All variants in the SERPING1 gene

Information The variants shown are described using the NM_000062.2 transcript reference sequence.

1270 entries on 13 pages. Showing entries 1 - 100.
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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     
?/? _1_8_ c.-2332054_*136672dup r.0? p.0? ACMG VUS g.55033164_57518726dup g.55265691_57751253dup - - SERPING1_000776 Long duplicated sequence encompassing the entire SERPING1 gene. Introduced as VUS in ClinVar by GenomeConnect Lewisburg PA; no assertion provided - ClinVar-000684481 - De novo - - - - - Christian Drouet
?/-? _1_8_ c.-218202_*285168dup r.0? p.0? - benign g.57147016_57667222dup g.57379543_57899749dup - - SERPING1_000778 Long duplicated sequence encompassing the whole SERPING1 gene. Introduced as benign variant in Clinvar by Cincinnati Children's Hospital Medical Center Genetics and Genomics Diagnostic Laboratory, Cincinnati OH; no assertion provided - ClinVar-000613420 - Germline - - - - - Christian Drouet
?/-? _1_8_ c.-59207_*287295dup r.0? p.0? - benign g.57306011_57669349dup g.57538538_57901876dup - - SERPING1_000777 - - ClinVar-000613421 - CLASSIFICATION record - - - - - Christian Drouet
+?/-? _1 c.-7913G>A r.(=) p.(=) - likely benign g.57357305G>A g.57589832G>A - - SERPING1_000331 Significant difference in higher C1-INH antigenic levels in AMD cases versus controls. Journal: Gibson 2012 - rs2649663 Germline - 0.1757 - - - Christian Drouet
+/+ _1_2 c.-1998_98del r.? p.? ACMG pathogenic g.57363220_57367398del g.57595747_57599925del Large deletion 4.18 kb - SERPING1_001031 A 4.18-kb deletion encompassing entire exons 1 and 2 and a 5' section of exon 3 Journal: Ren 2023 - - Germline yes - - - - Christian Drouet
+/+ _1_3 c.-953_345del r.? p.? ACMG pathogenic g.57364265_57367645del g.57596792_57600172del - - SERPING1_001095 Variant resulting in the deletion of exons 1-2 and part of exon 3 (c.-953_345del) Submitted to ClinVar as pathogenic by InVitae, San Francisco CA - ClinVar-SCV005064300.1 - Germline - - - - - Christian Drouet
+/+ _1_8_ c.(-387?)_(*422?)del r(0?) p(0?) ACMG pathogenic g.(57364832?)_(57382477?)del g.(57597359?)_(57615004?)del 9.3 Mb interstitial deletion of chromosome 11 including at least a small sequence of the short arm and a larger region of the long arm and encompassing exons 1 to 8 - SERPING1_000731 - Journal: Ebo 2019 - - De novo yes - - - - Christian Drouet
+/+ _1_8_ c.(-387?)_(*422?)del r(0?) p(0?) ACMG pathogenic g.(57364832?)_(57382477?)del g.(57597359?)_(57615004?)del whole gene deletion from exon 1 to exon 8 - SERPING1_000731 Deletion variant with unidentified boundaries Journal: Markocsy 2024 - - Germline yes - - - - Christian Drouet
+/+ _1_2i c.-387_52-123del r.? p.? ACMG pathogenic g.57364831_57367229del g.57597358_57599756del - - SERPING1_000732 a 2,399-nt deletion variant encompassing exons 1 to 2 Journal: Roche 2005 Journal: Loules 2018 - - Germline yes - - - - Christian Drouet
+/+ _1_8_ c.-387_*422del r.(0?) p.(0?) ACMG pathogenic g.57364832_57382477del g.57597359_57615004del - - SERPING1_000731 a 17,646-nt deletion variant encompassing exons 1 to 8 Journal: Roche 2005 Journal: Loules 2018 - - Germline yes - - - - Christian Drouet
+?/+? 1 c.-163C>T r.(=) p.(=) ACMG pathogenic (recessive) g.57365055C>T g.57597582C>T c.[-103C>T];[-103C>T] - SERPING1_000185 c.-163C>T variant is the sole idiomorphic nucleotide change in the kindred, found homozygous in the proband, at variance with the dominant mode of transmission observed for structural mutations. Pathogenic when homozygous, with severe HAE. and low C1-INH levels; homozygosity because of consanguinity. Variant altering the first nucleotide of a putative CAAT box, the first promoter variant reported in the SERPING1 gene. In contrast, heterozygous individuals display C1-INH levels within the normal range, although often at its lower level, and were free of angioedema attacks. PubMed: Verpy 1996 ClinVar-000003956 rs1387768389 Germline no 0.000007 (gnomAD) - - - Christian Drouet
+?/+? 1 c.-161A>G r.(=) p.(=) ACMG pathogenic (recessive) g.57365057A>G g.57597584A>G c.-101A>G - SERPING1_000186 Putatively disrupts CAAT box. One homozygous proband presenting with a HAE-I phenotype; two homozygous affected siblings. Heterozygous carriers have been recorded as unaffected indicating a recessive c.-(161)A>G variant. Introduced in ClinVar by Research Centre for Medical Genetics, Moscow Russia that indicated variant -161A>G meets ACMG /ClinGen criteria to be classified as pathogenic: PS3, PP1_Str, PS4_Mod, PM2_Sup. Erroneously published as c.-101A>G Journal: Büyüköztürk 2009 Journal: Kesim 2011 ClinVar-SCV005061388.1 rs766344850 Germline no 0.0000319 (gnomAD) - - - Christian Drouet
+/+ 1;1 c.[-161A>G];[-161A>G] r.(=) p.(=) ACMG pathogenic (recessive) g.57365057A>G g.57597584A>G c.-101A>G; g.[57365057A>G];[57365057A>G] - SERPING1_000900 Regulatory c.–161A>G variant is coordinated to -687 relative to initiation codon; it changed the sequence of -CAAT- box in 5'UTR, with subsequent reduced level of C1-INH mRNA in the homozygous proband comparatively to that is shown in heterozygous individuals. Journal: Büyüköztürk 2009 Journal: Kesim 2011 - - Germline no - - - - Christian Drouet
?/? 1 c.-105C>A r.(=) p.(=) ACMG VUS g.57365113C>A g.57597640C>A - - SERPING1_000815 - - ClinVar-000305010 rs886048397 CLASSIFICATION record - 0.00006 (TOPMed) - - - Christian Drouet
?/. 1 c.-100C>G r.(=) p.(=) ACMG VUS g.57365118C>G g.57597645C>G c.-40C>T - SERPING1_000182 Pathogenic variant when in a cis configuration with variant c.506T>C. Variant c.-100C>G affecting a pyrimidine-rich region (c.-108 to c.-77) of potential H-DNA structure. PubMed: Verpy 1996 Journal: Ponard 2019 ClinVar-000877957 rs578018379 Germline yes 0.0002 (1000Genomes) - - - Christian Drouet
-?/-? 1 c.-100C>G r.(=) p.(=) - likely benign g.57365118C>G g.57597645C>G [-100C>G;816_818del] - SERPING1_000901 - PubMed: Verpy 1996 - - Germline ? - - - - Christian Drouet
?/-? 1 c.-99C>G r.(=) p.(=) - VUS (!) g.57365119C>G g.57597646C>G - - SERPING1_001029 Introduced as VUS in ClinVar by Illumina, San Diego CA, and benign by Prevention Genetics, Marshfield WI; methods: clinical testing, no C1-INH function analysis - ClinVar-SCV000372543.3 rs866115469 Germline - 0.00010 - - - Christian Drouet
?/. 1 c.-99dup r.(=) p.(=) ACMG likely benign g.57365119dup g.57597646dup - - SERPING1_000857 - - ClinVar-SCV000372541.2 rs28362939 Germline - 0.001203 (TOPMed); 0.0080 (40/5008; 1000 Genome) - - - Christian Drouet
-?/-? 1 c.-66_-65del r.(?) p.(=) - likely benign g.57365152_57365153del g.57597679_57597680del - - SERPING1_000330 - Journal: Roche 2005 - rs917061427 Germline - 0.000056 (TOPMed) - - - Christian Drouet
-?/-? 1 c.-58T>C r.(=) p.(=) - benign g.57365160T>C g.57597687T>C c.-584T>C - SERPING1_000333 Variant found in one control Journal: Roche 2005 - - Germline - - - - - Christian Drouet
-?/-? 1 c.-56T>G r.(=) p.(=) ACMG VUS g.57365162T>G g.57597689T>G - - SERPING1_000335 Introduced in ClinVar as VUS by Illumina Laboratory Services, San Diego CA - ClinVar-SCV000372544.3 rs886048398 Germline - - - - - Christian Drouet
+/+ 1_8_ c.-35_*263del r.? p.? ACMG pathogenic g.57365183_57382317del g.57597710_57614844del - - SERPING1_000838 - Journal: Loli-Ausejo 2021 - - Germline - - - - - Christian Drouet
-?/. 1 c.-24G>C r.(=) p.(=) ACMG likely benign g.57365194G>C g.57597721G>C - - SERPING1_000336 - - ClinVar-RCV000312741.2 rs112290300 Germline - 0.00026 (gnomAD); 0.000725 (TOPMed) - - - Christian Drouet
+/+ _1_2i c.(-191_-23)_(51+1_52-1)del r.0? p.0? ACMG pathogenic g.(57365027_57365195)_(57365795_57367351)del g.(57597554_57597722)_(57598322_57599878)del exons 1_2 deletion of unknown length - SERPING1_000751 c.(-191_-23)_(51+1_52-1)del variant carried by a de novo proband Considered as pathogenic in agreement with ACMG criteria PVS1, PS2_Str, PS3, PS4, PP4 Journal: Lopez-Lera 2011 Journal: Ponard 2019 Journal: Wang 2022 - - De novo yes - - - - Christian Drouet
+/+ _1_2i c.(-191_-23)_(51+1_52-1)del r.0? p.0? ACMG pathogenic g.(57365027_57365195)_(57365795_57367351)del g.(57597554_57597722)_(57598322_57599878)del 4.18-kb deletion encompassing exons 1 and 2 - SERPING1_000751 Boundaries uncovered by WGS Journal: Ren 2023 - - Germline yes - - - - Christian Drouet
+/+ _1_3i c.(-191_-23)_(550+1_551-1)del r.0? p.0? ACMG pathogenic g.(57365027_57365195)_(57367851_57369507)del g.(57597554_57597722)_(57600378_57602034)del 4-kb deletion encompassing exons 1 to 3 - SERPING1_000769 2.8 kb deletion of the genomic region encompassing exons 1-3 of the SERPING1 gene Introduced in ClinVar as pathogenic variant by InVitae PubMed: Stoppa-Lyonnet 1991 Journal: Ponard 2019 ClinVar-SCV002243450.2 - Germline yes - - - - Christian Drouet
+/+ _1_4i c.(-191_-23)_(685+1_686-1)del r.0? p.0? ACMG pathogenic g.(57365027_57365195)_(57369643_57373482)del g.(57597554_57597722)_(57602170_57606009)del exons 1_4 deletion with a 9-kb deletion - SERPING1_000752 - PubMed: Stoppa-Lyonnet 1991 - - Germline yes - - - - Christian Drouet
+/+ _1_4i c.(-191_-23)_(685+1_686-1)del r.0? p.0? ACMG pathogenic g.(57365027_57365195)_(57369643_57373482)del g.(57597554_57597722)_(57602170_57606009)del exons 1_4 deletion with unknown length - SERPING1_000752 - PubMed: Duponchel 2001 Journal: Johnsrud 2015 Journal: Ponard 2019 Journal: Loli-Ausejo 2021 Journal: Hashimura 2021 Journal: Wang 2022 ClinVar-SCV002243450.2 - Germline yes - - - - Christian Drouet
+/+ _1_6i c.(-191_-23)_(1029+1_1030-1)del r.0? p.0? ACMG pathogenic g.(57365027_57365195)_(57374021_57379189)del g.(57597554_57597722)_(57606548_57611716)del large deletion (17-kb long) of exons 1 to 6 - SERPING1_000753 - PubMed: Stoppa-Lyonnet 1991 Journal: Ponard 2019 Journal: Förster 2021 ClinVar-SCV003790197.1 - Germline yes - - - - Christian Drouet
+/+ _1_6i c.(-191_-23)_(1029+1_1030-1)del r.0? p.0? ACMG pathogenic g.(57365027_57365195)_(57374021_57379189)del g.(57597554_57597722)_(57606548_57611716)del exons 1_6 deletion with a 9.3-kb deletion - SERPING1_000753 - Journal: Aradhya 2012 - - Germline - - - - - Christian Drouet
+/+ _1_6i c.(-191_-23)_(1029+1_1030-1)del r.0? p.0? ACMG pathogenic g.(57365027_57365195)_(57374021_57379189)del rrg.(57597554_57597722)_(57606548_57611716)del EX1_6del - SERPING1_000753 A large deletion of unknown length encompassing exons 1 to 6 Journal: Grombikirova 2023 - - Germline yes - - - - Christian Drouet
+/+ _1_7i c.(-191_-23)_(1249+1_1250-1)del r.0? p.0? ACMG pathogenic g.(57365027_57365195)_(57379410_57381800)del g.(57597554_57597722)_(57611937_57614327)del large deletion encompassing exons 1 to 7 - SERPING1_000754 - Journal: Pedrosa 2016 - - Germline - - - - - Christian Drouet
+/+ _1_8_ c.(-191_-23)_(*272_?)del r.0? p.0? ACMG pathogenic g.(57365027_57365195)_(57382326_?)del g.(57597554_57597722)_(57614853_?)del exons 1_8 deletion of unknown length - SERPING1_000755 Recurrent gross deletion Introduced in ClinVar as a pathogenic variant by InVitae, San Francisco CA PubMed: Duponchel 2001 Journal: Roche 2005 Journal: Iwamoto 2012 Journal: Johnsrud 2015 Journal: Ponard 2019 Journal: Loli-Ausejo 2021 Journal: Hashimura 2021 Journal: Szabó 2022 Journal: Grombikirova 2023 ClinVar-SCV001591839.3 - De novo yes - - - - Christian Drouet
+/. 1i c.-23+5del r.spl? p.? - VUS g.57365200del g.57597727del -23+5delG - SERPING1_000012 - Journal: Ponard 2019 - - Germline yes - - - - Christian Drouet
+/? 1i c.-23+5G>A r.(spl)? p.0? - VUS g.57365200G>A g.57597727G>A - - SERPING1_000016 - Journal: Ponard 2019 - - Germline yes - - - - Christian Drouet
+?/. 1i c.-23+45T>C r.(=) p.(=) - VUS (!) g.57365240T>C g.57597767T>C c.-504T>C - SERPING1_000334 Uncharacterized sequence variant at intron 1. Found in one patient and one affected relative. Co-segregates with disease. Journal: Roche 2005 - rs183957596 Germline yes 0.0052 (TOPMED); 0.00527 (GnomAD); 0.0012 (6/5008, 1000G) - - - Christian Drouet
+/+ 1i c.-23+184_-22-152del r.(=) p.(=) ACMG likely pathogenic g.57365379_57365570del g.57597906_57598097del - - SERPING1_000415 A 191-nt deletion within intron 1 Journal: Ponard 2019 - - Germline yes - - - - Christian Drouet
+/+ 1i c.-22-155G>T r.(=) p.(=) ACMG pathogenic g.57365567G>T g.57598094G>T - - SERPING1_000786 The c.-22-155G>T variant introduces in the genome a new donor site stronger than the wild type leading to a larger exon 1 in the RNA level. The c.-22-155G>T variant disrupts an ISS recognized by a number of transcriptional factors and subsequently stops the suppression of the intronic cryptic donor site. Transcriptional analysis indicated that the mutant mRNA is susceptible to degradation. The c.-22-155G > T variant co-segregated with C1-INH-HAE in all of the 4 analyzed patients, while it was absent from 3 healthy family members. The c.-22-155G > T variant meets ACMG criteria to be considered pathogenic: PS3, PS4, PM2, PP1_Str, PP3, PP4 Journal: Vatsiou 2020 ClinVar-000870444 rs1945307391 Germline yes - - - - Christian Drouet
+/+? 1i c.-22-25_-22-9del r.spl? p.0? - VUS g.57365697_57365713del g.57598224_57598240del -22-25_-9del - SERPING1_000017 - Journal: Ponard 2019 - - Germline yes - - - - Christian Drouet
+/+ 1i c.-22-19_-22-4del r.spl? p.? ACMG likely pathogenic g.57365703_57365718del g.57598230_57598245del - - SERPING1_000018 Mutation near splice site in SERPING1 transcript 1. Journal: Ponard 2019 Journal: Obtulowicz 2020 Journal: Grombirikova 2023 - - De novo yes - - - - Christian Drouet
+/+ 1i c.-22-10_-22-7del r.(=) p.(=) - likely pathogenic g.57365712_57365715del g.57598239_57598242del c.-22-10_-7delGGCT - SERPING1_000019 Transcript expression failed. Family presenting with a compound heterozygous situation c.[(-21)T>C](;)[-22-10_-22-7del] in a trans configuration (n=2) and in both cis and trans configurations (n=1), with clinical phenotype affected; -patient 1, female, c.[(-21)T>C];[-22-10_-22-7del];[(-21)T>C], severe; -patient 2, female, c.[(-21)T>C];[-22-10_-22-7del], moderate; -patient 3, male, c.[(-21)T>C];[-22-10_-22-7del], mild Journal: Ponard 2019 - - Germline yes - - - - Christian Drouet
+/+? 1i c.-22-7_-22-3del r.spl? p.? - VUS g.57365715_57365719del g.57598242_57598246del -22-7_-3delTCCGC - SERPING1_000020 c.-22-7_-22-3delTCCGC variant might affect the acceptor splice site of intron 1. Additional investigations on transcripts are expected. Journal: Ponard 2019 - - Germline - - - - - Christian Drouet
+/+? 1i c.-22-3C>G r.spl? p.? ACMG likely pathogenic g.57365719C>G g.57598246C>G - - SERPING1_001125 The c.-22-3C>G variant in SERPING1 meets ACMG/ClinGen criteria to be classified as likely pathogenic: PP4_Str, PS1_Mod, PM2_Sup, PP3. Introduced in ClinVar as likely pathogenic by Research Centre For Medical Genetics, Moscow Russia - ClinVar-SCV005077925.1 - Germline yes - - - - Christian Drouet
+/+ 1i c.-22-2A>C r.spl? p.0? ACMG pathogenic g.57365720A>C g.57598247A>C c.-22-2A>C - SERPING1_000135 - Journal: Aabom 2017 - - Germline/De novo (untested) yes - - - - Christian Drouet
+/+ 1i c.-22-2A>G r.spl? p.0? ACMG pathogenic g.57365720A>G g.57598247A>G c.-22-2A>G - SERPING1_000134 Recurrent variant c.-22-2A>G variant is likely to affect the acceptor splice site of intron 1. Variant with responsibility for decreased C1 Inhibitor function and HAE Journal: Bygum 2011 Journal: Veronez 2019 Journal: Ponard 2019 Journal: Hashimura 2021 - - Germline yes - - - - Christian Drouet
+/+ 1i c.-22-2A>T r.spl? p.0? ACMG pathogenic g.57365720A>T g.57598247A>T - - SERPING1_000021 - Journal: Ponard 2019 - - Germline - - - - - Christian Drouet
+/+ 1i c.-22-1G>A r.-22_51del p.? ACMG pathogenic g.57365721G>A g.57598248G>A - - SERPING1_000137 Recurrent variant Variant that affects intron 1 acceptor splice site, with subsequent partial to complete exon 2 skipping. Introduced in ClinVar as pathogenic by Research Centre for Medical Genetics, Moscow Russia, indicating variant c.-22-1G>A meets ACMG/ClinGen criteria to be classified as pathogenic: PVS1, PP4_Str, PS4_Mod, PM2_Sup. PubMed: Verpy 1996 Journal: Gösswein 2008 Journal: Rijavec 2013 Journal: Andrejević 2015 Journal: Loules 2018 ClinVar-SCV005077924.1 - Germline yes - - - - Christian Drouet
+/+ 1i_2i c.(-23+1_-22-1)_(51+1_52-1)del r.(?) p.(0?) ACMG pathogenic g.(57365196_57365721)_(57365795_57367351)del g.(57597723_57598248)_(57598322_57599878)del - - SERPING1_000756 exon 2 deletion, probably encompassing introns 1 and 2 Journal: Lopez-Lera 2011 - - Germline yes - - - - Christian Drouet
+/+ 1i_3i c.(-23+1_-22-1)_(550+1_551-1)del r.spl? p.? ACMG pathogenic g.(57365196_57365721)_(57367851_57369507)del g.(57597723_57598248)_(57600378_57602034)del deletion of exons 2_3 - SERPING1_000131 - Journal: Ponard 2019 - - Germline - - - - - Christian Drouet
+/+ _1_4i c.(-191_-22-1)_(685+1_686-1)del r.(0?) p.(0?) ACMG pathogenic g.(57365027_57365721)_(57369643_57373482)del g.(57597554_57598248)_(57602170_57606009)del deletion of exons 1 to 4 - SERPING1_000749 9-kb deletion variant identified by RFLP PubMed: Stoppa-Lyonnet 1991 - - Germline yes - - - - Christian Drouet
+/+ _2_4_ c.(-23+1_-22-1)_(685+1_686-1)del r.(52_685del) p.(?) ACMG pathogenic g.(57365196_57365721)_(57369643_57373482)del g.(57597723_57598248)_(57602170_57606009)del exons 2-4 deletion - SERPING1_000982 - Journal: Wang 2022 - - Germline - - - - - Christian Drouet
+/+ 1i_5i c.(-23+1_-22-1)_(889+1_890-1)del r.? p.0? ACMG pathogenic g.(57365196_57365721)_(57373687_57373880)del g.(57597723_57598248)_(57606214_57606407)del deletion exons 2_5 - SERPING1_000132 variant identified by FAMA Journal: Ponard 2019 - - Germline - - - - - Christian Drouet
-?/-? 2 c.-21T>C r.(?) p.(=) ACMG likely benign g.57365723T>C g.57598250T>C SERPING1(NM_000062.2):c.-21T>C - SERPING1_000001 VKGL data sharing initiative Nederland Journal: Duponchel 2006 Journal: Ponard 2019 ClinVar-SCV000372547.3 rs28362944 CLASSIFICATION record no 0.029081 (GnomAD_exome); 0.030270 (TOPMed) - - - VKGL-NL_Utrecht
-/-? 2 c.-21T>C r.(?) p.(=) ACMG benign g.57365723T>C g.57598250T>C SERPING1(NM_000062.2):c.-21T>C - SERPING1_000001 VKGL data sharing initiative Nederland - - rs28362944 CLASSIFICATION record - 0.02908 - - - VKGL-NL_Nijmegen
-?/-? 2 c.-21T>C r.(?) p.(=) ACMG likely benign g.57365723T>C g.57598250T>C c.[-566T>C;2650T>C] - SERPING1_000659 Pathogenic compound heterozygosity in a cis combination c.[-21T>C;506T>C]. PubMed: Verpy 1996 ClinVar-SCV000372547.3 rs28362944 Germline - 0.0314 (gnomADv3) - - - Christian Drouet
-/-? 2 c.-21T>C r.(=) p.(=) - benign g.57365723T>C g.57598250T>C - - SERPING1_000001 The c.-21 T>C variant positioned in trans with another causal variant in the SERPING1 gene was associated with the higher frequency of attacks, lower age at disease onset and higher clinical severity score (Grombirikova 2023) PubMed: Bafunno 2013, Journal: Bafunno 2013 Journal: Grombirikova 2023 ClinVar-SCV000372547 rs28362944 Germline - 0.029081 - - - Johan den Dunnen
+?/+? 2;2 c.[-21T>C];[-21T>C] r.(-22_51del) p.0? ACMG likely pathogenic (recessive) g.57365723T>C g.57598250T>C c.-21T>C - SERPING1_000658 exon 2 skipped (minigene transfected in Hep3B cells); variant likely pathogenic when homozygous Average frequency (large NGS studies) 0.02415 Freq EA 264/8028 Freq AA 27/4024 (omner Ivo F.A.C. Fokkema) Journal: Duponchel 2006 Journal: Rijavec 2013 ClinVar-RCV000365154.1 rs28362944 Germline no 0.02893 (gnomAD) 0.014577 (1000Genomes) - - - Christian Drouet
+/+ _2_8_ c.(-23+1_-22-1)_(*272?)del r.(52_*272)del p.? ACMG pathogenic g.(57365196_57365721)_(57382326_?)del g.(57597723_57598248)_(57614853_?)del exon 2-8 deletion with 16,312 bp loss - SERPING1_001196 - Journal: Gao 2025 - - Germline yes - - - - Christian Drouet
+/+ 3i_8_ c.(550+1_551-1)_(*422?)del r.(?) p? ACMG pathogenic g.(57367851_57369507)_(57382477?)del g.(57600378_57602034)_(57615004?)del deletion of exon 4 to 8 - SERPING1_000898 - Journal: Hashimura 2021 - - Germline - - - - - Christian Drouet
+/+ _1_6_ c.(_-163)_(1029+20_1030-1)del r.? p.? ACMG pathogenic g.(57365055_57374040)del g.(57597582_57606567)del exons 1-6 deletion - SERPING1_001097 Gross deletion of the genomic region encompassing exons 1-6 of unknown length, which includes the initiation codon. This deletion extends beyond the assayed region for SERPING1 gene and therefore may encompass additional genes. It is expected to result in an absent or disrupted protein product. Submitted to ClinVar as pathogenic by InVitae, San Francisco CA. - ClinVar-SCV003790197.3 - Germline/De novo (untested) - - - - - Christian Drouet
+/+ _1_3_ c.(_-163)_(550+20_551-1)del r.? p.? ACMG pathogenic g.(57365055)_(57367870)del g.(57597582)_(57600397)del exons 1-3 deletion of unknown length - SERPING1_001098 Gross deletion of the genomic region encompassing exons 1-3, which includes the initiation codon. This deletion extends beyond the assayed region for this gene and therefore may encompass additional genes. Submitted to ClinVar as pathogenic by InVitae, San Francisco CA. - ClinVar-SCV002243450.4 - Germline/De novo (untested) - - - - - Christian Drouet
+/+ _1_4_ c.(_-163)_(685+20_686-1)del r.? p.? ACMG pathogenic g.(57365055)_(57369662)del g.(57597582)_(57602189)del exons 1-4 deletion of unknown length - SERPING1_001099 Gross deletion of the genomic region encompassing exons 1-4, which includes the initiation codon. This deletion extends beyond the assayed region for SERPING1 gene and therefore may encompass additional genes. Submitted to ClinVar as pathogenic by InVitae, San Francisco CA. - ClinVar-SCV002231896.4 - Germline/De novo (untested) - - - - - Christian Drouet
+/+ _1_4i c.-1272_(686-538)del r.(-22_685?)del p.? ACMG pathogenic g.57363946_57372945del g.57596473_57605472del g.57363946_57372945del - SERPING1_001197 - Journal: Gao 2025 - - Germline yes - - - - Christian Drouet
?/? _1_8_ c.-191_*272{2} r.(=) p.(=) - VUS g.57139699_57703639dup g.57372226_57936167dup - - SERPING1_000865 - - ClinVar-000058162 - CLASSIFICATION record - - - - - Christian Drouet
+/+ 7;7 c.[1202T>C] r.[(1202u>c)] p.[(Ile401Thr)] ACMG pathogenic (recessive) g.57379362T>C g.57611889T>C g.[57379362T>C];[57379362T>C] - SERPING1_000725 Two homozygous individuals presenting with a more severe clinical phenotype than the single affected heterozygous individual, presenting with a nearly asymptomatic HAE phenotype. The Ile to Thr substitution at position 401 might affect the function of the gate Journal: Mete Gökmen 2020 - rs1263371770 Germline no 0.000004 - - - Christian Drouet
+/+ 3 c.[536C=/>T] r.(?) p.(Thr179Ile) ACMG pathogenic g.[57367836C=/>T] g.[57600363C=/>T] - - SERPING1_001174 Gonadal mosaicism in a family in which only both sisters, but not the parents, show clinical and laboratory findings typical of HAE, with allele segregation demonstrated using Sanger sequencing. c.536C>T variant not detected in DNA derived from lymphocytes from the father and the mother, whereas present on the DNA prepared from the sperm of the father and on the paternal transmitted chromosome. Journal: Ebo 2018 - - Uniparental disomy, paternal allele yes - - - - Christian Drouet
+/+ 4 c.[597C=/>G] r.(?) p.(Tyr199*) ACMG pathogenic g.[57369554C=/>G] g.[57602081C=/>G] - - SERPING1_000516 Gonadal mosaicism in a family in which only both sons, but not the parents, show clinical and laboratory findings typical of HAE, with allele segregation demonstrated using DHPLC. c.597C>G variant not not detected in DNA derived from buccal cells, urinary cells, hair roots and cultured fibroblasts from the mother, whereas occurred on the maternal transmitted chromosome. Journal: Guarino 2006 ClinVar-VCV000003957.1 rs121907951 Uniparental disomy, maternal allele yes 0.00000 (0/78700) - - - Christian Drouet
+/+ 3 c.[69_139del/=] r.(?) p.(Pro24Asnfs*10) ACMG pathogenic g.57367369_57367439del g.57599896_57599966del - - SERPING1_000652 Erroneously identified as a c.3_73del;p.(Asn1fs*34) variant by Yu et al 2007. Identified as c.67_137del in IDbases Gonadal mosaicism in a family in which both brothers, but not the parents, show clinical and laboratory findings typical of HAE, with allele segregation demonstrated using Sanger sequencing. c.69_139del variant not detected in DNA derived from lymphocytes from the father and the mother, whereas present on the DNA prepared from the sperm of the father. Journal: Yu 2007 - - Uniparental disomy, paternal allele yes - - - - Christian Drouet
+/+ 5i c.[890-1G=/>C] r.spl? p.? ACMG pathogenic g.[57373880G=/>C] g.[57606407G=/>C] c.[890-1G=/>C] - SERPING1_001173 Demonstrated exon 6 skiping and an aberrant in-frame insertion of 9-aa residues N-terminus of exon 6. Despite normal C1-INH function in the parents, the mother was found a mutation carrier. The inverted profile of the Sanger peaks compared with the patient, strongly suggests the presence of gonosomal mosaicism in the mother. Journal: Batlle-Masó 2025 - - Uniparental disomy, maternal allele yes - - - - Christian Drouet
+/+ 8 с.1442_1443insGCTGCGTGCT r.(?) p.(Trp482Leufs*19) ACMG pathogenic g.57381993_57381994insGCTGCGTGC g.57614520_57614521insGCTGCGTGC - - SERPING1_001187 - Journal: Baysheva 2024 - - Germline/De novo (untested) - - - - - Christian Drouet
+/+ 2 c.1A>C r.(?) p.(Met1Leu) ACMG pathogenic g.57365744A>C g.57598271A>C - - SERPING1_000464 c.1A>C variant affecting the initiation codon of the transcript of SERPING1 gene. Introduced in ClinVar as pathogenic variant by Department of Immunology and Histocompatibility, University of Thessaly. Variant validated by the SERPING1-NGS platform using NGS-Ion Torrent by Loules 2018 Journal: Speletas 2015 Journal: Loules 2018 ClinVar-SCV000900062.1 rs1565168898 Germline - - - - - Christian Drouet
+/+ 2 c.1A>G r.(1a>g) p.(Met1Val) ACMG pathogenic g.57365744A>G g.57598271A>G c.1A>G - SERPING1_000147 c.1A>G variant affects the initiation codon of the transcript of SERPING1 gene. Recurrent variant found in multiple pedigrees, Germany, Turkey, Greece, Spain, France, Hungary, China. One pedigree is presenting with a de novo mutation. According to ACMG Guidelines, with criteria PVS1, PS1, PS2, PS4, PM2, PP1, PP4, the variant is considered pathogenic. Journal: Gösswein 2008PubMed: Papadopoulou 2008Journal: Speletas 2009Journal: López-Lera 2011Journal: Bors 2013Journal: Ponard 2019Journal: Szabó 2022 Journal: Wang 2022 ClinVar-VCV000626352.1 rs1565168898 De novo yes - - - - Christian Drouet
+/+ 2 c.1A>T r.(?) p.(Met1Leu) ACMG likely pathogenic g.57365744A>T g.57598271A>T - - SERPING1_000463 c.1A>T variant affects the initiation codon required for SERPING1 gene expression Journal: Pappalardo 2008 Journal: Kesim 2011 - - Germline yes - - - - Christian Drouet
+/+ 2 c.2T>A r.(?) p.(Met1Lys) ACMG pathogenic g.57365745T>A g.57598272T>A - - SERPING1_000465 c.2T>A variant affects the initiation codon of the transcript of SERPING1 gene. Journal: Pappalardo 2008 - - Germline yes - - - - Christian Drouet
+/+ 2 c.2T>C r.(?) p.(Met1Thr) ACMG pathogenic g.57365745T>C g.57598272T>C - - SERPING1_000466 c.2T>C variant affects the initiation codon of the transcript of SERPING1 gene. Journal: Bafunno 2014 - - Germline - - - - - Christian Drouet
+?/+? 2 c.2T>G r.(?) p.(Met1Arg) ACMG pathogenic g.57365745T>G g.57598272T>G - - SERPING1_000467 c.2T>G variant affect the initiation codon required for SERPING1 gene expression. Introduced in ClinVar as pathogenic by Labcorp Genetics, San Francisco CA Journal: Loules 2018 ClinVar-RCV003734626.1 ClinVar-SCV004538883.1 - Germline yes - - - - Christian Drouet
+/+ 2 c.3G>A r.(?) p.(Met1?) ACMG pathogenic g.57365746G>A g.57598273G>A 589G>A (traditional) - SERPING1_000468 p.(Met1Ile) disrupts the initiation codon of SERPING1 gene expression. Italian paient samples exhibit a high level of circulating cleaved HK species, with 27.7% to 45.8% of total HK, consistent with an involvement of kallikrein-kinin system. Journal: Gösswein 2008 Journal: Pappalardo 2008 Journal: Suffritti 2014 Journal: Hashimura 2021 - - Germline yes - - - - Christian Drouet
+/+ 2_3 c.3_73del r.? p.? ACMG pathogenic g.57365746_57367373del g.57598273_57599900del - - SERPING1_000916 - PubMed: Lei 2011 - - Germline yes - - - - Christian Drouet
+/. 2 c.5C>T r.(?) p.(Ala2Val) - pathogenic g.57365748C>T g.57598275C>T SERPING1(NM_000062.2):c.5C>T (p.A2V), SERPING1(NM_000062.3):c.5C>T (p.A2V) - SERPING1_000002 VKGL data sharing initiative Nederland - ClinVar-000305016 rs185342631 CLASSIFICATION record - 0.0009841 (gnomAD v3) - - - VKGL-NL_AMC
-?/-? 2;7 c.5C>T r.(?) p.(Ala2Val) ACMG likely benign g.57365748C>T g.57598275C>T [5C>T];[1045C>T] - SERPING1_000370 Likely benign/benign with the following criteria BS1, BS2, BP2, BP4. Compound heterozygous proband c.[5C>T];[1045C>T] presenting with a HAE of the intermediate type. Two variants are in trans, c.5C>T maternal allele/c.1045C>T paternal allele, both parents are asymptomatic, proband's sister is not a carrier Journal: Ponard 2019 ClinVar-000305016 rs185342631 Germline no 0.0009841 (c.5C>T; gnomAD v3) - - - Christian Drouet
?/-? 2 c.5C>T r.(?) p.(Ala2Val) - VUS g.57365748C>T g.57598275C>T - - SERPING1_000002 conflicting interpretations of pathogenicity; 14 heterozygous, no homozygous; Clinindb (India) PubMed: Narang 2020 Journal: Narang 2020 - rs185342631 Germline - 14/2795 individuals - - - Mohammed Faruq
-?/-? 2 c.5C>T r.(?) p.(Ala2Val) ACMG VUS g.57365748C>T g.57598275C>T - - SERPING1_000002 Conflicting presentations of the c.5C>T variant. Introduced in ClinVar as a VUS by Center for Genomics, Ann and Robert H. Lurie Children's Hospital of Chicago. Variant c.5C>T is carried by 7 affected families. Families 1 & 2, Germany, 4 affected heterozygous individuals. HAE type not documented. Family 3, France, with a single affected individual presenting with a HAE type I mild phenotype. Family 4, France, a family where the proband is a compound heterozygous c.[5C>T];[1045C>T] and the c.5C>T allele is carried by the pauci-symptomatic mother. Family 5, Denmark, with a c.5C>T carrier associated with anti-C1Inh antibody. Family 6, Iran, with 2 male patients affected and presenting with a HAE type I phenotype. Family 7, Belarus (n=1), a young female patient presenting with a HAE type I phenotype since puberty. Family 8, Turkey (n=2) with a female patient suffering since 2.5 years old (moderate) and a male individual since 10 years old with laryngeal attacks. Five homozygous carriers c.[5C>T];[5C>T] have been recorded. Journal: Gösswein 2008 Journal: Ponard 2019 Journal: Rasmussen 2019 Journal: Nabilou 2020 Journal: Guryanova 2021 Journal: Soyak Aytekin 2021 ClinVar-SCV000898969.1 rs185342631 Germline yes 0.00089 (gnomAD v3); 0.001003 (TOPMED); 0.000799 (1000Genomes) - - - Christian Drouet
?/-? 2 c.5C>T r.(?) p.(Ala2Val) - VUS g.57365748C>T - SERPING1(NM_000062.2):c.5C>T (p.A2V), SERPING1(NM_000062.3):c.5C>T (p.A2V) - SERPING1_000002 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_VUmc
-?/-? 2;8 c.5C>T r.(?) p.(Ala2Val) ACMG VUS g.57365748C>T g.57598275C>T c.[5C>T(;)1397G>A] - SERPING1_000002 Combination of variants c.[5C>T(;)1397G>A] in a single individual presenting with a HAE type 1 phenotype; the pathogenic variant c.1397G>A fully explains the phenotype. Segregation analysis of c.5C>T in the family not possible, variant c.5C>T supposed having no impact on clinical phenotype and laboratory observations. Journal: Markocsy 2024 - - Germline ? - - - - Christian Drouet
?/. - c.5C>T r.(?) p.(Ala2Val) - VUS g.57365748C>T - SERPING1(NM_000062.2):c.5C>T (p.A2V), SERPING1(NM_000062.3):c.5C>T (p.A2V) - SERPING1_000002 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Groningen
+/+ 2 c.5_6del r.(?) p.(Ala2Valfs*17) ACMG pathogenic g.57365748_57365749del g.57598275_57598276del 5_6delCC - SERPING1_000876 - Journal: Hashimura 2021 - - Germline - - - - - Christian Drouet
+/+ 2 c.6dup r.(?) p.(Ser3Leufs*17) ACMG pathogenic g.57365749dup g.57598276dup - - SERPING1_001106 Patient presenting with recurrent and frequent angioedema attacks. The function and concentration of C1-INH and antigenic C4 of the parents and younger brother are normal. Immediate and closest relatives within 3 generations did not have a angioedema history; the possibility of a sporadic genetic mutation is considered. Journal: Zhang 2025 - - Germline/De novo (untested) yes - - - - Christian Drouet
+/+ 2 c.6_13del r.(?) p.(Ser3Aspfs*14) ACMG pathogenic g.57365749_57365756del g.57598276_57598283del - - SERPING1_001110 The c.6_13del variant in SERPING1 meets ACMG/ClinGen criteria to be classified as pathogenic: PVS1, PP4_Mod, PM2_Sup. - ClinVar-SCV005186251.1 - De novo yes - - - - Christian Drouet
+/+ 2 c.6_13dup r.(?) p.(Leu5Profs*4) ACMG pathogenic g.57365749_57365756dup g.57598276_57598283dup - - SERPING1_000138 A c.3_10dup was erroneously introduced in SERPING1base, ID @R4X8, accession S0049 on 30 July 2004; structure.bmc.lu.se/idbase/SERPING1base/ PubMed: Verpy 1996 Journal: Ponard 2019 - - Germline yes - - - - Christian Drouet
+?/+? 2 c.9del r.(?) p.(Arg4Glyfs*2) - likely pathogenic g.57365752del g.57598279del - - SERPING1_000797 No functional evidence for this variant No familial identification was provided - ClinVar-000830237 rs1945310324 CLASSIFICATION record - - - - - Christian Drouet
+/+ 2 c.15_16dup r.(?) p.(Thr6Argfs*4) ACMG pathogenic g.57365758_57365759dup g.57598285_57598286dup c.15_16dupGA - SERPING1_000141 Variant introduced in the Lund SERPING1 database http://structure.bmc.lu.se/idbase/SERPING1base/ Journal: Zuraw 2000 Journal: Chan 2024 - - Germline yes - - - - Christian Drouet
+/+ 2 c.17_24dup r.(?) p.(Thr9Profs*3) ACMG pathogenic g.57365760_57365767dup g.57598287_57598294dup 17_24dupCCCTGCTG - SERPING1_000877 - Journal: Hashimura 2021 - - Germline - - - - - Christian Drouet
+/+ 2 c.19dup r.(?) p.(Leu7Profs*13) ACMG likely pathogenic g.57365762dup g.57598289dup c.19dupC - SERPING1_000142 - Journal: Johnsrud 2015 - - Germline - - - - - Christian Drouet
+/+ 2 c.23dup r.(?) p.(Thr9Aspfs*11) ACMG pathogenic g.57365766dup g.57598293dup c.23insT - SERPING1_000140 - Journal: Bygum 2011 Journal: Veronez 2019 - - Germline/De novo (untested) - - - - - Christian Drouet
?/- 2 c.25A>C r.(?) p.(Thr9Pro) ACMG benign g.57365768A>C g.57598295A>C - - SERPING1_000745 - - ClinVar-SCV001726012.4 rs201455616 Not applicable - 0.000355 (gnomAD) 0.000637 (ExAC) - - - Christian Drouet
+/+ 2 c.29T>G r.(?) p.(Leu10Arg) ACMG likely pathogenic g.57365772T>G g.57598299T>G - - SERPING1_000022 Variant Leu10Arg is not expressed after transient transfection of minigene into 293T cells. Altering the hydrophobic core by substitution by positively charged amino acids can disrupt signal peptide transportation and impair protein secretion. Journal: Ponard 2019 Journal: Ren 2025 - - De novo yes - - - - Christian Drouet
+/+ 2 c.32dup r.(?) p.(Leu12Alafs*8) ACMG pathogenic g.57365775dup g.57598302dup - - SERPING1_000342 - Journal: Mete Gökmen 2019 - - Germline/De novo (untested) yes - - - - Christian Drouet
+/+ 2 c.33dup r.(?) p.(Leu12Alafs*8) ACMG pathogenic g.57365776dup g.57598303dup - - SERPING1_000343 - Journal: Pappalardo 2008 - - Germline - - - - - Christian Drouet
+/+ 2 c.35T>G r.(?) p.(Leu12Arg) ACMG likely pathogenic g.57365778T>G g.57598305T>G - - SERPING1_000469 Simulation by SignalP excludes the possibility that substitutions Leu to Arg has an important role in the proteolytic cleavage after the uptake of the protein into the endoplasmic reticulum. The variant p.(Leu12Arg) is not expressed when minigene is transfected into 293T cells. Altering the hydrophobic core by substitution with positively charged amino acids can disrupt signal peptide transportation and impair protein secretion. The c.35T>G variant in SERPING1 meets ACMG/ClinGen SVI guidance criteria to be classified as likely pathogenic: PP4_Mod, PS3, PS4_Mod, PM2_Sup, PP2, PP3 Journal: Bafunno 2014 Journal: Ren 2025 ClinVar-SCV005088165.1 - Germline yes - - - - Christian Drouet
+/+ 2 c.36dup r.(?) p.(Leu13Alafs*7) ACMG pathogenic g.57365779dup g.57598306dup - - SERPING1_000344 - Journal: Bafunno 2014 - - Germline - - - - - Christian Drouet
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