Unique variants in the SEPN1 gene

NOTE: gene symbol was recently changed from SEPN1 to SELENON. This database is one of the gene variant databases from the "Leiden Muscular Dystrophy pages" (LMDp).
Information The variants shown are described using the NM_020451.2 transcript reference sequence.

200 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     
-/. 3 1 c.-42T>C r.(?) p.(=) - benign g.26126680T>C g.25800189T>C - - SEPN1_000102 also found in controls, VKGL data sharing initiative Nederland from website {DBsub-Emory} - rs12121707 CLASSIFICATION record, Germline, Unknown - - - - - Madhuri Hegde, Andreas Laner, VKGL-NL_Nijmegen
+/. 3 1 c.-30_64del r.(?) p.0?, p.? - pathogenic, pathogenic (recessive) g.26126692_26126785del g.25800201_25800294del 1-34_60del94 - SEPN1_000074 - - - - Germline, Unknown - - - - - Shu Yau, Tom Winder
+?/., -/. 2 1 c.-22_72del r.(?) p.0? - benign, likely pathogenic (recessive) g.26126700_26126793del g.25800209_25800302del -25_69del, 1-25_69del - SEPN1_000050 combination of variants not reported PubMed: Maiti 2009, PubMed: Villar-Quiles 2020 - - Germline - 1/132 cases - - - Johan den Dunnen
?/. 1 1 c.-14_-5dup r.(?) p.(=) - VUS g.26126708_26126717dup g.25800217_25800226dup -14_-5dup10 - SEPN1_000037 - - - - Germline - - - - - Tom Winder
?/. 2 1 c.-12C>G r.(?) p.(=) - VUS g.26126710C>G g.25800219C>G - - SEPN1_000077 - - - - Germline - - - - - Shu Yau
+/., +?/. 6 1 c.-11_81del r.(?) p.0?, p.? ACMG likely pathogenic (recessive), pathogenic, pathogenic (recessive) g.26126711_26126802del g.25800220_25800311del -11_81del92, -19_73del, 1-11_81del92 - SEPN1_000001 combination of variants not reported, linked to 1p36 PubMed: Clarke 2006, PubMed: Ferreiro 2004, PubMed: Lin 2023, Journal: Lin 2023, 1 more item - - Germline ? 11/132 cases - - - Johan den Dunnen, Shu Yau, Tom Winder, Barbara Vona
+?/. 1 1 c.-10_135del r.(?) p.0? - likely pathogenic g.26126712_26126856del g.25800221_25800365del - - SEPN1_000101 deletion of the terminal 10 nucleotides of the 5' UTR and the first 135 nucleotides of exon 1 - - - Germline - - - - - Tom Winder
-/- 2 1_13 c.= r.= p.= - benign g.? - - - SEPN1_000000 no variants 2nd chromosome - - - Germline - - - - - Johan den Dunnen
+/., +?/., -/. 21 1 c.1A>G r.(?) p.(0?), p.(Met1?), p.(Met1Val), p.0? ACMG benign, likely pathogenic, likely pathogenic (recessive), pathogenic, pathogenic (recessive) g.26126722A>G g.25800231A>G (Met1Val), M1V, Met1Val - SEPN1_000002 combination of variants not reported, mRNA level near normal, VKGL data sharing initiative Nederland PubMed: Bouman 2023, PubMed: Clarke 2006, PubMed: Ferreiro 2002, OMIM:var0003, PubMed: Allamand 2006, 2 more items - rs121908184 CLASSIFICATION record, Germline - 20/132 cases - - - Johan den Dunnen, Shu Yau, Lab Müller-Reible, Tom Winder, Wolfram Kress, VKGL-NL_Nijmegen, MobiDetails
+/. 3 1 c.2dup r.(?), r.2dup p.0? - pathogenic, pathogenic (recessive) g.26126723dup g.25800232dup 1_2insT - SEPN1_000004 mRNA level 0.6-0.7; potential secondary translation initiation PubMed: Okamoto 2006 - - Germline - 0/100 - - - Johan den Dunnen
+/. 2 1 c.2T>G r.(?) p.0? - pathogenic (recessive) g.26126723T>G g.25800232T>G (Met1Val) - SEPN1_000073 - - - - Germline - - - - - Tom Winder
+?/. 1 1 c.8_12dup r.(?) p.(Gln8Argfs*60) - likely pathogenic (recessive) g.26126729_26126733dup g.25800238_25800242dup - - SEPN1_000153 combination of variants not reported PubMed: Villar-Quiles 2020 - - Germline - 1/132 cases - - - Johan den Dunnen
+/., +?/. 3 1 c.9_33del r.(?) p.(Ala4fs*54), p.(Ala4Profs*54), p.(Arg4Profs*54) - likely pathogenic, pathogenic g.26126730_26126754del g.25800239_25800263del 9_33del24, 9_33del25 - SEPN1_000060 - - - - Germline - - - - - Shu Yau, Tom Winder
+/., +?/. 20 1 c.13_22dup r.(?) p.(Gln8fs), p.(Gln8Profs*78) ACMG likely pathogenic (recessive), pathogenic, pathogenic (recessive) g.26126734_26126743dup g.25800243_25800252dup 12_21dup10, 13_22dup10, 13_22dupCGGCCGGGCC, 22dup10, 8_17dup - SEPN1_000003 combination of variants not reported from website {DBsub-Emory}, PubMed: Bouman 2023, PubMed: Ferreiro 2002, PubMed: Allamand 2006, 3 more items - rs970951421 Germline, Unknown - 12/132 cases - - - Johan den Dunnen, Shu Yau, Lab Müller-Reible, Madhuri Hegde, Tom Winder
+/. 1 1 c.23_32dup r.(?) p.(Pro12Thrfs*75) - pathogenic (recessive) g.26126744_26126753dup g.25800253_25800262dup 23_32dup10 - SEPN1_000038 variant identical to 13_22dup10? PubMed: Clarke 2006 - - Germline - - - - - Johan den Dunnen
-/., ?/. 3 1 c.42C>T r.(?) p.(=), p.? - benign, VUS g.26126763C>T g.25800272C>T - - SEPN1_000036 VKGL data sharing initiative Nederland from website {DBsub-Emory} - - CLASSIFICATION record, Germline, Unknown - - - - - Madhuri Hegde, Tom Winder, VKGL-NL_Nijmegen
+/., +?/. 3 1 c.44_72dup r.(?) p.(Arg25Alafs*51) - likely pathogenic, likely pathogenic (recessive), pathogenic g.26126765_26126793dup g.25800274_25800302dup 44_72dup29 - SEPN1_000059 combination of variants not reported PubMed: Villar-Quiles 2020 - rs797044620 Germline - 2/132 cases - - - Johan den Dunnen, Tom Winder
+/. 1 1 c.56_83dup r.(?) p.(Arg29Alafs*63) - pathogenic g.26126777_26126804dup g.25800286_25800313dup 56_83dup28 - SEPN1_000095 - - - - Germline - - - - - Shu Yau
+/. 3 1 c.60_79dup r.60_79dup, r.? p.Arg27Leufs*62 - pathogenic, pathogenic (recessive) g.26126781_26126800dup g.25800290_25800309dup - - SEPN1_000005 mRNA level 0.3; potential secondary translation initiation PubMed: Okamoto 2006 - - Germline - 0/100 - - - Johan den Dunnen
+?/. 1 1 c.69_76dup r.(?) p.(Arg26Hisfs*43) - likely pathogenic (recessive) g.26126790_26126797dup g.25800299_25800306dup 66_73dup - SEPN1_000154 combination of variants not reported PubMed: Villar-Quiles 2020 - rs911937146 Germline - 2/132 cases - - - Johan den Dunnen
+?/. 1 1 c.77_84dup r.(?) p.(Arg29Alafs*40) - likely pathogenic (recessive) g.26126798_26126805dup g.25800307_25800314dup - - SEPN1_000155 combination of variants not reported PubMed: Villar-Quiles 2020 - - Germline - 2/132 cases - - - Johan den Dunnen
?/. 2 1 c.103G>C r.(?) p.(Gly35Arg) - VUS g.26126824G>C g.25800333G>C - - SEPN1_000093 - - - - Germline - - - - - Shu Yau
+?/. 1 1 c.125_136dup r.(?) p.(Ala42_Ala45dup) - likely pathogenic g.26126846_26126857dup g.25800355_25800366dup - - SEPN1_000113 - - - - Germline - - - - - Tom Winder
+?/. 1 1 c.142del r.(?) p.(Val48Serfs*18) - likely pathogenic (recessive) g.26126863del g.25800372del - - SEPN1_000156 combination of variants not reported PubMed: Villar-Quiles 2020 - - Germline - 1/132 cases - - - Johan den Dunnen
+?/. 1 1 c.163dup r.(?) p.(Ala55Glyfs*28) - likely pathogenic g.26126884dup g.25800393dup 163dupG - SEPN1_000100 - - - - Germline - - - - - Tom Winder
+/. 2 1 c.166C>T r.(?) p.(Gln56*) - pathogenic (recessive) g.26126887C>T g.25800396C>T - - SEPN1_000080 - - - - Germline - - - - - Shu Yau
-/. 1 - c.183+116G>A r.(=) p.(=) - benign g.26127020G>A - SELENON(NM_020451.3):c.183+116G>A - SEPN1_000186 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Utrecht
+?/. 1 - c.234del r.(?) p.? - likely pathogenic (recessive) g.26127584del g.25801093del 233delC - SEPN1_000144 no variant 2nd allele PubMed: Park 2017 - - Germline - 1/209 cases - - - Johan den Dunnen
+/., +?/. 4 2 c.249_250dup r.(?) p.(Asp84Glyfs*17), p.(Asp84GlyfsTer17) - likely pathogenic, likely pathogenic (recessive), pathogenic g.26127599_26127600dup g.25801108_25801109dup 249_250dupGG, c.249_250dupGG - SEPN1_000056 combination of variants not reported PubMed: Ganapathy 2019, PubMed: Villar-Quiles 2020 - - Germline - 1/132 cases - - - Johan den Dunnen, Tom Winder
?/. 1 2 c.253A>G r.(?) p.(Met85Val) - VUS g.26127603A>G g.25801112A>G - - SEPN1_000027 - - - - Germline - - - - - Tom Winder
+?/. 1 - c.272_273insCTGAT r.(?) p.(Glu91Aspfs*2) - likely pathogenic g.26127622_26127623insCTGAT g.25801131_25801132insCTGAT - - SEPN1_000151 combination of variants not reported PubMed: Topf 2020 - - Germline - 2/1001 cases - - - Johan den Dunnen
+?/. 1 2 c.279dup r.(?) p.(Pro94Thrfs*4) - likely pathogenic (recessive) g.26127629dup g.25801138dup 277dupA - SEPN1_000157 combination of variants not reported PubMed: Villar-Quiles 2020 - - Germline - 1/132 cases - - - Johan den Dunnen
+?/. 1 - c.301G>C r.spl? p.(?,Gly101Arg) ACMG likely pathogenic (recessive) g.26127651G>C g.25801160G>C - - SEPN1_000197 - PubMed: Bouman 2023 - - Germline - - - - - Johan den Dunnen
+?/. 1 - c.301+1G>T r.spl p.? ACMG pathogenic (recessive) g.26127652G>T g.25801161G>T - - SEPN1_000177 ACMG PVS1, PM2, PM3, PM5 PubMed: Natera-de Benito 2021 - - Germline - - - - - Johan den Dunnen
-/. 1 - c.302-200C>T r.(=) p.(=) - benign g.26128307C>T - SELENON(NM_020451.3):c.302-200C>T - SEPN1_000187 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Utrecht
-/. 1 - c.302-9_302-8del r.(=) p.(=) - benign g.26128498_26128499del g.25802007_25802008del SELENON(NM_020451.3):c.302-9_302-8delTT - SEPN1_000123 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_AMC
-/. 1 - c.302-8del r.(=) p.(=) - benign g.26128499del - SELENON(NM_020451.3):c.302-8delT - SEPN1_000188 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Utrecht
+/., +?/., ?/. 4 3_3i c.402_403+2del r.spl, r.spl? p.(del?), p.? - likely pathogenic, pathogenic, VUS g.26128607_26128610del g.25802116_25802119del 402_403+2delGAgt, SELENON(NM_020451.2):c.401_403+1delTGAG - SEPN1_000066 VKGL data sharing initiative Nederland from website {DBsub-Emory} - - CLASSIFICATION record, Germline, Unknown - - - - - Lab Müller-Reible, Madhuri Hegde, VKGL-NL_Rotterdam
+/., +?/. 4 3i c.404-1G>A r.spl, r.spl? p.? ACMG likely pathogenic, likely pathogenic (recessive), pathogenic, pathogenic (recessive) g.26131632G>A g.25805141G>A - - SEPN1_000129 ACMG PVS1, PM2, PM3, combination of variants not reported, VKGL data sharing initiative Nederland PubMed: Gonzalez-Quereda 2020, PubMed: Natera-de Benito 2021, PubMed: Villar-Quiles 2020 - - CLASSIFICATION record, Germline - 1/132 cases - - - Johan den Dunnen, VKGL-NL_Nijmegen
-/., -?/., ?/. 10 4 c.409A>G r.(?) p.(Thr137Ala), p.? - benign, likely benign, VUS g.26131638A>G g.25805147A>G SELENON(NM_020451.3):c.409A>G (p.T137A), SEPN1(NM_020451.2):c.409A>G (p.(Thr137Ala)) - SEPN1_000048 conflicting interpretations of pathogenicity; 46 heterozygous, no homozygous; Clinindb (India), 2 more items from website {DBsub-Emory}, PubMed: Narang 2020, Journal: Narang 2020 - rs35019869 CLASSIFICATION record, Germline, Unknown - 46/2794 individuals - - - Shu Yau, Madhuri Hegde, VKGL-NL_Leiden, VKGL-NL_Utrecht, VKGL-NL_AMC, Mohammed Faruq
-?/., ?/. 3 4 c.415G>A r.(?) p.(Ala139Thr), p.? - likely benign, VUS g.26131644G>A g.25805153G>A SELENON(NM_020451.3):c.415G>A (p.A139T), SEPN1(NM_020451.2):c.415G>A (p.(Ala139Thr)) - SEPN1_000028 VKGL data sharing initiative Nederland - - - CLASSIFICATION record, Germline - - - - - Tom Winder, VKGL-NL_Leiden, VKGL-NL_Utrecht
-/. 8 4 c.425G>A r.(?) p.(=), p.(Cys142Tyr), p.? - benign g.26131654G>A g.25805163G>A 425A>G (Y142C), SELENON(NM_020451.3):c.425G>A (p.C142Y), Y142C - SEPN1_000006 control chromosomes, VKGL data sharing initiative Nederland from website {DBsub-Emory}, PubMed: Allamand 2006, PubMed: Tajsharghi 2005 - rs7349185 CLASSIFICATION record, Germline, Unknown - 19/22, 279/340 - - - Johan den Dunnen, Madhuri Hegde, Andreas Laner, VKGL-NL_Groningen, VKGL-NL_Utrecht, VKGL-NL_Nijmegen, VKGL-NL_AMC
+?/. 1 4 c.425_453del r.(425_453del) p.(Cys142Ter) ACMG pathogenic (recessive) g.26131654_26131682del g.25805163_25805191del - - SEPN1_000205 ACMG: PVS1, PM2_SUP, PM3_SUP - - - Germline ? - - - - Andreas Laner
-?/., ?/. 2 4 c.436_438dup r.(?), r.? p.(Glu146dup), p.? - likely benign, VUS g.26131665_26131667dup g.25805174_25805176dup 436_438dupGAG, SELENON(NM_020451.3):c.436_438dupGAG (p.C142_E143=) - SEPN1_000103 VKGL data sharing initiative Nederland from website {DBsub-Emory} - - CLASSIFICATION record, Unknown - - - - - Madhuri Hegde, VKGL-NL_Utrecht
+?/. 1 4 c.441dup r.(?) p.(Pro148Alafs*3) - likely pathogenic (recessive) g.26131670dup g.25805179dup - - SEPN1_000158 combination of variants not reported PubMed: Villar-Quiles 2020 - - Germline - 2/132 cases - - - Johan den Dunnen
+/. 1 4 c.446dup r.(?) p.(Asp150*) - pathogenic g.26131675dup g.25805184dup 446dupC - SEPN1_000096 - - - - Germline - - - - - Shu Yau
-?/. 1 4 c.465G>A r.(?) p.(=) - likely benign g.26131694G>A g.25805203G>A - - SEPN1_000029 - - - - Germline - - - - - Tom Winder
+?/. 1 4 c.467T>C r.(?) p.(Leu156Pro) - likely pathogenic (recessive) g.26131696T>C g.25805205T>C - - SEPN1_000159 combination of variants not reported PubMed: Villar-Quiles 2020 - - Germline - 1/132 cases - - - Johan den Dunnen
+?/. 1 4 c.473T>G r.(?) p.(Ile158Thr) - likely pathogenic (recessive) g.26131702T>G g.25805211T>G - - SEPN1_000160 combination of variants not reported PubMed: Villar-Quiles 2020 - - Germline - 1/132 cases - - - Johan den Dunnen
+?/. 1 4 c.481C>T r.(?) p.(Arg161*) - likely pathogenic g.26131710C>T g.25805219C>T - - SEPN1_000055 - - - - Germline - - - - - Tom Winder
-/. 1 - c.538-145C>G r.(=) p.(=) - benign g.26134926C>G - SELENON(NM_020451.3):c.538-145C>G - SEPN1_000189 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Utrecht
?/. 2 4i c.538-48del r.(?) p.(=) - VUS g.26135023del g.25808532del 538-47delC - SEPN1_000068 - - - - Germline - - - - - Lab Müller-Reible
+?/. 1 4i c.538-2A>T r.spl p.? - likely pathogenic g.26135069A>T g.25808578A>T - - SEPN1_000112 - - - - Germline - - - - - Tom Winder
?/. 2 - c.550G>C r.(?) p.? - VUS g.26135083G>C g.25808592G>C SEPN1(NM_020451.2):c.550G>C (p.(Ala184Pro)) - SEPN1_000148 conflicting interpretations of pathogenicity; 1 heterozygous, no homozygous; Clinindb (India), 1 more item PubMed: Narang 2020, Journal: Narang 2020 - rs199742668 CLASSIFICATION record, Germline - 1/2794 individuals - - - VKGL-NL_Leiden, Mohammed Faruq
+?/. 3 5 c.565C>T r.(?) p.(Arg189*), p.? - likely pathogenic, likely pathogenic (recessive) g.26135098C>T g.25808607C>T - - SEPN1_000130 combination of variants not reported, VKGL data sharing initiative Nederland PubMed: Topf 2020, PubMed: Villar-Quiles 2020 - rs775713184 CLASSIFICATION record, Germline - 1/132 cases, 2/1001 cases - - - Johan den Dunnen, VKGL-NL_Nijmegen
+/. 2 5 c.571_572del r.(?) p.(Trp191Aspfs*28) - pathogenic (recessive) g.26135104_26135105del g.25808613_25808614del 571_572delTG - SEPN1_000071 - - - - Germline - - - - - Lab Müller-Reible
+?/-, -/., -?/. 16 5 c.583G>A r.(?) p.(Ala195Thr), p.? - benign, likely benign, likely pathogenic g.26135116G>A g.25808625G>A A195T, SELENON(NM_020451.3):c.583G>A (p.A195T), SEPN1(NM_020451.2):c.583G>A (p.(Ala195Thr)) - SEPN1_000007 51 heterozygous, no homozygous; Clinindb (India), unknown variant 2nd chromosome, 1 more item from website {DBsub-Emory}, PubMed: Narang 2020, Journal: Narang 2020, PubMed: Tajsharghi 2005 - rs115852080 CLASSIFICATION record, Germline, Unknown - 1/22, 51/2793 individuals - - - Johan den Dunnen, Shu Yau, Lab Müller-Reible, Madhuri Hegde, VKGL-NL_Leiden, VKGL-NL_Utrecht, Mohammed Faruq
+?/. 1 - c.619del r.(?) p.? - likely pathogenic (recessive) g.26135152del g.25808661del 618delC - SEPN1_000145 no variant 2nd allele PubMed: Park 2017 - - Germline - 1/209 cases - - - Johan den Dunnen
+?/. 1 5 c.665G>A r.(?) p.(Trp222*) - likely pathogenic (recessive) g.26135198G>A g.25808707G>A - - SEPN1_000161 combination of variants not reported PubMed: Villar-Quiles 2020 - - Germline - 1/132 cases - - - Johan den Dunnen
-?/. 1 - c.675C>T r.(?) p.? - likely benign g.26135208C>T - SEPN1(NM_020451.2):c.675C>T (p.(Pro225=)) - SEPN1_000180 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
+/., +?/. 2 5 c.683_689dup r.(?) p.(Met230Ilefs*73) - likely pathogenic (recessive), pathogenic g.26135216_26135222dup g.25808725_25808731dup - - SEPN1_000117 combination of variants not reported PubMed: Villar-Quiles 2020 - - Germline - 9/132 cases - - - Johan den Dunnen, Tom Winder
?/. 1 - c.712A>C r.(?) p.? - VUS g.26135245A>C - - - SEPN1_000184 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
+/., +?/., -/. 29 5 c.713dup r.(?), r.? p.(Asn238fs), p.(Asn238Lysfs*62), p.(Asn238Lysfs*63), p.(Asn238Lysfs3), p.(Asn238Lysfs4), p.? ACMG benign, likely pathogenic (recessive), pathogenic, pathogenic (recessive) g.26135246dup g.25808755dup 713-714insA, 713dupA, 713_714insA, SELENON(NM_206926.2):c.611dupA (p.N204Kfs*63) - SEPN1_000008, SEPN1_000137 combination of variants not reported, RNA fibroblast SEPN1 RNA level 20%, 2 more items PubMed: Bouman 2023, PubMed: Ferreiro 2002, OMIM:var0006, PubMed: Maiti 2009, PubMed: Schara 2008, 4 more items - rs750857935 CLASSIFICATION record, Germline - 15/132 cases - - - Johan den Dunnen, Shu Yau, Lab Müller-Reible, Tom Winder, Andreas Laner, VKGL-NL_Utrecht
?/. 1 - c.713_714dup r.(?) p.(Arg239Thrfs*19) - VUS g.26135246_26135247dup g.25808755_25808756dup - - SEPN1_000146 - PubMed: Punetha 2016 - - Germline/De novo (untested) - 1/94 cases - - - Johan den Dunnen
+/., +?/. 3 5 c.714_715insA r.(?) p.(Arg239Thrfs*62) - likely pathogenic (recessive), pathogenic g.26135247_26135248insA g.25808756_25808757insA - - SEPN1_000064 combination of variants not reported PubMed: Villar-Quiles 2020 - - Germline - 1/132 cases - - - Johan den Dunnen, Lab Müller-Reible
-?/., ?/. 2 - c.729G>A r.(?) p.? - likely benign, VUS g.26135262G>A - SELENON(NM_020451.3):c.729G>A (p.P243=), SEPN1(NM_020451.2):c.729G>A (p.(Pro243=)) - SEPN1_000183 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden, VKGL-NL_Utrecht
+/. 1 5_5i c.746_747+38del r.(?) p.? - pathogenic g.26135279_26135318del g.25808788_25808827del 746_747+38del38 - SEPN1_000075 - - - - Germline - - - - - Shu Yau
?/. 1 5i c.747+39C>T r.(?) p.(=) - VUS g.26135319C>T g.25808828C>T - - SEPN1_000104 - from website {DBsub-Emory} - - Unknown - - - - - Madhuri Hegde
-/. 1 5i c.748-40G>A r.(?) p.(=) - benign g.26135477G>A g.25808986G>A - - SEPN1_000105 - from website {DBsub-Emory} - - Unknown - - - - - Madhuri Hegde
?/. 1 - c.791T>C r.(?) p.? - VUS g.26135560T>C - SELENON(NM_020451.3):c.791T>C (p.F264S) - SEPN1_000198 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_VUmc
+?/. 1 6 c.802C>A r.(?) p.(Arg268Ser) - likely pathogenic g.26135571C>A g.25809080C>A - - SEPN1_000069 - - - - Germline - - - - - Lab Müller-Reible
+/., +?/. 6 6 c.802C>T r.(?) p.(Arg268Cys) - likely pathogenic, likely pathogenic (recessive), pathogenic g.26135571C>T g.25809080C>T - - SEPN1_000083 combination of variants not reported PubMed: Villar-Quiles 2020 - rs368074297 Germline, Unknown - 1/132 cases - - - Johan den Dunnen, Shu Yau, Tom Winder
+?/. 2 6 c.803G>A r.(?) p.(Arg268His) - likely pathogenic, likely pathogenic (recessive) g.26135572G>A g.25809081G>A - - SEPN1_000116 combination of variants not reported PubMed: Villar-Quiles 2020 - - Germline - 1/132 cases - - - Johan den Dunnen, Tom Winder
+/. 10 6 c.817G>A r.(?) p.(Gly273Arg) - pathogenic (recessive) g.26135586G>A g.25809095G>A 817G>A (G273E); conflicting - SEPN1_000009 - PubMed: D'Amico 2005, PubMed: Moghadaszadeh 2001, OMIM:var0001, PubMed: Schara 2008 - - Germline - - - - - Johan den Dunnen
+?/. 1 6 c.818G>A r.(?) p.(Gly273Glu) - likely pathogenic (recessive) g.26135587G>A g.25809096G>A - - SEPN1_000162 combination of variants not reported PubMed: Villar-Quiles 2020 - rs121908182 Germline - 8/132 cases - - - Johan den Dunnen
+?/., -?/. 8 6 c.820G>C r.(?) p.(Ala274Pro) - likely benign, likely pathogenic g.26135589G>C g.25809098G>C - - SEPN1_000081 - - - - Germline - - - - - Shu Yau
+/., +?/. 4 6 c.827_829dup r.(827_829dup), r.(?) p.(Ala276Cys277insSer), p.(Ala276_Cys277insSer) ACMG likely pathogenic, likely pathogenic (recessive), pathogenic (recessive) g.26135596_26135598dup g.25809105_25809107dup 826_827insCCT, c.827_829dupCCT - SEPN1_000136 ACMG: PM3, PM4, PS3_SUP, PM2_SUP, combination of variants not reported PMID: 30932294, 26780752, 30932294, PubMed: Ganapathy 2019, PubMed: Saini 2018, 1 more item - rs797045950 Germline yes 1/132 cases - - - Johan den Dunnen, Andreas Laner
-/. 1 6 c.846C>T r.(?) p.(=) - benign g.26135615C>T g.25809124C>T - - SEPN1_000049 - from website {DBsub-Emory} - rs34177164 Unknown - - - - - Madhuri Hegde
?/. 1 - c.847G>A r.(?) p.? - VUS g.26135616G>A - SELENON(NM_020451.3):c.847G>A (p.(Asp283Asn)) - SEPN1_000200 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
+?/. 1 6 c.861_871del r.spl? p.(Val288Aspfs*9) ACMG pathogenic (recessive) g.26135630_26135640del g.25809139_25809149del - - SEPN1_000206 ACMG: PVS1, PM2_SUP, PM3_SUP - - - Germline ? - - - - Andreas Laner
+/., +?/. 2 6 c.863_864del r.(?) p.(Val288Aspfs*12) - likely pathogenic (recessive), pathogenic (recessive) g.26135632_26135633del g.25809141_25809142del - - SEPN1_000053 combination of variants not reported PubMed: Sponholz 2006, PubMed: Schara 2008, PubMed: Villar-Quiles 2020 - - Germline - 2/132 cases - - - Johan den Dunnen
+/. 2 6 c.869_870del r.(?) p.(Phe290Serfs*10) - pathogenic g.26135638_26135639del g.25809147_25809148del 869_870delTC - SEPN1_000067 - - - - Germline - - - - - Lab Müller-Reible
+/., +?/. 3 6 c.871C>T r.(?) p.(Arg291Trp) - likely pathogenic (recessive), pathogenic g.26135640C>T g.25809149C>T - - SEPN1_000076 combination of variants not reported PubMed: Villar-Quiles 2020 - rs757446463 Germline - 1/132 cases - - - Johan den Dunnen, Shu Yau, Tom Winder
+/., +?/., ?/. 10 6 c.872G>A r.(?), r.(spl?) p.(Arg291Gln), p.? - likely pathogenic, likely pathogenic (recessive), pathogenic, VUS g.26135641G>A g.25809150G>A SEPN1(NM_020451.2):c.872G>A (p.(Arg291Gln)) - SEPN1_000039 combination of variants not reported, possible splice site effect, 1 more item PubMed: Villar-Quiles 2020 - rs199564797 CLASSIFICATION record, Germline - 5/132 cases - - - Johan den Dunnen, Shu Yau, Lab Müller-Reible, Tom Winder, VKGL-NL_Leiden, VKGL-NL_Nijmegen
-/. 2 6i c.872+100C>G r.(=), r.(?) p.(=) - benign g.26135741C>G g.25809250C>G SELENON(NM_020451.3):c.872+100C>G - SEPN1_000121 VKGL data sharing initiative Nederland - - rs2280997 CLASSIFICATION record, Germline - - - - - Andreas Laner, VKGL-NL_Utrecht
+?/. 1 6i c.873-1G>C r.spl p.? - likely pathogenic (recessive) g.26136173G>C g.25809682G>C - - SEPN1_000163 combination of variants not reported PubMed: Villar-Quiles 2020 - - Germline - 2/132 cases - - - Johan den Dunnen
+?/. 1 6i_12i c.(872+1_873-1)_(1602+1_1603-1)del r.? p.? - likely pathogenic (recessive) g.? - - - NPHS2_000000 combination of variants not reported PubMed: Villar-Quiles 2020 - - Germline - 1/132 cases - - - Johan den Dunnen
+/., +?/. 2 - c.877C>T r.(?) p.(His293Tyr) ACMG likely pathogenic (recessive) g.26136178C>T g.25809687C>T - - SEPN1_000178 ACMG PM2, PM3, PM5, PP3 PubMed: Natera-de Benito 2021 - - Germline - - - - - Johan den Dunnen
+/., +?/. 5 7 c.878A>G r.(?) p.(His293Arg) - likely pathogenic (recessive), pathogenic (recessive) g.26136179A>G g.25809688A>G - - SEPN1_000010 combination of variants not reported PubMed: Clarke 2006, PubMed: Ferreiro 2002, PubMed: Moghadaszadeh 2001, PubMed: Villar-Quiles 2020 - rs776738184 Germline - 5/132 cases - - - Johan den Dunnen
+/., +?/. 2 7 c.883G>A r.(?) p.(Glu295Lys) - likely pathogenic (recessive), pathogenic g.26136184G>A g.25809693G>A - - SEPN1_000088 combination of variants not reported PubMed: Villar-Quiles 2020 - rs978886878 Germline - 2/132 cases - - - Johan den Dunnen, Shu Yau
+?/. 1 7 c.893T>C r.(?) p.(Leu298Pro) - likely pathogenic g.26136194T>C g.25809703T>C - - SEPN1_000091 - Bachmann 2019, submitted - - Germline - - - - - Shu Yau
+/., +?/., -/. 67 7 c.943G>A r.(?), r.943c>a (.(Gly315Ser), p.(Gly315Ser), p.?, p.Gly315Ser ACMG benign, likely pathogenic, likely pathogenic (recessive), pathogenic, pathogenic (recessive) g.26136244G>A g.25809753G>A SEPN1(NM_020451.2):c.943G>A (p.(Gly315Ser)) - SEPN1_000011 combination of variants not reported, RNA fibroblast SEPN1 RNA level normal, 2 more items Ferreiro et al. AJHG 71:739-749, 2002, from website {DBsub-Emory}, PubMed: Bouman 2023, 8 more items - rs121908188 CLASSIFICATION record, Germline, Germline/De novo (untested), Unknown - 17/132 cases -HaeIII - - Johan den Dunnen, Shu Yau, Lab Müller-Reible, Madhuri Hegde, Tom Winder, Wolfram Kress, VKGL-NL_Leiden
+?/. 1 - c.943G>C r.(?) p.(Gly315Arg) ACMG likely pathogenic (recessive) g.26136244G>C g.25809753G>C - - SEPN1_000179 ACMG PM2, PM3, PM5, PP3 PubMed: Natera-de Benito 2021 - - Germline - - - - - Johan den Dunnen
+?/. 1 7 c.944G>A r.(?) p.(Gly315Asp) - likely pathogenic g.26136245G>A g.25809754G>A - - SEPN1_000092 - - - - Germline - - - - - Shu Yau
+/., +?/. 3 7 c.951del r.(?) p.(Ile317Ilefs*23), p.(Ile318SerfsTer22) ACMG pathogenic, pathogenic (recessive) g.26136252del g.25809761del 951delC - SEPN1_000175 ACMG PVS1+PM2+PP4, ACMG PVS1, PM2, PM3 PubMed: Natera-de Benito 2021 - - Germline - - - - - Johan den Dunnen, Svetlana Gorokhova
+?/. 1 7 c.952del r.(?) p.(Ile318Serfs*22) - likely pathogenic (recessive) g.26136253del g.25809762del - - SEPN1_000164 combination of variants not reported PubMed: Villar-Quiles 2020 - - Germline - 1/132 cases - - - Johan den Dunnen
+/. 1 7 c.965_967delinsCATCATCT r.(?) p.(Asp322Alafs*20) - pathogenic g.26136266_26136268delinsCATCATCT g.25809775_25809777delinsCATCATCT 965_967delACGinsCATCATCT - SEPN1_000072 - - - - Germline - - - - - Wolfram Kress
+?/. 1 7 c.976G>A r.(?) p.(Val326Ile) - likely pathogenic (recessive) g.26136277G>A g.25809786G>A - - SEPN1_000165 combination of variants not reported PubMed: Villar-Quiles 2020 - rs764032922 Germline - 1/132 cases - - - Johan den Dunnen
-/., -?/. 5 7 c.981C>T r.(?) p.(=), p.(Arg327Arg), p.? - benign, likely benign g.26136282C>T g.25809791C>T (R327R), SELENON(NM_020451.3):c.981C>T (p.R327=), SEPN1(NM_020451.2):c.981C>T (p.(Arg327=)) - SEPN1_000012 control chromosomes, VKGL data sharing initiative Nederland from website {DBsub-Emory}, PubMed: Allamand 2006 - - CLASSIFICATION record, Germline, Unknown - 10/340 - - - Johan den Dunnen, Madhuri Hegde, Tom Winder, VKGL-NL_Leiden, VKGL-NL_AMC
+/. 1 7 c.988del r.(?) p.(Arg330Glyfs*10) - pathogenic g.26136289del g.25809798del 988delC - SEPN1_000047 - - - - Germline - - - - - Varvara Kadnikova
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