Unique variants in the CLN6 gene

An NCL gene variant database
Information The variants shown are described using the NM_017882.2 transcript reference sequence.

65 entries on 1 page. Showing entries 1 - 65.
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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.-3677160_*871395del r.? p.? - pathogenic g.67629083_72199082del - - - MAP2K5_000011 decreased gene dosage PubMed: DDDS 2015, Journal: DDDS 2015 - - De novo - - - - - Johan den Dunnen
+/. 1 1 c.-158_(83+1_84-1){0} r.0? p.0? - pathogenic (recessive) g.(68510989_68521839)_(68522080_?)del - -158_83del - CLN6_000034 - - - - Germline - - - - - Corina-Marcela Rus
+?/. 1 - c.1_11del r.(?) p.(Met1?) ACMG likely pathogenic g.68521913_68521923del g.68229575_68229585del - - CLN6_000004 - PubMed: Trujillano 2017 - - Germline - - - - - Daniel Trujillano
-?/., ?/. 3 - c.5A>G r.(?) p.(Glu2Gly) - likely benign, VUS g.68521918T>C g.68229580T>C CLN6(NM_017882.2):c.5A>G (p.E2G), CLN6(NM_017882.3):c.5A>G (p.E2G) - CLN6_000019 classification based on frequency in 305 unrelated individuals, VKGL data sharing initiative Nederland PubMed: Le 2019 - - CLASSIFICATION record, Germline - frequency 0.015 - - - Global Variome, with Curator vacancy, VKGL-NL_Rotterdam, VKGL-NL_Groningen
?/. 1 - c.11C>T r.(?) p.(Thr4Met) - VUS g.68521912G>A g.68229574G>A CLN6(NM_017882.3):c.11C>T (p.T4M) - CLN6_000029 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Groningen
?/. 1 - c.13C>T r.(?) p.(Arg5Trp) - VUS g.68521910G>A - CLN6(NM_017882.3):c.13C>T (p.R5W) - CLN6_000059 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Utrecht
-?/. 3 - c.34G>A r.(?) p.(Ala12Thr) - likely benign g.68521889C>T g.68229551C>T CLN6(NM_017882.2):c.34G>A (p.(Ala12Thr)), CLN6(NM_017882.3):c.34G>A (p.A12T) - CLN6_000018 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden, VKGL-NL_Groningen, VKGL-NL_Utrecht
-?/. 1 - c.42C>A r.(?) p.(=) - likely benign g.68521881G>T - CLN6(NM_017882.3):c.42C>A (p.G14=) - CLN6_000061 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Utrecht
-?/. 1 - c.49G>A r.(?) p.(Gly17Ser) - likely benign g.68521874C>T g.68229536C>T CLN6(NM_017882.2):c.49G>A (p.G17S) - CLN6_000028 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-?/. 1 - c.53C>T r.(?) p.(Ala18Val) - likely benign g.68521870G>A g.68229532G>A CLN6(NM_017882.2):c.53C>T (p.(Ala18Val)) - CLN6_000026 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
-?/. 1 - c.64G>T r.(?) p.(Ala22Ser) - likely benign g.68521859C>A g.68229521C>A CLN6(NM_017882.2):c.64G>T (p.(Ala22Ser)) - CLN6_000024 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
+?/. 1 - c.83G>A r.(?) p.(Arg28Lys) - likely pathogenic g.68521840C>T g.68229502C>T - - CLN6_000021 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
?/. 1 - c.100G>A r.(?) p.(Ala34Thr) - VUS g.68510972C>T g.68218634C>T CLN6(NM_017882.2):c.100G>A (p.A34T) - CLN6_000023 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-?/. 1 - c.103G>A r.(?) p.(Asp35Asn) - likely benign g.68510969C>T - CLN6(NM_017882.2):c.103G>A (p.D35N) - CLN6_000058 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+?/. 1 - c.144G>A r.(?) p.(Trp48*) - likely pathogenic (recessive) g.68510928C>T - - - CLN6_000033 - - - - Germline - - - - - Corina-Marcela Rus
+?/. 1 - c.198+1G>A r.spl p.(?) - likely pathogenic g.68510873C>T g.68218535C>T c.198+1G>A - CLN6_000055 homozygous PubMed: Jilani 2019 - - Germline ? - - - - LOVD
-/. 1 - c.199-104dup r.(=) p.(=) - benign g.68506835dup g.68214497dup CLN6(NM_017882.3):c.199-104dupG - CLN6_000022 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Utrecht
-?/. 2 - c.199-5C>T r.spl? p.? - likely benign g.68506731G>A - CLN6(NM_017882.2):c.199-5C>T, CLN6(NM_017882.3):c.199-5C>T - CLN6_000044 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam, VKGL-NL_Groningen
-?/. 1 - c.213C>G r.(?) p.(Leu71=) - likely benign g.68506712G>C g.68214374G>C CLN6(NM_017882.2):c.213C>G (p.L71=) - CLN6_000016 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
-/., -?/. 3 - c.214G>C r.(?) p.(Glu72Gln) - benign, likely benign g.68506711C>G g.68214373C>G CLN6(NM_017882.2):c.214G>C (p.E72Q, p.(Glu72Gln)), CLN6(NM_017882.3):c.214G>C (p.E72Q) - CLN6_000015 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden, VKGL-NL_Rotterdam, VKGL-NL_Utrecht
?/. 1 - c.241G>T r.(?) p.(Val81Phe) - VUS g.68506684C>A g.68214346C>A CLN6(NM_017882.3):c.241G>T (p.V81F) - CLN6_000014 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Utrecht
+?/. 1 - c.244G>T r.(?) p.(Gly82Trp) - likely pathogenic g.68506681C>A g.68214343C>A 3 compound heterozygous AR CLN6 c.244G>T p.(G82W) c.892G>A (Sun et al., 2018) p.(E298K) - CLN6_000054 heterozygous PubMed: Surl 2020 - - Germline yes - - - - LOVD
+/. 1 3 c.252C>G r.(?) p.(Tyr84*) - pathogenic g.68506673G>C g.68214335G>C Y84X - CLN6_000001 - PubMed: Faruq 2014, Journal: Faruq 2014 - - Germline yes - - - - Johan den Dunnen
+?/. 4 3 c.257A>G r.(?) p.(His86Arg) ACMG likely pathogenic (recessive) g.68506668T>C - - - CLN6_000035 - - - - Germline - - - - - Corina-Marcela Rus
?/. 2 - c.259A>G r.(?) p.(Met87Val) - VUS g.68506666T>C g.68214328T>C CLN6(NM_017882.2):c.259A>G (p.M87V) - CLN6_000013 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam, VKGL-NL_Nijmegen
+?/. 1 - c.296A>G r.(?) p.(Lys99Arg) ACMG likely pathogenic g.68506629T>C g.68214291T>C c.296A>G, p.Lys99Arg - CLN6_000053 Homozygous PubMed: Dozieres-Puyravel 2020 - - Germline ? - - - - LOVD
-/., -?/. 2 - c.298-6C>T r.(=) p.(=) - benign, likely benign g.68504207G>A g.68211869G>A CLN6(NM_017882.3):c.298-6C>T - CLN6_000012 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Utrecht, VKGL-NL_Nijmegen
?/. 1 - c.304G>A r.(?) p.(Glu102Lys) - VUS g.68504195C>T g.68211857C>T - - CLN6_000032 2 heterozygous, no homozygous; Clinindb (India) PubMed: Narang 2020, Journal: Narang 2020 - rs796052352 Germline - 2/2787 individuals - - - Mohammed Faruq
?/. 1 - c.307C>T r.(?) p.(Arg103Trp) - VUS g.68504192G>A g.68211854G>A - - CLN6_000031 3 heterozygous, no homozygous; Clinindb (India) PubMed: Narang 2020, Journal: Narang 2020 - rs201095412 Germline - 3/2748 individuals - - - Mohammed Faruq
?/. 1 - c.308G>A r.(?) p.(Arg103Gln) - VUS g.68504191C>T g.68211853C>T - - CLN6_000030 11 heterozygous, no homozygous; Clinindb (India) PubMed: Narang 2020, Journal: Narang 2020 - rs154774634 Germline - 11/2715 individuals - - - Mohammed Faruq
?/. 1 - c.316C>T r.(?) p.(Arg106Cys) - VUS g.68504183G>A g.68211845G>A - - CLN6_000020 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
-?/. 1 - c.339G>A r.(?) p.(Thr113=) - likely benign g.68504160C>T g.68211822C>T CLN6(NM_017882.3):c.339G>A (p.T113=) - CLN6_000011 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Utrecht
+/. 1 - c.395_396del r.(?) p.(Ser132Cysfs*18) - pathogenic g.68504105_68504106del - CLN6(NM_017882.2):c.395_396delCT (p.S132Cfs*18) - CLN6_000043 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+?/. 1 - c.396dup r.(?) (p.Val133fs) - likely pathogenic g.68504103dup g.68211765dup c.396dupT, (p.Val133fs) - CLN6_000056 homozygous PubMed: Kozina 2020 - - Germline/De novo (untested) ? - - - - LOVD
+?/. 2 4 c.397_398del r.(?) p.(Val133Glnfs*17) ACMG likely pathogenic, likely pathogenic (recessive) g.68504101_68504102del - - - CLN6_000036 - - - - Germline - - - - - Corina-Marcela Rus
+/. 1 - c.407G>A r.(?) p.(Arg136His) - pathogenic g.68504092C>T g.68211754C>T NM_017882: c.G407A; p.R136H - CLN6_000006 - PubMed: Karaca 2015 - - Germline - - - - - Johan den Dunnen
+/. 1 - c.424dup r.(?) p.(Tyr142Leufs*9) - pathogenic g.68504075dup - - - CLN6_000057 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
+/. 1 - c.443T>A r.(?) p.(Val148Asp) - pathogenic (recessive) g.68504056A>T g.68211718A>T - - CLN6_000040 - PubMed: Zhu 2015 - - Germline - - - - - Johan den Dunnen
+?/. 3 - c.445C>T r.(?) p.(Arg149Cys) - likely pathogenic g.68504054G>A g.68211716G>A c.445C>T, p.(Arg149Cys) - CLN6_000052 homozygous PubMed: Jilani 2019 - - Germline yes - - - - LOVD
?/. 1 - c.446G>A r.(?) p.(Arg149His) - VUS g.68504053C>T g.68211715C>T CLN6(NM_017882.2):c.446G>A (p.R149H) - CLN6_000027 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
?/. 1 - c.472A>C r.(?) p.(Lys158Gln) - VUS g.68504027T>G - CLN6(NM_017882.2):c.472A>C (p.K158Q) - CLN6_000049 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+?/. 1 4 c.476C>T r.(?) p.(Pro159Leu) - likely pathogenic g.68504023G>A g.68211685G>A - - CLN6_000048 - PubMed: Ganapathy 2019 - - Germline - - - - - Johan den Dunnen
-?/. 1 - c.477G>A r.(?) p.(Pro159=) - likely benign g.68504022C>T - CLN6(NM_017882.2):c.477G>A (p.P159=) - CLN6_000042 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+?/. 1 4i c.486+2T>A r.spl p.? ACMG likely pathogenic (recessive) g.68504011A>T - - - CLN6_000037 - - - - Germline - - - - - Corina-Marcela Rus
+?/. 1 4i c.486+2T>C r.spl p.? ACMG likely pathogenic (recessive) g.68504011A>G - - - CLN6_000039 - - - - Germline - - - - - Corina-Marcela Rus
-?/. 2 - c.486+8C>T r.(=) p.(=) - likely benign g.68504005G>A g.68211667G>A CLN6(NM_017882.3):c.486+8C>T - CLN6_000010 4 heterozygous, no homozygous; Clinindb (India), VKGL data sharing initiative Nederland PubMed: Narang 2020, Journal: Narang 2020 - rs149692285 CLASSIFICATION record, Germline - 4/2795 individuals - - - VKGL-NL_Utrecht, Mohammed Faruq
+?/. 1 - c.486+28T>C r.spl p.(?) ACMG likely pathogenic g.68503985A>G - - - CLN6_000045 1 more item PubMed: Courage 2021, Journal: Courage 2021 - - Germline - - - - - Carolina Courage
-?/. 1 - c.487-13G>A r.(=) p.(=) - likely benign g.68503669C>T - CLN6(NM_017882.3):c.487-13G>A - CLN6_000041 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Utrecht
-?/. 1 - c.487-8T>C r.(=) p.(=) - likely benign g.68503664A>G g.68211326A>G CLN6(NM_017882.3):c.487-8T>C - CLN6_000009 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Utrecht
+?/. 1 5i c.543-2A>G r.spl p.? ACMG likely pathogenic (recessive) g.68502099T>C - - - CLN6_000038 - - - - Germline - - - - - Corina-Marcela Rus
?/. 1 - c.653G>A r.(?) p.(Gly218Asp) - VUS g.68501987C>T - - - CALML4_000006 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
+?/. 1 - c.662A>C r.(?) p.(Tyr221Ser) ACMG likely pathogenic g.68501978T>G g.68209640T>G - - CLN6_000003 - PubMed: Trujillano 2017 - - Germline - - - - - Daniel Trujillano
?/. 1 7 c.679G>A r.(?) p.(Glu227Lys) - VUS g.68500735C>T g.68208397C>T - - CLN6_000047 - PubMed: Ganapathy 2019 ClinVar-RCV000625854.1 rs746753722 Germline - - - - - Johan den Dunnen
?/. 1 - c.749G>A r.(?) p.(Arg250His) - VUS g.68500665C>T - CLN6(NM_017882.3):c.749G>A (p.(Arg250His)) - CALML4_000007 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Leiden
?/. 1 - c.755G>C r.(?) p.(Arg252Pro) - VUS g.68500659C>G g.68208321C>G - - CLN6_000060 - PubMed: Fernandez-Marmiesse 2014 - - Germline - - - - - Johan den Dunnen
-?/. 1 - c.759C>G r.(?) p.(Leu253=) - likely benign g.68500655G>C - CLN6(NM_017882.2):c.759C>G (p.L253=) - CALML4_000005 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
?/. 1 - c.765_785del r.(?) p.(Asp256_Leu262del) - VUS g.68500639_68500659del g.68208301_68208321del - - CALML4_000003 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Nijmegen
+?/. 1 7 c.766G>A r.(?) p.(Asp256Asn) - likely pathogenic g.68500648C>T g.68208310C>T D256E - CLN6_000002 - PubMed: Faruq 2014, Journal: Faruq 2014 - - Germline yes - - - - Johan den Dunnen
-/. 1 - c.769A>G r.(?) p.(Ser257Gly) - benign g.68500645T>C g.68208307T>C CLN6(NM_017882.2):c.769A>G (p.S257G) - CLN6_000008 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+?/. 1 7 c.775G>C r.(?) p.(Gly259Arg) - likely pathogenic g.68500639C>G g.68208301C>G - - CLN6_000046 - PubMed: Ganapathy 2019 - - Germline - - - - - Johan den Dunnen
+?/. 1 - c.776G>A r.(?) p.(Gly259Asp) ACMG likely pathogenic g.68500638C>T g.68208300C>T c.776G>A, p.Gly259Asp - CLN6_000051 Homozygous PubMed: Dozieres-Puyravel 2020 - - Germline ? - - - - LOVD
+/., +?/., ?/. 5 - c.794_796del r.(?) p.(Ser265del) ACMG likely pathogenic, pathogenic, pathogenic (recessive), VUS g.68500623_68500625del g.68208285_68208287del - - CLN6_000005 VKGL data sharing initiative Nederland PubMed: Fernandez-Marmiesse 2014, PubMed: Froukh 2020, PubMed: Trujillano 2017 - - CLASSIFICATION record, Germline, Unknown - - - - - Johan den Dunnen, Daniel Trujillano, IMGAG, VKGL-NL_Nijmegen
?/. 1 - c.832G>A r.(?) p.(Ala278Thr) - VUS g.68500582C>T - CLN6(NM_017882.2):c.832G>A (p.A278T) - CALML4_000004 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam
+?/. 1 - c.892G>A r.(?) p.(Glu298Lys) - likely pathogenic g.68500522C>T g.68208184C>T 3 compound heterozygous AR CLN6 c.244G>T p.(G82W) c.892G>A (Sun et al., 2018) p.(E298K) - CLN6_000050 heterozygous PubMed: Surl 2020 - - Germline yes - - - - LOVD
-?/. 2 - c.923G>C r.(?) p.(Ser308Thr) - likely benign g.68500491C>G g.68208153C>G CLN6(NM_017882.2):c.923G>C (p.S308T), CLN6(NM_017882.3):c.923G>C (p.S308T) - CLN6_000007 VKGL data sharing initiative Nederland - - - CLASSIFICATION record - - - - - VKGL-NL_Rotterdam, VKGL-NL_Utrecht
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