.78002232T>A - - POLR3A_000037 - PubMed: Bernard 2011, PubMed: Wolf 2014, PubMed: Yazidi 2019 - - Germline - - - - - Johan den Dunnen
-?/. 1 - c.2334C>T r.(?) p.(Leu778=) - likely benign g.79761980G>A - POLR3A(NM_007055.3):c.2334C>T (p.L778=) - POLR3A_000080 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-?/. 1 - c.2344C>T r.(?) p.(Leu782=) - likely benign g.79761970G>A g.78002212G>A POLR3A(NM_007055.3):c.2344C>T (p.L782=) - POLR3A_000054 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+/. 2 17 c.2350G>A r.(?) p.(Gly784Ser) - pathogenic (recessive) g.79761964C>T g.78002206C>T - - POLR3A_000057 - PubMed: Wolf 2014 - - Germline - - - - - Johan den Dunnen
+/. 1 - c.2357A>G r.(?) p.(Lys786Arg) - pathogenic (dominant) g.79761957T>C g.78002199T>C - - POLR3A_000147 ACMG PP2, PP3, PM2, PS2, PS3 Journal: Ramos 2025 - - De novo - - - - - Johan den Dunnen
+/. 2 18 c.2381A>C r.(?) p.(Gln794Pro) - pathogenic (recessive) g.79760831T>G g.78001073T>G - - POLR3A_000114 - PubMed: La Piana 2016 - - Germline - - - - - Johan den Dunnen
-?/. 1 - c.2394T>C r.(?) p.(Cys798=) - likely benign g.79760818A>G - POLR3A(NM_007055.4):c.2394T>C (p.C798=) - POLR3A_000100 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Utrecht
?/. 1 - c.2395G>C r.(?) p.(Val799Leu) - VUS g.79760817C>G - POLR3A(NM_007055.4):c.2395G>C (p.(Val799Leu)) - POLR3A_000141 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
+/. 1 - c.2422C>T r.(?) p.(Arg808*) - pathogenic g.79760790G>A - - - POLR3A_000140 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
-?/. 1 - c.2433C>T r.(?) p.(Asp811=) - likely benign g.79760779G>A g.78001021G>A POLR3A(NM_007055.3):c.2433C>T (p.D811=) - POLR3A_000053 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+?/. 1 - c.2434G>A r.(?) p.(Gly812Ser) - likely pathogenic (recessive) g.79760778C>T g.78001020C>T - - POLR3A_000113 variants in POLR3A are disease causing, either solely or in combination with the POLR3B variants PubMed: Mirchi 2023 - - Germline - - - - - Johan den Dunnen
+/. 1 - c.2471A>C r.(?) p.(His824Pro) - pathogenic (recessive) g.79760741T>G g.78000983T>G - - POLR3A_000128 - PubMed: Salinas 2020 VCV000590969.1 - Germline - - - - - Johan den Dunnen
-?/. 2 - c.2472C>T r.(?) p.(His824=) - likely benign g.79760740G>A g.78000982G>A POLR3A(NM_007055.3):c.2472C>T (p.H824=), POLR3A(NM_007055.4):c.2472C>T (p.(His824=)) - POLR3A_000009 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden, VKGL-NL_Rotterdam
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