Unique variants in the SMC1A gene

Information The variants shown are described using the NM_006306.2 transcript reference sequence.

139 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.-231639_109+108dup r.0 p.0 - pathogenic g.53449333_53681188dup - - - HSD17B10_000011 increased gene dosage PubMed: DDDS 2015, Journal: DDDS 2015 - - Germline - - - - - Johan den Dunnen
-/., -?/., ?/? 4 1 c.-19C>T r.(=), r.(?) p.(=) - benign, likely benign, VUS g.53449568G>A g.53422619G>A SMC1A(NM_006306.4):c.-19C>T - SMC1A_000012, SMC1A_000043 VKGL data sharing initiative Nederland - - rs1264011 CLASSIFICATION record, Germline - 0.14-0.64 - - - Johan den Dunnen, Yu Sun, VKGL-NL_Groningen
+?/+? 1 1 c.31A>T r.(?) p.(Asn11Tyr) - likely pathogenic g.53449519T>A g.53422570T>A - - SMC1A_000030 not in >200 control chromosomes Redeker (unpublished) - - De novo - - - - - Bert Redeker
?/? 1 1 c.82A>C r.(?) p.Thr28Pro - VUS g.53449468T>G g.53422519T>G - - SMC1A_000013 - - - rs34530151 Germline - 0.00-0.10 - - - Johan den Dunnen
+?/. 1 - c.92T>C r.(?) p.(Ile31Thr) - likely pathogenic g.53449458A>G g.53422509A>G - - RIBC1_000009 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
-/., -?/. 3 1i c.109+104dup r.(=) p.(=) - benign, likely benign g.53449338dup g.53422386dup SMC1A(NM_006306.3):c.109+103dupC - RIBC1_000008, SMC1A_000044 VKGL data sharing initiative Nederland - - - CLASSIFICATION record, Germline - - - - - Yu Sun, VKGL-NL_Utrecht
-?/. 1 - c.109+575A>C r.(=) p.(=) - likely benign g.53448866T>G - SMC1A(NM_001281463.1):c.22A>C (p.T8P) - RIBC1_000007 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-?/. 1 - c.109+582A>C r.(=) p.(=) - likely benign g.53448859T>G - SMC1A(NM_001281463.1):c.29A>C (p.(His10Pro)) - RIBC1_000006 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
+?/. 1 2 c.110G>T r.(?) p.(Gly37Val) - likely pathogenic (dominant) g.53442118C>A g.53415169C>A G110T - SMC1A_000131 - PubMed: Yuan 2019 - - De novo - - - - - Johan den Dunnen
+/. 1 2 c.116C>G r.(?) p.(Ser39*) - pathogenic (dominant) g.53442112G>C g.53415163G>C - - SMC1A_000130 - PubMed: Yuan 2019 - - De novo - - - - - Johan den Dunnen
+?/. 2 2 c.121C>T r.(?) p.(Leu41Phe) - likely pathogenic g.53442107G>A g.53415158G>A C121T - SMC1A_000120 - PubMed: Yuan 2019 - - Germline - - - - - Johan den Dunnen
+?/. 1 2 c.128A>T r.(?) p.(Asp43Val) - likely pathogenic (dominant) g.53442100T>A g.53415151T>A - - SMC1A_000129 - PubMed: Yuan 2019 - - De novo - - - - - Johan den Dunnen
+?/. 1 2 c.140T>G r.(?) p.(Phe47Cys) - likely pathogenic (dominant) g.53442088A>C g.53415139A>C - - SMC1A_000128 - PubMed: Yuan 2019 - - De novo - - - - - Johan den Dunnen
+/+, +/? 2 2 c.173_187del r.(?) p.(Val58_Arg62del) - pathogenic g.53442049_53442063del g.53415100_53415114del 173del15 - SMC1A_000005 not in 220 control chromosomes PubMed: Deardorff 2007, OMIM:var0003, PubMed: Gervasini 2013 - - De novo - - - - - Johan den Dunnen, Bert Redeker
+?/. 1 2 c.298G>C r.(?) p.(Gly100Arg) - likely pathogenic (dominant) g.53441930C>G g.53414981C>G - - SMC1A_000127 - PubMed: Yuan 2019 - - De novo - - - - - Johan den Dunnen
-?/. 2 - c.372G>C r.(?) p.(Leu124Phe) - likely benign g.53441746C>G - SMC1A(NM_001281463.1):c.306G>C (p.L102F), SMC1A(NM_006306.4):c.372G>C (p.L124F) - SMC1A_000106 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam, VKGL-NL_Groningen
+/? 1 3 c.397T>G r.(?) p.(Phe133Val) - pathogenic g.53441721A>C g.53414772A>C - - SMC1A_000006 not in 220 control chromosomes PubMed: Deardorff 2007 - - De novo - - - - - Johan den Dunnen
-?/. 1 - c.412-10C>T r.(=) p.(=) - likely benign g.53440395G>A g.53413445G>A SMC1A(NM_006306.3):c.412-10C>T - SMC1A_000075 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Utrecht
+/+ 1 3 c.421G>A r.(?) p.(Glu141Lys) - pathogenic g.53440376C>T g.53413426C>T - - SMC1A_000020 not in >220 control chromosomes PubMed: Liu 2009 - - De novo - - - - - Bert Redeker
?/. 1 - c.494C>T r.(?) p.(Ala165Val) - VUS g.53440303G>A - SMC1A(NM_001281463.1):c.428C>T (p.A143V) - SMC1A_000096 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_VUmc
+?/. 1 - c.557A>G r.(?) p.(Tyr186Cys) - likely pathogenic g.53440240T>C g.53413290T>C - - SMC1A_000092 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
+?/. 1 - c.563G>A r.(?) p.(Arg188His) - likely pathogenic g.53440234C>T g.53413284C>T - - SMC1A_000080 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
+?/. 2 4 c.586C>T r.(?) p.(Arg196Cys) ACMG likely pathogenic (dominant), pathogenic (dominant) g.53440211G>A g.53413261G>A - - SMC1A_000126 ACMG: PS2_MOD, PS4_MOD, PM5, PP3_MOD, PM2_SUP, PP2 PMID: 30158690, PubMed: Yuan 2019 VCV000159961.6 - De novo, Germline ? - - - - Johan den Dunnen, Andreas Laner
+/+, +/+?, +/? 3 4 c.587G>A r.(?) p.(Arg196His) - pathogenic g.53440210C>T g.53413260C>T 587G>A - SMC1A_000007, SMC1A_000016 not in 120 control chromosomes, not in 220 control chromosomes PubMed: Borck 2007, PubMed: Deardorff 2007, PubMed: Pié 2010 - - De novo - - - - - Johan den Dunnen, Bert Redeker
?/. 1 - c.598A>C r.(?) p.(Lys200Gln) - VUS g.53440199T>G g.53413249T>G SMC1A(NM_001281463.1):c.532A>C (p.K178Q) - SMC1A_000095 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+?/. 1 - c.607A>G r.(?) p.(Lys203Glu) ACMG likely pathogenic g.53440190T>C g.53413240T>C - - SMC1A_000133 ACMG PS2,PM2,PM6,PP3 PubMed: Chuan 2022 - - Germline/De novo (untested) - - - - - Johan den Dunnen
+/. 1 5 c.655del r.(?) p.(Ala219Leufs*45) - pathogenic (dominant) g.53440051del g.53413101del - - SMC1A_000125 - PubMed: Yuan 2019 - - De novo - - - - - Johan den Dunnen
-?/. 1 - c.699G>A r.(?) p.(=) - likely benign g.53440005C>T - SMC1A(NM_001281463.1):c.633G>A (p.V211=) - SMC1A_000140 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-?/., ?/. 2 - c.707A>G r.(?) p.(Glu236Gly) - likely benign, VUS g.53439997T>C g.53413047T>C SMC1A(NM_001281463.1):c.641A>G (p.(Glu214Gly)) - SMC1A_000074 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden, VKGL-NL_Nijmegen
+?/. 1 - c.725T>C r.(?) p.(Leu242Pro) - likely pathogenic g.53439979A>G - - - SMC1A_000134 - - - - CLASSIFICATION record - - - - - MobiDetails
?/. 1 - c.751_753del r.(?) p.(Lys251del) - VUS g.53439953_53439955del g.53413003_53413005del SMC1A(NM_001281463.1):c.685_687delAAG (p.K229del) - SMC1A_000073 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+?/. 1 - c.784G>T r.(?) p.(Glu262*) - pathogenic g.53439920C>A g.53412970C>A - - SMC1A_000093 - PubMed: Minardi 2020 - - De novo - - - - - Francesca Bisulli
+/+, +/+?, +/., +?/. 4 5 c.802_804del r.(?) p.(Lys268del) - likely pathogenic, pathogenic, pathogenic (dominant) g.53439900_53439902del, g.53439908_53439910del g.53412950_53412952del, g.53412958_53412960del 802_804del3, 802_804delAAG - SMC1A_000021 not in 100 control alleles, not in >220 control chromosomes PubMed: Liu 2009, PubMed: Pié 2010, PubMed: Yuan 2019 - - De novo - - - - - Johan den Dunnen, Bert Redeker
-?/. 2 - c.855-15C>T r.(=) p.(=) - likely benign g.53439218G>A g.53412268G>A SMC1A(NM_001281463.1):c.789-15C>T, SMC1A(NM_006306.3):c.855-15C>T - SMC1A_000072 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Groningen, VKGL-NL_Utrecht
+/+ 1 5 c.916_918del r.(?) p.(Ser306del) - pathogenic g.53439142_53439144del g.53412192_53412194del 916_918del3 - SMC1A_000022 not in >220 control chromosomes PubMed: Liu 2009 - - De novo - - - - - Bert Redeker
+/. 1 - c.1113+1G>A r.spl? p.? - pathogenic g.53438944C>T g.53411994C>T - - SMC1A_000078 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
+/. 1 7i c.1114-2A>G r.spl p.? - pathogenic (dominant) g.53438853T>C g.53411903T>C - - SMC1A_000124 - PubMed: Yuan 2019 - - Germline - - - - - Johan den Dunnen
-?/. 1 - c.1119G>A r.(?) p.(Lys373=) - likely benign g.53438846C>T - SMC1A(NM_001281463.1):c.1053G>A (p.K351=) - SMC1A_000102 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+?/. 1 - c.1141G>A r.(?) p.(Glu381Lys) - likely pathogenic g.53438824C>T - SMC1A(NM_001281463.1):c.1075G>A (p.E359K) - SMC1A_000110 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_VUmc
+?/+? 1 7 c.1169C>A r.(?) p.(Ala390Asp) - likely pathogenic g.53438796G>T g.53411846G>T - - SMC1A_000029 inherited from mildly affected mother Redeker (unpublished) - - Germline - - - - - Bert Redeker
-?/. 1 - c.1173G>A r.(?) p.(Gln391=) - likely benign g.53438792C>T - SMC1A(NM_001281463.1):c.1107G>A (p.Q369=) - SMC1A_000109 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+/+ 1 7 c.1192C>G r.(?) p.(Arg398Gly) - pathogenic g.53438773G>C g.53411823G>C - - SMC1A_000035 - PubMed: Gervasini 2013 - - De novo - - - - - Bert Redeker
+/+ 1 7 c.1193G>A r.(?) p.(Arg398Gln) - pathogenic g.53438772C>T g.53411822C>T - - SMC1A_000023 not in >220 control chromosomes PubMed: Liu 2009 - - De novo - - - - - Bert Redeker
?/. 1 - c.1217G>A r.(?) p.(Arg406His) - VUS g.53438748C>T g.53411798C>T SMC1A(NM_006306.3):c.1217G>A (p.(Arg406His)) - SMC1A_000071 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
?/. 1 7 c.1235G>A r.(?) p.(Arg412Gln) ACMG VUS g.53438730C>T g.53411780C>T - - SMC1A_000104 - PubMed: Squeo 2020 - - Germline/De novo (untested) - - - - - Johan den Dunnen
-?/. 2 - c.1254+4G>A r.spl? p.? - likely benign g.53438707C>T g.53411757C>T SMC1A(NM_001281463.1):c.1188+4G>A, SMC1A(NM_006306.3):c.1254+4G>A - SMC1A_000070 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam, VKGL-NL_Utrecht
-?/. 1 - c.1254+11G>C r.(=) p.(=) - likely benign g.53438700C>G g.53411750C>G SMC1A(NM_006306.3):c.1254+11G>C - SMC1A_000069 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Utrecht
-/. 1 - c.1254+62C>A r.(=) p.(=) - benign g.53438649G>T g.53411699G>T SMC1A(NM_006306.3):c.1254+62C>A - SMC1A_000068 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Utrecht
-?/. 1 - c.1284T>G r.(?) p.(Ile428Met) - likely benign g.53436405A>C g.53409474A>C SMC1A(NM_001281463.1):c.1218T>G (p.I406M) - SMC1A_000089 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-?/. 1 - c.1312C>T r.(?) p.(=) - likely benign g.53436377G>A - SMC1A(NM_006306.4):c.1312C>T (p.(Leu438=)) - SMC1A_000150 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
-?/. 1 - c.1323C>T r.(?) p.(Tyr441=) - likely benign g.53436366G>A g.53409435G>A SMC1A(NM_006306.4):c.1323C>T (p.Y441=) - SMC1A_000067 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Groningen
?/. 2 8i c.1338-32C>A r.(=), r.(spl?) p.(=) - VUS g.53436232G>T g.53409301= - - SMC1A_000042 - - - - Germline - - - - - Yu Sun
?/. 1 - c.1361A>G r.(?) p.(Lys454Arg) - VUS g.53436177T>C - SMC1A(NM_006306.3):c.1361A>G (p.(Lys454Arg)) - SMC1A_000137 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
+/? 2 9 c.1478A>C r.(?) p.(Glu493Ala), p.Glu493Ala - NA, pathogenic g.53436060T>G g.53409129T>G - - SMC1A_000003 increased affinity of SMC hinge dimers for DNA binding, 1 more item PubMed: Musio 2006, OMIM:var0002, PubMed: Revenkova 2009, OMIM:var0002 - - De novo, In vitro (cloned) - - - - - Johan den Dunnen
+/? 1 9 c.1486C>T r.(?) p.(Arg496Cys) - pathogenic g.53436052G>A g.53409121G>A - - SMC1A_000008 not in 220 control chromosomes PubMed: Deardorff 2007 - - Germline - - - - - Johan den Dunnen
+/., +/? 8 9 c.1487G>A r.(?) p.(Arg496His), p.Arg496His - NA, pathogenic, pathogenic (dominant) g.53436051C>T g.53409120C>T - - SMC1A_000004 increased affinity of SMC hinge dimers for DNA binding, not in 220 control chromosomes, 2 more items PubMed: Deardorff 2007, PubMed: Deardorff 2007, OMIM:var0004, PubMed: Revenkova 2009, OMIM:var0004, 1 more item - - De novo, Germline, In vitro (cloned), Unknown - - - - - Johan den Dunnen, Jorge Oliveira
-?/. 3 - c.1545+4A>C r.spl? p.? - likely benign g.53435989T>G g.53409058T>G 1 more item - SMC1A_000066 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam, VKGL-NL_Groningen, VKGL-NL_Utrecht
-?/. 1 - c.1548C>T r.(?) p.(Tyr516=) - likely benign g.53432886G>A g.53405954G>A SMC1A(NM_001281463.1):c.1482C>T (p.(=)) - SMC1A_000098 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
+/. 1 - c.1576A>C r.(?) p.(Thr526Pro) - pathogenic g.53432858T>G - SMC1A(NM_006306.3):c.1576A>C (p.T526P) - SMC1A_000135 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_VUmc
?/. 1 - c.1678A>G r.(?) p.(Ile560Val) - VUS g.53432756T>C - SMC1A(NM_006306.4):c.1678A>G (p.I560V) - SMC1A_000116 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Groningen
-/. 1 - c.1698G>A r.(?) p.(Glu566=) - benign g.53432736C>T g.53405804C>T SMC1A(NM_001281463.1):c.1632G>A (p.E544=) - SMC1A_000088 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-?/. 1 - c.1767G>A r.(?) p.(Lys589=) - likely benign g.53432569C>T g.53405637C>T SMC1A(NM_001281463.1):c.1701G>A (p.K567=) - SMC1A_000097 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-?/. 1 - c.1788T>C r.(?) p.(Asp596=) - likely benign g.53432548A>G g.53405616A>G SMC1A(NM_006306.3):c.1788T>C (p.D596=) - SMC1A_000087 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Utrecht
-?/. 1 - c.1863C>T r.(?) p.(Asn621=) - likely benign g.53432473G>A - SMC1A(NM_001281463.1):c.1797C>T (p.N599=) - SMC1A_000103 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Utrecht
?/. 1 - c.1904G>A r.(?) p.(Arg635His) - VUS g.53432432C>T g.53405500C>T SMC1A(NM_006306.2):c.1904G>A (p.(Arg635His)) - SMC1A_000065 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
-?/. 1 - c.1912-14C>T r.(=) p.(=) - likely benign g.53432337G>A g.53405405G>A SMC1A(NM_006306.3):c.1912-14C>T - SMC1A_000086 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Utrecht
+?/+? 1 12 c.1937T>C r.(?) p.(Phe646Ser) - likely pathogenic g.53432298A>G g.53405366A>G - - SMC1A_000033 mother skewed X-inactivation (96:4) PubMed: Tzschach 2015 - - Germline - - - - - Andreas Tzschach
+/+ 1 12 c.1951G>A r.(?) p.(Val651Met) - pathogenic g.53432284C>T g.53405352C>T - - SMC1A_000034 - PubMed: Gervasini 2013 - - De novo - - - - - Bert Redeker
+?/+? 1 12 c.1955T>A r.(?) p.(Ile652Asn) - likely pathogenic g.53432280A>T g.53405348A>T - - SMC1A_000041 - Redeker (unpublished) - - Unknown - - - - - Bert Redeker
+?/. 1 - c.1968_1970del r.(?) p.(Ser657del) - likely pathogenic g.53432265_53432267del - - - SMC1A_000138 - - - - CLASSIFICATION record - - - - - MobiDetails
+/+ 1 12 c.2046_2048del r.(?) p.(Glu684del) - pathogenic g.53432187_53432189del g.53405255_53405257del 2046_2048delAGA - SMC1A_000024 not in >220 control chromosomes PubMed: Liu 2009 - - De novo - - - - - Bert Redeker
+/+ 1 12 c.2077C>G r.(?) p.(Arg693Gly) - pathogenic g.53432063G>C g.53405131G>C - - SMC1A_000025 not in >220 control chromosomes PubMed: Liu 2009 - - De novo - - - - - Bert Redeker
+/+ 1 13 c.2078G>A r.(?) p.(Arg693Gln) - pathogenic g.53432062C>T g.53405130C>T - - SMC1A_000036 - PubMed: Gervasini 2013 - - De novo - - - - - Bert Redeker
+?/+?, +?/., ./. 3 13 c.2095C>T r.(?) p.(Arg699Cys) - likely pathogenic, likely pathogenic (dominant), pathogenic g.53432045G>A g.53405113G>A - - SMC1A_000018, SMC1A_000052 - Redeker (unpublished), PubMed: DDDS 2015, Journal: DDDS 2015 - - De novo - - - - - Johan den Dunnen, Bert Redeker, Alejandro Brea-Fernández
?/. 1 - c.2099A>C r.(?) p.(Gln700Pro) - VUS g.53432041T>G g.53405109T>G - - SMC1A_000081 - - - - Unknown - - - - - IMGAG
+/. 1 - c.2122del r.(?) p.(Leu708CysfsTer12) - pathogenic g.53432018del - - - SMC1A_000111 - - - - CLASSIFICATION record - - - - - MobiDetails
+/? 1 13 c.2131C>T r.(?) p.(Arg711Trp) - pathogenic g.53432009G>A g.53405077G>A - - SMC1A_000009 not in 220 control chromosomes PubMed: Deardorff 2007 - - De novo - - - - - Johan den Dunnen
+/+? 1 13 c.2132G>A r.(?) p.(Arg711Gln) - pathogenic g.53432008C>T g.53405076C>T 2132G>A - SMC1A_000031 not in 100 control alleles PubMed: Pié 2010 - - De novo - - - - - Bert Redeker
+?/. 1 13 c.2161C>T r.(?) p.(Gln721*) - likely pathogenic g.53431979G>A g.53405047G>A C2161T - SMC1A_000119 - PubMed: Yuan 2019 - - De novo - - - - - Johan den Dunnen
-/., -?/. 2 - c.2197-5T>C r.spl? p.? - benign, likely benign g.53430830A>G g.53403898A>G SMC1A(NM_001281463.1):c.2131-5T>C (p.?), SMC1A(NM_006306.3):c.2197-5T>C - SMC1A_000064 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden, VKGL-NL_Utrecht
+/+ 1 14 c.2342G>T r.(?) p.(Cys781Phe) - pathogenic g.53430576C>A g.53403644C>A - - SMC1A_000026 not in >220 control chromosomes PubMed: Liu 2009 - - De novo - - - - - Bert Redeker
+/+, +/+?, +/. 3 15 c.2351T>C r.(?), r.2351u>c p.(Ile784Thr), p.Ile784Thr - pathogenic g.53430567A>G g.53403635A>G - - SMC1A_000032, SMC1A_000037 RNA expression both allele PubMed: Fieremans 2016, PubMed: Gervasini 2013, PubMed: Limongelli 2010 - - De novo - - - - skewed X-inactivation 0:1, skewed X-inactivation (93:7 pat:mat) Johan den Dunnen, Bert Redeker
+/. 1 14 c.2364del r.(?) p.(Asn788Lysfs*10) - pathogenic g.53430554del g.53403622del - - SMC1A_000050 - PubMed: Gilissen 2014 - - De novo - - - - - Marianne Vos (LOVD-team)
+?/. 1 - c.2368C>T r.(?) p.(Arg790Trp) - likely pathogenic g.53430550G>A - SMC1A(NM_001281463.1):c.2302C>T (p.R768W) - SMC1A_000139 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+/+, +/? 2 15 c.2369G>A r.(?) p.(Arg790Gln) - pathogenic g.53430549C>T g.53403617C>T - - SMC1A_000010, SMC1A_000038 not in 220 control chromosomes PubMed: Deardorff 2007, PubMed: Gervasini 2013 - - De novo - - - - - Johan den Dunnen, Bert Redeker
?/. 1 - c.2389G>A r.(?) p.(Val797Met) - VUS g.53430529C>T - SMC1A(NM_001281463.1):c.2323G>A (p.V775M) - SMC1A_000101 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Groningen
+?/. 1 15 c.2394del r.(?) p.(Lys798AsnfsTer31) - likely pathogenic g.53430526del g.53403594del c.2394delA - SMC1A_000108 - PubMed: Ganapathy 2019 - - Germline - - - - - Johan den Dunnen
+/. 1 15 c.2394dup r.(?) p.(Arg799Thrfs*4) - pathogenic (dominant) g.53430526dup g.53403594dup - - SMC1A_000123 - PubMed: Yuan 2019 - - De novo - - - - - Johan den Dunnen
+?/. 2 15 c.2420G>A r.(?) p.(Arg807His) - likely pathogenic, likely pathogenic (dominant) g.53430498C>T g.53403566C>T - - SMC1A_000105 - PubMed: Yuan 2019 - - De novo, Unknown - - - - - Johan den Dunnen, IMGAG
+/. 1 15i_16i c.2421-352_2563-1354del r.? p.? - pathogenic g.53424895_53427008del g.53397971_53400084del g.53424894_53427008del - SMC1A_000051 - PubMed: Gilissen 2014 - - De novo - - - - - Johan den Dunnen
+/+ 2 15, 16 c.2446C>G r.(?) p.(Arg816Gly) - pathogenic g.53426627G>C g.53399705G>C - - SMC1A_000027 not in >220 control chromosomes Redeker (unpublished), PubMed: Liu 2009 - - De novo, Unknown - - - - - Bert Redeker
+/? 1 16 c.2493_2495del r.(?) p.(Asp831_Gln832delinsGlu) - pathogenic (recessive) g.53426579_53426581del g.53399657_53399659del 2493_2495del - SMC1A_000002 not in >600 control chromosomes: SMC1A protein detectable PubMed: Musio 2006, OMIM:var0001 - - Germline - - - - - Johan den Dunnen
?/. 1 - c.2524G>T r.(?) p.(Val842Leu) - VUS g.53426549C>A g.53399627C>A SMC1A(NM_001281463.1):c.2458G>T (p.V820L) - SMC1A_000063 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+/. 1 16 c.2547del r.(?) p.(Ile849Metfs*12) - pathogenic (dominant) g.53426526del g.53399604del - - SMC1A_000122 - PubMed: Yuan 2019 - - De novo - - - - - Johan den Dunnen
?/. 1 - c.2588T>A r.(?) p.(Ile863Lys) - VUS g.53423512A>T g.53396592A>T SMC1A(NM_006306.3):c.2588T>A (p.I863K) - SMC1A_000085 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Utrecht
+/. 1 - c.2611C>T r.(?) p.(Gln871*) ACMG pathogenic g.53423489G>A - C2611T (Q871X) - SMC1A_000114 - PubMed: Halvardson 2016 - - De novo - - - - - Johan den Dunnen
+/., +?/. 2 - c.2683C>G r.(?) p.(Arg895Gly) - likely pathogenic, pathogenic g.53423417G>C g.53396497G>C SMC1A(NM_006306.4):c.2683C>G (p.R895G) - SMC1A_000077 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Groningen, VKGL-NL_Nijmegen
-?/. 1 - c.2691A>G r.(?) p.(Lys897=) - likely benign g.53423409T>C g.53396489T>C SMC1A(NM_001281463.1):c.2625A>G (p.K875=) - SMC1A_000062 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
?/. 1 - c.2694C>T r.(?) p.(=) - VUS g.53423406G>A - SMC1A(NM_006306.4):c.2694C>T (p.(Leu898=)) - SMC1A_000149 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
-?/. 1 - c.2708A>G r.(?) p.(Lys903Arg) - likely benign g.53423392T>C g.53396472T>C SMC1A(NM_001281463.1):c.2642A>G (p.K881R) - SMC1A_000061 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
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