Unique variants in the PNPT1 gene

Information The variants shown are described using the NM_033109.4 transcript reference sequence.

65 entries on 1 page. Showing entries 1 - 65.
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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.-6160223_*28262964dup r.0? p.0? - VUS g.27600408_62081181dup - chr2:27600408–62081181 - FSHR_000025 - PubMed: Ellingsford 2018 - - Germline - - - - - LOVD
?/. 1 - c.77G>A r.(?) p.(Arg26Gln) - VUS g.55920882C>T g.55693747C>T PNPT1(NM_033109.4):c.77G>A (p.R26Q) - PNPT1_000013 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-?/. 1 - c.122G>C r.(?) p.(Ser41Thr) - likely benign g.55920837C>G g.55693702C>G PNPT1(NM_033109.4):c.122G>C (p.S41T) - PNPT1_000012 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+/. 1 - c.137C>G r.(?) p.(Ala46Gly) - pathogenic (recessive) g.55920822G>C g.55693687G>C - - PNPT1_000059 - PubMed: Vanniya 2022 - - Germline - - - - - Johan den Dunnen
+/. 1 - c.208T>C r.(?) p.(Ser70Pro) - pathogenic (recessive) g.55914794A>G g.55687659A>G - - PNPT1_000056 - PubMed: Pennisi 2022 - - Germline - - - - - Johan den Dunnen
?/. 1 - c.222+3A>C r.spl? p.? - VUS g.55914777T>G - PNPT1(NM_033109.5):c.222+3A>C - PNPT1_000049 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_VUmc
-?/. 1 - c.223-9A>T r.(=) p.(=) - likely benign g.55913588T>A g.55686453T>A PNPT1(NM_033109.4):c.223-9A>T - PNPT1_000028 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
?/. 1 - c.269C>G r.(?) p.(Pro90Arg) - VUS g.55913533G>C - PNPT1(NM_033109.5):c.269C>G (p.P90R) - PNPT1_000048 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_VUmc
+?/+? 1 4 c.350T>C r.(?) p.(Leu117Pro) - likely pathogenic g.55912131A>G g.55684996A>G - - PNPT1_000044 - MORL Deafness Variation Database - - SUMMARY record - - - - - Global Variome, with Curator vacancy
-/. 1 - c.361A>G r.(?) p.(Ile121Val) - benign g.55912120T>C g.55684985T>C PNPT1(NM_033109.5):c.361A>G (p.I121V) - PNPT1_000011 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Groningen
+/+ 1 4 c.394C>T r.(?) p.(Arg132*) - pathogenic g.55912087G>A g.55684952G>A - - PNPT1_000043 - MORL Deafness Variation Database - - SUMMARY record - - - - - Global Variome, with Curator vacancy
+/., ?/? 2 4i c.404-1G>A r.spl, r.spl? p.? - pathogenic (recessive), VUS g.55910970C>T g.55683835C>T 401-1G>A - PNPT1_000042 - MORL Deafness Variation Database, PubMed: Slavotinek 2015, PubMed: Slavotinek 2015 - - Germline, SUMMARY record - - - - - Global Variome, with Curator vacancy, Johan den Dunnen
+/., +?/. 2 5 c.407G>A r.(?) p.(Arg136His) - likely pathogenic, pathogenic (recessive) g.55910966C>T g.55683831C>T g.55910966G>A - PNPT1_000001 - PubMed: Pennisi 2022 - - Germline - - - - - Johan den Dunnen, Sanna Matilainen
-?/. 1 - c.412A>G r.(?) p.(Ile138Val) - likely benign g.55910961T>C g.55683826T>C PNPT1(NM_033109.4):c.412A>G (p.I138V) - PNPT1_000033 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+?/. 1 5 c.419C>T r.(?) p.(Pro140Leu) - likely pathogenic g.55910954G>A g.55683819G>A g.55910954C>T - PNPT1_000002 - - - - Germline - - - - - Sanna Matilainen
-?/. 1 - c.453+10_453+11insAG r.(=) p.(=) - likely benign g.55910910_55910911insTC g.55683775_55683776insTC PNPT1(NM_033109.4):c.453+10_453+11insAG - PNPT1_000032 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
?/. 1 - c.472G>T r.(?) p.(Ala158Ser) - VUS g.55908035C>A - PNPT1(NM_033109.4):c.472G>T (p.A158S) - PNPT1_000051 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+?/+?, -?/. 2 6 c.493C>T r.(?) p.(Pro165Ser) - likely benign, likely pathogenic g.55908014G>A g.55680879G>A PNPT1(NM_033109.4):c.493C>T (p.P165S) - PNPT1_000041 VKGL data sharing initiative Nederland MORL Deafness Variation Database - - CLASSIFICATION record, SUMMARY record - - - - - Global Variome, with Curator vacancy, VKGL-NL_Rotterdam
./. 1 - c.562G>C r.(?) p.(Val188Leu) - VUS g.55907850C>G g.55680715C>G - - PNPT1_000017 - PubMed: Saeed 2018 - - Germline - - - - - Johan den Dunnen
-?/. 1 - c.566-6T>C r.(=) p.(=) - likely benign g.55906936A>G g.55679801A>G PNPT1(NM_033109.4):c.566-6T>C - PNPT1_000027 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
?/. 1 - c.568G>T r.(?) p.(Ala190Ser) - VUS g.55906928C>A - PNPT1(NM_033109.4):c.568G>T (p.A190S) - PNPT1_000045 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+?/+? 1 8 c.575G>A r.(?) p.(Arg192Gln) - likely pathogenic g.55906921C>T g.55679786C>T - - PNPT1_000040 - MORL Deafness Variation Database - - SUMMARY record - - - - - Global Variome, with Curator vacancy
?/. 1 - c.587T>C r.(?) p.(Ile196Thr) - VUS g.55906909A>G - PNPT1(NM_033109.5):c.587T>C (p.I196T) - PNPT1_000060 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Groningen
-?/. 1 - c.622G>C r.(?) p.(Glu208Gln) - likely benign g.55906874C>G - PNPT1(NM_033109.4):c.622G>C (p.E208Q) - PNPT1_000047 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-?/. 1 - c.667A>G r.(?) p.(Lys223Glu) - likely benign g.55906829T>C g.55679694T>C PNPT1(NM_033109.4):c.667A>G (p.K223E) - PNPT1_000031 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-?/. 1 - c.789A>G r.(?) p.(Val263=) - likely benign g.55900105T>C - PNPT1(NM_033109.4):c.789A>G (p.V263=) - PNPT1_000050 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-?/. 1 - c.852G>C r.(?) p.(Val284=) - likely benign g.55900042C>G g.55672907C>G PNPT1(NM_033109.4):c.852G>C (p.V284=) - PNPT1_000030 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-?/. 1 - c.862C>T r.(?) p.(His288Tyr) - likely benign g.55900032G>A g.55672897G>A PNPT1(NM_033109.4):c.862C>T (p.H288Y) - PNPT1_000026 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+?/+? 1 10 c.887A>G r.(?) p.(Tyr296Cys) - likely pathogenic g.55899161T>C g.55672026T>C - - PNPT1_000039 - MORL Deafness Variation Database - - SUMMARY record - - - - - Global Variome, with Curator vacancy
-?/. 1 - c.1051A>G r.(?) p.(Ile351Val) - likely benign g.55895019T>C g.55667884T>C PNPT1(NM_033109.4):c.1051A>G (p.I351V) - PNPT1_000025 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-/. 1 - c.1074-19A>G r.(=) p.(=) - benign g.55894247T>C g.55667112T>C PNPT1(NM_033109.5):c.1074-19A>G - PNPT1_000010 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Groningen
-?/. 1 - c.1074-4A>T r.spl? p.? - likely benign g.55894232T>A g.55667097T>A PNPT1(NM_033109.4):c.1074-4A>T - PNPT1_000009 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+/+ 1 13 c.1160A>G r.(?) p.(Gln387Arg) - pathogenic g.55894142T>C g.55667007T>C - - PNPT1_000038 - MORL Deafness Variation Database, PubMed: Vedrenne 2012 - - SUMMARY record - - - - - Global Variome, with Curator vacancy
-?/. 1 - c.1176+7del r.(=) p.(=) - likely benign g.55894121del g.55666986del PNPT1(NM_033109.4):c.1176+7delT - PNPT1_000016 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
?/. 1 - c.1189G>A r.(?) p.(Val397Ile) - VUS g.55889149C>T g.55662014C>T PNPT1(NM_033109.4):c.1189G>A (p.V397I) - PNPT1_000008 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
?/. 1 - c.1228C>G r.(?) p.(Gln410Glu) - VUS g.55889110G>C - PNPT1(NM_033109.4):c.1228C>G (p.(Gln410Glu)) - PNPT1_000063 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
-?/. 1 - c.1231G>A r.(?) p.(Val411Ile) - likely benign g.55889107C>T g.55661972C>T PNPT1(NM_033109.3):c.1231G>A (p.(Val411Ile)) - PNPT1_000024 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
-?/. 1 - c.1251G>A r.(?) p.(Gly417=) - likely benign g.55887325C>T g.55660190C>T PNPT1(NM_033109.4):c.1251G>A (p.G417=) - PNPT1_000007 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-/. 1 - c.1285-4del r.spl? p.? - benign g.55883522del g.55656387del PNPT1(NM_033109.4):c.1285-4delT - PNPT1_000022 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-/. 1 - c.1390C>A r.(?) p.(Arg464=) - benign g.55883317G>T g.55656182G>T PNPT1(NM_033109.5):c.1390C>A (p.R464=) - PNPT1_000006 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Groningen
+/. 1 - c.1399C>T r.(?) p.(Pro467Ser) - pathogenic (recessive) g.55883308G>A g.55656173G>A - - PNPT1_000055 - PubMed: Pennisi 2022 - - Germline - - - - - Johan den Dunnen
?/. 1 - c.1414G>A r.(?) p.(Val472Ile) - VUS g.55883293C>T - PNPT1(NM_033109.4):c.1414G>A (p.V472I) - PNPT1_000046 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+/+ 1 17 c.1424A>G r.(?) p.(Glu475Gly) - pathogenic g.55883283T>C g.55656148T>C - - PNPT1_000037 - MORL Deafness Variation Database, PubMed: von Ameln 2012 - - SUMMARY record - - - - - Global Variome, with Curator vacancy
+/+, +?/. 2 18 c.1453A>G r.(?) p.(Met485Val) - likely pathogenic, pathogenic g.55882077T>C g.55654942T>C PNPT1(NM_033109.4):c.1453A>G (p.(Met485Val)) - PNPT1_000036 VKGL data sharing initiative Nederland MORL Deafness Variation Database - - CLASSIFICATION record, SUMMARY record - - - - - Global Variome, with Curator vacancy, VKGL-NL_Leiden
+/. 1 - c.1495G>A r.(?) p.(Gly499Arg) - pathogenic (recessive) g.55882035C>T g.55654900C>T - - PNPT1_000054 - PubMed: Pennisi 2022 - - Germline - - - - - Johan den Dunnen
+/., +?/+?, +?/. 4 19 c.1519G>T r.(?) p.(Ala507Ser) - likely pathogenic, pathogenic (recessive) g.55874565C>A g.55647430C>A PNPT1(NM_033109.3):c.1519G>T (p.(Ala507Ser)) - PNPT1_000021 VKGL data sharing initiative Nederland MORL Deafness Variation Database, PubMed: Slavotinek 2015, PubMed: Pennisi 2022, 1 more item - - CLASSIFICATION record, Germline, SUMMARY record - - - - - Global Variome, with Curator vacancy, Johan den Dunnen, VKGL-NL_Leiden
+/., +?/+? 2 19 c.1525G>A r.(?) p.(Val509Ile) - likely pathogenic, pathogenic (recessive) g.55874559C>T g.55647424C>T - - PNPT1_000035 - MORL Deafness Variation Database, PubMed: Posey 2016, PubMed: Yang 2013, PubMed: Pennisi 2022 - - Germline, SUMMARY record - - - - - Global Variome, with Curator vacancy, Johan den Dunnen
+/. 1 - c.1579_1580insGAT r.(?) p.(Tyr527Ter) - pathogenic (recessive) g.55874505_55874506insTCA g.55647370_55647371insTCA - - PNPT1_000053 - PubMed: Pennisi 2022 - - Germline - - - - - Johan den Dunnen
?/. 1 - c.1582C>A r.(?) p.(Arg528Ser) - VUS g.55874502G>T g.55647367G>T - - PNPT1_000015 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
+/., +?/+?, +?/. 3 19 c.1592C>G r.(?) p.(Thr531Arg) - likely pathogenic, pathogenic (recessive) g.55874492G>C g.55647357G>C PNPT1(NM_033109.3):c.1592C>G (p.(Thr531Arg)) - PNPT1_000020 VKGL data sharing initiative Nederland MORL Deafness Variation Database, PubMed: Posey 2016, PubMed: Yang 2013, PubMed: Pennisi 2022 - - CLASSIFICATION record, Germline, SUMMARY record - - - - - Global Variome, with Curator vacancy, Johan den Dunnen, VKGL-NL_Leiden
+/. 1 - c.1619A>G r.(?) p.(Asn540Ser) - pathogenic (recessive) g.55873605T>C g.55646470T>C - - PNPT1_000058 - PubMed: Vanniya 2022 - - Germline - - - - - Johan den Dunnen
?/. 1 - c.1676C>G r.(?) p.(Ala559Gly) - VUS g.55873456G>C - PNPT1(NM_033109.5):c.1676C>G (p.(Ala559Gly)) - PNPT1_000065 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
-?/. 1 - c.1929T>C r.(?) p.(Asp643=) - likely benign g.55870538A>G g.55643403A>G PNPT1(NM_033109.4):c.1929T>C (p.D643=) - PNPT1_000029 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-?/. 1 - c.1938G>A r.(?) p.(Thr646=) - likely benign g.55870529C>T - PNPT1(NM_033109.4):c.1938G>A (p.T646=) - PNPT1_000057 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
?/. 1 - c.1972C>T r.(?) p.(His658Tyr) - VUS g.55870495G>A - PNPT1(NM_033109.5):c.1972C>T (p.(His658Tyr)) - PNPT1_000064 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
+?/+? 1 25 c.2050G>A r.(?) p.(Ala684Thr) - likely pathogenic g.55870312C>T g.55643177C>T - - PNPT1_000034 - MORL Deafness Variation Database - - SUMMARY record - - - - - Global Variome, with Curator vacancy
?/. 1 - c.2068A>C r.(?) p.(=) - VUS g.55870294T>G - PNPT1(NM_033109.5):c.2068A>C (p.(Arg690=)) - PNPT1_000061 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
-?/. 1 - c.2070-5T>C r.spl? p.? - likely benign g.55867845A>G g.55640710A>G PNPT1(NM_033109.4):c.2070-5T>C - PNPT1_000005 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+/. 1 - c.2137G>T r.(?) p.(Asp713Tyr) - pathogenic (recessive) g.55867773C>A g.55640638C>A - - PNPT1_000052 - PubMed: Pennisi 2022 - - Germline - - - - - Johan den Dunnen
-?/. 1 - c.2149-5dup r.spl? p.? - likely benign g.55864747dup g.55637612dup PNPT1(NM_033109.3):c.2149-5dup (p.?) - PNPT1_000019 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
?/. 1 - c.2185C>A r.(?) p.(Gln729Lys) - VUS g.55864698G>T g.55637563G>T - - PNPT1_000014 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
?/. 1 - c.2213G>A r.(?) p.(Arg738His) - VUS g.55863511C>T g.55636376C>T PNPT1(NM_033109.4):c.2213G>A (p.R738H) - PNPT1_000018 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-?/. 1 - c.2247A>G r.(?) p.(Arg749=) - likely benign g.55863477T>C g.55636342T>C PNPT1(NM_033109.4):c.2247A>G (p.R749=) - PNPT1_000004 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
?/. 1 - c.2275G>A r.(?) p.(Val759Met) - VUS g.55863449C>T - PNPT1(NM_033109.4):c.2275G>A (p.(Val759Met)) - PNPT1_000062 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
-/. 1 - c.*11dup r.(?) p.(=) - benign g.55863371dup g.55636236dup PNPT1(NM_033109.5):c.2352_*1insT - PNPT1_000003 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Groningen
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