Unique variants in the KNG1 gene

Information The variants shown are described using the NM_001102416.2 transcript reference sequence.

26 entries on 1 page. Showing entries 1 - 26.
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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 _1 c.-85T>A r.(=) p.(=) - VUS g.186435247T>A g.186717458T>A - - KNG1_000002 - Journal: Loules 2020 ClinVar-VCV000827589.1 rs1579107719 Germline - - - - - Christian Drouet
?/. 1 1 c.166T>G r.(?) p.(Leu56Val) - VUS g.186435497T>G g.186717708T>G - - KNG1_000019 1 more item - ClinVar-SCV003807556.1 - Germline - - - - - Christian Drouet
+/. 1 2i c.306+2T>A r.spl p.? - pathogenic (recessive) g.186438006T>A g.186720217T>A c.[306+2T>A];[306+2T>A] - KNG1_000009 1 more item Journal: Adenaeuer 2023 ClinVar-SCV004031439.1 - Germline - - - - - Christian Drouet
?/. 1 4 c.421G>A r.(?) p.(Asp141Asn) - VUS g.186442906G>A g.186725117G>A - - KNG1_000003 - Journal: Loules 2020 ClinVar-VCV000827590.1 rs1579116763 Germline - 0.0 (0/10680; ALFA Project) - - - Christian Drouet
+/. 1 4 c.460C>A r.(?) p.(Pro154Thr) - pathogenic (recessive) g.186442945C>A g.186725156C>A - - KNG1_000023 - Journal: Chen 2024 - - Germline yes - - - - Christian Drouet
+/. 1 4 c.488del r.(?) p.(Gly163Alafs*20) - pathogenic g.186442973del g.186725184del c.[488del];[1165C>T] - KNG1_000012 Compound c.[488del];[1165C>T] variants abolish expression of HK and LK and function of HK Journal: Jeung 2020 Journal: Adenaeuer 2022 - - Germline - - - - - Christian Drouet
+/. 1 4 c.523_524dup r.(?) p.(Leu176Profs*8) - pathogenic g.186443008_186443009dup g.186725219_186725220dup c.[523_524dup];[523_524dup] - KNG1_000013 1 more item Journal: Fukushima 2014 Journal: Adenaeuer 2022 - - Germline yes - - - - Christian Drouet
+/. 3 5 c.586C>T r.(?) p.(Arg196*) - pathogenic, pathogenic (recessive) g.186445047C>T g.186727258C>T c.[586C>T];[586C>T], c.[c.586C>T];[c.586C>T] - KNG1_000010 Proband plasma presenting with a complete deficit of HK and LK, 2 more items Journal: Hayashi 1990 Journal: Adenaeuer 2022, Journal: Nazir 2019 Journal: Adenaeuer 2022, 1 more item ClinVar-VCV000000572.1 rs121918131 Germline - 0.00001061 (gnomAD) - - - Christian Drouet
-/., -?/. 2 5 c.587G>A r.(?) p.(Arg196Gln) - likely benign, likely benign (!) g.186445048G>A g.186727259G>A KNG1(NM_001102416.3):c.587G>A (p.R196Q) - KNG1_000008 VKGL data sharing initiative Nederland, 1 more item - - rs144123648 CLASSIFICATION record, Unknown - 0.0004597 - - - VKGL-NL_Utrecht, Christian Drouet
+/. 1 5 c.618T>G r.(?) p.(Cys206Trp) ACMG likely pathogenic g.186445079T>G g.186727290T>G c.[618T>G](;)[1165C>T] - KNG1_000024 1 more item Journal: Lv 2025 - - Germline - - - - - Christian Drouet
+?/. 1 5 c.654C>A r.(?) p.(Cys218*) - VUS (!) g.186445115C>A g.186727326C>A - - KNG1_000020 1 more item Journal: Dias de Castro 2024 - - Germline - - - - - Christian Drouet
+/. 1 6 c.718C>T r.(?) p.(Arg240*) - pathogenic (recessive) g.186449379C>T g.186731590C>T c.[718C>T];[1038+1G>A] - KNG1_000011 1 more item Journal: Adenaeuer 2022 ClinVar-SCV004031437.1 rs761496908 Germline - 0.000006206 - - - Christian Drouet
+/., +?/. 2 8i c.1038+1G>A r.spl p.? - pathogenic (recessive) g.186456996G>A g.186739207G>A c.[1038+1G>A](;)[1165C>T], c.[718C>T];[1038+1G>A] - KNG1_000007 2 more items Journal: Adenaeuer 2022, PubMed: Barco 2020, Journal: Barco 2020 Journal: Adenaeuer 2022 ClinVar-SCV004031440.1 rs377594184 Germline - 0.000009311 - - - Christian Drouet
+/., +?/. 2 10 c.1136T>A r.(?) p.(Met379Lys) ACMG pathogenic g.186459321T>A g.186741532T>A - - KNG1_000004 2 more items Journal: Hardy 2023, PubMed: Bork 2019 Journal: Bork 2019 Journal: Dickeson 2023 ClinVar-SCV001712268.1, ClinVar-SCV001712268 rs752411996 Germline yes 6.860E-7 - - - Christian Drouet
+/., +?/. 3 10 c.1165C>T r.(?) p.(Arg389*) ACMG pathogenic (recessive) g.186459350C>T g.186741561C>T c.[1038+1G>A];[1165C>T], c.[488delG];[1165C>T], c.[618T>G](;)[1165C>T] - KNG1_000006 3 more items Journal: Jeung 2020 Journal: Adenaeuer 2022, Journal: Lv 2025, 1 more item ClinVar-VCV001803726.1 rs752411996 Germline - 0.000006211 - - - Christian Drouet
+/. 1 10 c.1216dup r.(?) p.(His406Profs*10) - pathogenic (recessive) g.186459401dup g.186741612dup c.[1216dup];[1216dup] - KNG1_000014 A family with 1 homozygous symptomatic proband and 5 heterozygous asymptomatic carriers Journal: Shigekiyo 2007 Journal: Adenaeuer 2022 ClinVar-VCV000000575.1 rs797044430 Germline - - - - - Christian Drouet
-?/. 1 - c.1311A>C r.(?) p.(Arg437Ser) - likely benign g.186459496A>C - KNG1(NM_001102416.3):c.1311A>C (p.R437S) - KNG1_000017 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Utrecht
+/. 1 10 c.1404G>C r.(1404G>C) p.(Gln468His) ACMG pathogenic g.186459589G>C g.186741800G>C - - KNG1_000026 - Journal: Chen 2025 - - Germline/De novo (untested) yes - - - - Christian Drouet
+/. 1 10 c.1456C>T r.(?) p.(Gln486*) - pathogenic (recessive) g.186459641C>T g.186741852C>T c.[1456C>T];[1456C>T] - KNG1_000015 1 more item Journal: Yang 2020 Journal: Adenaeuer 2022 - - Germline yes - - - - Christian Drouet
+/. 1 10 c.1493del r.(?) p.(Lys498Serfs*54) - pathogenic g.186459678del g.186741889del c.[1493del];[1493del] - KNG1_000016 1 more item Journal: Krijanovski 2003 Journal: Adenaeuer 2022 ClinVar-VCV000000573.1 rs797044429 Germline - - - - - Christian Drouet
?/. 1 - c.1609_1610del r.(?) p.(Gln537Aspfs*25) - VUS g.186459794_186459795del - KNG1(NM_001102416.3):c.1609_1610del (p.(Gln537Aspfs*25)) - KNG1_000025 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
-?/., ?/. 2 - c.1643T>C r.(?) p.(Ile548Thr) - likely benign, VUS g.186459828T>C - KNG1(NM_001102416.2):c.1643T>C (p.(Ile548Thr)), KNG1(NM_001102416.3):c.1643T>C (p.I548T) - KNG1_000018 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden, VKGL-NL_Utrecht
+/. 1 10 c.1720C>G r.(?) p.(Pro574Ala) ACMG pathogenic (recessive) g.186459905C>G g.186742116C>G - - KNG1_000005 1 more item PubMed: Loules 2020 Journal: Loules 2020 ClinVar-SCV001712269.1 rs1369253342 Germline yes 0.000003181 - - - Christian Drouet
+?/. 1 17 c.1742T>C r.(?) p.(Ile581Thr) - association g.186459927T>C g.186742138T>C - - KNG1_000021 1 more item Journal: Houlihan 2010 Journal: Morange 2011 Journal: Li 2023 - rs710446 Germline - 0.416246 - - - Christian Drouet
?/. 1 - c.1744C>T r.(?) p.(Gln582*) - VUS g.186459929C>T - KNG1(NM_001102416.3):c.1744C>T (p.Q582*) - KNG1_000022 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Groningen
-?/., ./. 4 - c.*1404C>T r.(=), r.(?) p.(=) - benign, pathogenic g.186461524C>T g.186743735C>T NM_000893.4:c.1234C>T - KNG1_000001 association variant/phenotype uncertain, 1 more item Journal: Mathey 2022 Journal: Zhang 2023, PubMed: DDDS 2015, Journal: DDDS 2015 ClinVar-VCV000403021.3 rs76438938 Germline - 0.03019 (gnomAD) - - - Johan den Dunnen, Christian Drouet
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