All variants in the C1S gene

Ehlers Danlos Syndrome Variant Database


Information The variants shown are described using the NM_001734.3 transcript reference sequence.

55 entries on 1 page. Showing entries 1 - 55.
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Effect     

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     
-?/. - c.100A>G r.(?) p.(Ser34Gly) - likely benign g.7169873A>G g.7062569A>G C1S(NM_201442.3):c.100A>G (p.S34G) - C1S_000001 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-?/. - c.214-16G>T r.(=) p.(=) - likely benign g.7170178G>T g.7062874G>T C1S(NM_201442.4):c.214-16G>T - C1S_000006 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_VUmc
-?/. - c.306A>G r.(?) p.(Pro102=) - likely benign g.7170286A>G g.7062982A>G C1S(NM_001734.3):c.306A>G (p.P102=), C1S(NM_001734.5):c.306A>G (p.P102=), C1S(NM_201442.3):c.306A>G (p.P102=) - C1S_000007 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-?/. - c.306A>G r.(?) p.(Pro102=) - likely benign g.7170286A>G g.7062982A>G C1S(NM_001734.3):c.306A>G (p.P102=), C1S(NM_001734.5):c.306A>G (p.P102=), C1S(NM_201442.3):c.306A>G (p.P102=) - C1S_000007 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Utrecht
-?/. - c.306A>G r.(?) p.(Pro102=) - likely benign g.7170286A>G - C1S(NM_001734.3):c.306A>G (p.P102=), C1S(NM_001734.5):c.306A>G (p.P102=), C1S(NM_201442.3):c.306A>G (p.P102=) - C1S_000007 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_VUmc
+/. 4 c.330del r.(?) p.(Phe110Leufs*23) - pathogenic (recessive) g.7170310del g.7063006del - - C1S_000025 Probands presenting with a strongly decreased complement haemolytic activity and a decreased antigenic C1q. Heterozygous parents with a half complement haemolytic activity. Journal: Alshekaili 2023 - - Germline yes - - - - Christian Drouet
-?/. - c.356G>A r.(?) p.(Arg119His) - likely benign g.7170336G>A g.7063032G>A C1S(NM_201442.4):c.356G>A (p.R119H) - C1S_000008 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
-/. - c.356G>A r.(?) p.(Arg119His) - benign g.7170336G>A g.7063032G>A C1S(NM_201442.4):c.356G>A (p.R119H) - C1S_000008 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_VUmc
?/. - c.440G>A r.(?) p.(Cys147Tyr) - VUS g.7171619G>A - C1S(NM_201442.4):c.440G>A (p.C147Y) - C1S_000021 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Groningen
-/. - c.441C>T r.(?) p.(Cys147=) - benign g.7171620C>T g.7064316C>T C1S(NM_201442.4):c.441C>T (p.C147=) - C1S_000009 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_VUmc
-/. - c.518-13_518-6del r.(=) p.(=) - benign g.7172391_7172398del - C1S(NM_001734.5):c.518-13_518-6del, C1S(NM_001734.5):c.518-13_518-6delTTTTCTCT - C1S_000033 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_VUmc
?/. - c.518-13_518-6del r.(=) p.(=) - VUS g.7172391_7172398del - C1S(NM_001734.5):c.518-13_518-6del, C1S(NM_001734.5):c.518-13_518-6delTTTTCTCT - C1S_000033 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
+/. 6 c.612C>G r.(?) p.(Tyr204*) - pathogenic (recessive) g.7172498C>G g.7065194C>G - - C1S_000029 Proband and her 3 brothers presenting with an undetectable antigenic C1s in serum, a lack of haemolytic complement and partial C1r deficiency (27–36% of normal). Absence of C1s synthesis by fibroblasts. Heterozygous parents have moderately decreased antigenic C1s (73–75%) and C1r (70–72%). Journal: Amano et al., 2008 - - Germline yes - - - - Christian Drouet
+/+ 6 c.612C>G r.(?) p.(Tyr204*) - pathogenic (recessive) g.7172498C>G g.7065196C>G - - C1S_000029 heterozygous parents have moderately decreased antigenic C1s (73–75%) and C1r (70–72%). PubMed: Amano et al., 2008 Journal: Amano et al., 2008 - - Germline yes - - - - Christian Drouet
-?/. - c.656G>A r.(?) p.(Arg219Gln) - likely benign g.7172542G>A - C1S(NM_201442.3):c.656G>A (p.R219Q) - C1S_000018 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
?/. - c.717+6T>A r.(=) p.(=) - VUS g.7172609T>A - C1S(NM_001734.5):c.717+6T>A - C1S_000037 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
?/. - c.733C>T r.(?) p.(Arg245Trp) - VUS g.7173136C>T - C1S(NM_201442.4):c.733C>T (p.R245W) - C1S_000034 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Groningen
?/. - c.804_805dup r.(?) p.(Asp269Valfs*9) - VUS g.7173207_7173208dup - C1S(NM_001734.3):c.804_805dupTG (p.(Asp269fs)) - C1S_000035 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
+/+? 8 c.880T>C r.(?) p.(Cys294Arg) - pathogenic g.7173830T>C g.7066526T>C - - C1S_000026 p.(Cys294Arg) variant affects Sushi CCP1 domain. C1S p.(Cys294Arg) variant exposes a normally inaccessible cleavage site that escapes normal control by C1-INH and triggers abnormal C1s activation, with subsequent collagen degradation. Described in ClinVar as pathogenic by Division of Human Genetics, Medical University Innsbruck Austria Journal: Kapferer-Seebacher 2016 Journal: Bally 2019 ClinVar-SCV000494614 rs886040975 Germline yes - - - - Christian Drouet
-?/. - c.920C>T r.(?) p.(Ala307Val) - likely benign g.7173870C>T - C1S(NM_001734.3):c.920C>T (p.A307V) - C1S_000016 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Utrecht
?/. - c.943G>A r.(?) p.(Asp315Asn) - VUS g.7173893G>A g.7066589G>A C1S(NM_001734.3):c.943G>A (p.(Asp315Asn), p.D315N), C1S(NM_001734.5):c.943G>A (p.D315N), C1S(NM_201442.3):c.943G>A (p.D315N), C1S(NM_201442.4):c.94... - C1S_000005 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
-?/. - c.943G>A r.(?) p.(Asp315Asn) - likely benign g.7173893G>A g.7066589G>A C1S(NM_001734.3):c.943G>A (p.(Asp315Asn), p.D315N), C1S(NM_001734.5):c.943G>A (p.D315N), C1S(NM_201442.3):c.943G>A (p.D315N), C1S(NM_201442.4):c.94... - C1S_000005 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-?/. - c.943G>A r.(?) p.(Asp315Asn) - likely benign g.7173893G>A g.7066589G>A C1S(NM_001734.3):c.943G>A (p.(Asp315Asn), p.D315N), C1S(NM_001734.5):c.943G>A (p.D315N), C1S(NM_201442.3):c.943G>A (p.D315N), C1S(NM_201442.4):c.94... - C1S_000005 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Utrecht
?/. - c.943G>A r.(?) p.(Asp315Asn) - VUS g.7173893G>A - C1S(NM_001734.3):c.943G>A (p.(Asp315Asn), p.D315N), C1S(NM_001734.5):c.943G>A (p.D315N), C1S(NM_201442.3):c.943G>A (p.D315N), C1S(NM_201442.4):c.94... - C1S_000005 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Groningen
?/. - c.943G>A r.(?) p.(Asp315Asn) - VUS g.7173893G>A - C1S(NM_001734.3):c.943G>A (p.(Asp315Asn), p.D315N), C1S(NM_001734.5):c.943G>A (p.D315N), C1S(NM_201442.3):c.943G>A (p.D315N), C1S(NM_201442.4):c.94... - C1S_000005 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_VUmc
-?/. - c.943G>A r.(?) p.(Asp315Asn) - likely benign g.7173893G>A - C1S(NM_001734.3):c.943G>A (p.(Asp315Asn), p.D315N), C1S(NM_001734.5):c.943G>A (p.D315N), C1S(NM_201442.3):c.943G>A (p.D315N), C1S(NM_201442.4):c.94... - C1S_000005 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
+/+ 8 c.945_947del r.(?) p.(Asp315_Val316delinsGlu) - pathogenic g.7173895_7173897del g.7066591_7066593del - - C1S_000027 p.(Asp315_Val316delinsGlu) affects the Sushi CCP1 domain (near CUB2). C1S p.(Asp315_Val316delinsGlu) variant exposes a normally inaccessible cleavage site that escapes normal control by C1-INH and triggers abnormal C1s activation, with subsequent collagen degradation. Identified in ClinVar as pathogenic by Division of Human Genetics, Medical University Innsbruck Austria PubMed: Kapferer-Seebacher 2016 Journal: Kapferer-Seebacher 2016 Journal: Bally 2019] ClinVar-SCV000494615 rs886040974 Germline yes - - - - Christian Drouet
-?/. - c.963T>C r.(?) p.(Cys321=) - likely benign g.7173913T>C - C1S(NM_201442.3):c.963T>C (p.C321=) - C1S_000022 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
?/. - c.971G>A r.(?) p.(Gly324Glu) - VUS g.7173921G>A g.7066617G>A C1S(NM_001734.3):c.971G>A (p.(Gly324Glu)) - C1S_000002 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
-?/. - c.1116C>T r.(?) p.(Asp372=) - likely benign g.7174996C>T g.7067692C>T C1S(NM_201442.3):c.1116C>T (p.D372=) - C1S_000010 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+/. 10 c.1132_1135del r.(?) p.(Phe378Valfs*32) - pathogenic (recessive) g.7175012_7175015del g.7067708_7067711del - - C1S_000030 Introduced as pathogenic in ClinVar, last update Oct 15 2016 PubMed: Inoue 1998 Journal: Inoue 1998 ClinVar-SCV000038879.2 - Germline - - - - - Christian Drouet
+/. 10 c.1132_1135del r.(?) p.(Phe378Valfs*32) - pathogenic (recessive) g.7175012_7175015del g.7067708_7067711del - - C1S_000030 Proband is a compound heterozygous carrier c.[1132-1135del];[1789G>T], with a complete C1s deficiency; his parents and heterozygous carriers of the family are presenting with one-half of the normal antigenic C1s PubMed: Endo 1999, Journal: Endo 1999 - - Germline yes - - - - Christian Drouet
-/. - c.1167A>G r.(?) p.(Pro389=) - benign g.7175047A>G g.7067743A>G C1S(NM_201442.4):c.1167A>G (p.P389=) - C1S_000011 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_VUmc
-?/. - c.1198G>A r.(?) p.(Glu400Lys) - likely benign g.7175762G>A - C1S(NM_201442.3):c.1198G>A (p.E400K) - C1S_000020 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-?/. - c.1218C>T r.(?) p.(Asn406=) - likely benign g.7175782C>T - C1S(NM_201442.4):c.1218C>T (p.N406=) - C1S_000023 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Utrecht
-?/. - c.1258A>G r.(?) p.(Lys420Glu) - likely benign g.7175822A>G - C1S(NM_201442.3):c.1258A>G (p.K420E) - C1S_000017 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-?/. - c.1284C>G r.(?) p.(Pro428=) - likely benign g.7177172C>G g.7069868C>G C1S(NM_001734.3):c.1284C>G (p.P428=), C1S(NM_001734.5):c.1284C>G (p.P428=), C1S(NM_201442.3):c.1284C>G (p.P428=) - C1S_000003 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-?/. - c.1284C>G r.(?) p.(Pro428=) - likely benign g.7177172C>G - C1S(NM_001734.3):c.1284C>G (p.P428=), C1S(NM_001734.5):c.1284C>G (p.P428=), C1S(NM_201442.3):c.1284C>G (p.P428=) - C1S_000003 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Utrecht
-/. - c.1284C>G r.(?) p.(Pro428=) - benign g.7177172C>G - C1S(NM_001734.3):c.1284C>G (p.P428=), C1S(NM_001734.5):c.1284C>G (p.P428=), C1S(NM_201442.3):c.1284C>G (p.P428=) - C1S_000003 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_VUmc
-?/. - c.1326C>T r.(?) p.(Ser442=) - likely benign g.7177214C>T g.7069910C>T C1S(NM_001734.3):c.1326C>T (p.S442=), C1S(NM_001734.5):c.1326C>T (p.S442=), C1S(NM_201442.3):c.1326C>T (p.S442=) - C1S_000014 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-?/. - c.1326C>T r.(?) p.(Ser442=) - likely benign g.7177214C>T g.7069910C>T C1S(NM_001734.3):c.1326C>T (p.S442=), C1S(NM_001734.5):c.1326C>T (p.S442=), C1S(NM_201442.3):c.1326C>T (p.S442=) - C1S_000014 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Utrecht
-/. - c.1326C>T r.(?) p.(Ser442=) - benign g.7177214C>T - C1S(NM_001734.3):c.1326C>T (p.S442=), C1S(NM_001734.5):c.1326C>T (p.S442=), C1S(NM_201442.3):c.1326C>T (p.S442=) - C1S_000014 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_VUmc
?/. - c.1388C>T r.(?) p.(Ala463Val) - VUS g.7177276C>T g.7069972C>T 1388C>T - C1S_000019 - PubMed: Duvvari 2016 - rs375077429 Germline - - - - - LOVD
+/. - c.1537G>T r.(?) p.(Gly513Ter) - pathogenic g.7177425G>T g.7070121G>T C1S(NM_201442.4):c.1537G>T (p.G513*) - C1S_000013 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
+/. - c.1537G>T r.(?) p.(Gly513Ter) - pathogenic g.7177425G>T - C1S(NM_201442.4):c.1537G>T (p.G513*) - C1S_000013 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_VUmc
+/+ 12 c.1567C>T r.(?) p.(Arg523*) - likely pathogenic (recessive) g.7177455C>T g.7070151C>T - - C1S_000028 2-year-old female proband presenting with a complete C1s deficiency and multiple autoimmune features. Heterozygous parents present with normal CH50 activity and decreased antigenic C1s Journal: Dragon-Durey et al., 2001 - rs781856506 Germline - 0.000004 - - - Christian Drouet
+/. - c.1567C>T r.(?) p.(Arg523*) - pathogenic g.7177455C>T - C1S(NM_001734.5):c.1567C>T (p.(Arg523*)) - C1S_000028 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
+/. - c.1600C>T r.(?) p.(Arg534Trp) - pathogenic g.7177488C>T g.7070184C>T - - C1S_000015 1 heterozygous, no homozygous; Clinindb (India) PubMed: Narang 2020, Journal: Narang 2020 - rs121909582 Germline - 1/2795 individuals - - - Mohammed Faruq
-?/. - c.1665C>T r.(?) p.(=) - likely benign g.7177553C>T - C1S(NM_001734.5):c.1665C>T (p.S555=) - C1S_000036 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_VUmc
+/. 12 c.1789G>T r.(?) p.(Glu597*) - pathogenic (recessive) g.7177677G>T g.7070373G>T - - C1S_000031 Compound heterozygosity c.[1789G>T];[1889G>A] identified in the female proband and her brother Journal: Abe 2009 - - Germline yes - - - - Christian Drouet
+/. 12 c.1789G>T r.(?) p.(Glu597*) - pathogenic (recessive) g.7177677G>T g.7070373G>T - - C1S_000031 Proband is a compound heterozygous carrier c.[1132-1135de]l;[1789G>T], with a complete C1s deficiency; his parents and heterozygous carriers of the family are presenting with one-half of the normal antigenic C1s PubMed: Endo 1999, Journal: Endo 1999 - - Germline yes - - - - Christian Drouet
-?/. - c.1827C>T r.(?) p.(Ala609=) - likely benign g.7177715C>T g.7070411C>T C1S(NM_201442.3):c.1827C>T (p.A609=) - C1S_000004 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-?/. - c.1851C>T r.(?) p.(Ile617=) - likely benign g.7177739C>T - C1S(NM_001734.5):c.1851C>T (p.I617=) - C1S_000024 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_VUmc
+/. 12 c.1889G>A r.(?) p.(Gly630Glu) - pathogenic (recessive) g.7177777G>A g.7070473G>A - - C1S_000032 Compound heterozygosity c.[1789G>T];[1889G>A] identified in the female proband and her brother Journal: Abe 2009 - - Germline yes - - - - Christian Drouet
?/. - c.1945G>A r.(?) p.(Ala649Thr) - VUS g.7177833G>A - C1S(NM_001734.5):c.1945G>A (p.(Ala649Thr)) - C1S_000038 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
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