Legend
Please note that a short description of a certain column can be displayed when you move your mouse cursor over the column's header and hold it still. Below, a more detailed description is shown per column.
Effect : The variant's effect on the function of the gene/protein, displayed in the format 'R/C'. R is the value reported by the source (publication, submitter) and this classification may vary between records. C is the value concluded by the curator. Note that in some database the curator uses Summary records to give details on the classification of the variant.Values used: '+' indicating the variant affects function, '+?' probably affects function, '-' does not affect function, '-?' probably does not affect function, '?' effect unknown, '.' effect was not classified.
Exon : number of exon/intron containing variant; 2 = exon 2, 12i = intron 12, 2i_7i = from intron 2 to intron 7, 8i_9 = intron 8/exon 9 boundary, _1 = 5' to exon 1, 18_ = 3' of exon 18, _1_18_ = encompassing the entire 18-exon gene
DNA change (cDNA) : description of variant at DNA level, based on a coding DNA reference sequence (following HGVS recommendations); e.g. c.123C>T, c.123_145del, c.123_126dup. For deletions/duplications extending beyond the reference transcript resp. {0}/{2} is used to replace del/dup. Extent of the deletion/duplication should be specified using the genomic description (g.). "-" indicates the variant described on genomic level does not affect the coding DNA reference sequence.
RNA change : description of variant at RNA level (following HGVS recommendations).
r.123c>u
r.? = unknown
r.(?) = RNA not analysed but probably transcribed copy of DNA variant
r.spl? = RNA not analysed but variant probably affects splicing
r.(spl?) = RNA not analysed but variant may affect splicing
r.0? = change expected to abolish transcription
Protein : description of variant at protein level (following HGVS recommendations).
p.(Arg345Pro) = change predicted from DNA (RNA not analysed)
p.Arg345Pro = change derived from RNA analysis
p.? = unknown effect
p.0? = probably no protein produced
Allele : On which allele is the variant located? Does not necessarily imply inheritance! 'Paternal' (confirmed or inferred), 'Maternal' (confirmed or inferred), 'Parent #1' or #2 for compound heterozygosity without having screened the parents, 'Unknown' for heterozygosity without having screened the parents, 'Both' for homozygozity.
Classification method : The method used for the clinical classification of this variant.
All options:
ACMG
ACGS
EAHAD-CFDB
ENIGMA
IARC
InSiGHT
kConFab
other
Clinical classification : Clinical classification of variant, preferably based on standardised criteria (e.g. ACMG), directed on the clinical consequences as published/submitted, indicated using an enriched system including inheritance: e.g. pathogenic, pathogenic (dominant), pathogenic (recessive), pathogenic (!), pathogenic (maternal), pathogenic (paternal). Standard inheritance is covered by dominant/recessive, imprinting by maternal/paternal. A '!' warns for exceptional circumstances to be explained in the 'Remarks' field (low penetrance, variants pathogenic in heterozygous state only, hypomorphic/hypermorphic variants, protective variants, etc.). Non-disease consequences (e.g. drug metabolism (pharmacogenetics), risk factor, blood group, tasting bitter) are indicated using additions to the benign classification; benign (dominant), benign (recessive), benign (!), etc. The value 'association' is used for variants associated with a phenotype and 'NA' for variants from in vitro/in silico records. NOTE: classification may differ from the opinion of the curator as given in a variant SUMMARY-record or the 'Functional effect concluded'). NOTE: pathogenic/likely pathogenic should go together with "variant (probably) affects function" In ClassFunctional.
All options:
pathogenic
pathogenic (dominant)
pathogenic (recessive)
pathogenic (!)
pathogenic (maternal)
pathogenic (paternal)
likely pathogenic
likely pathogenic (dominant)
likely pathogenic (recessive)
likely pathogenic (!)
likely pathogenic (maternal)
likely pathogenic (paternal)
VUS
VUS (!)
likely benign
likely benign (dominant)
likely benign (recessive)
likely benign (!)
likely benign (maternal)
likely benign (paternal)
benign
benign (dominant)
benign (recessive)
benign (!)
benign (maternal)
benign (paternal)
conflicting
association
NA
DNA change (genomic) (hg19) : HGVS description of variant at DNA level, based on the genomic (chromosomal) DNA reference sequence; e.g. g.12345678C>T, g.12345679del, g.12345678_12345890dup
DNA change (hg38) : HGVS description of variant at DNA level, based on the hg38 genomic (chromosomal) eference sequence; e.g. g.12345678C>T, g.12345679del, g.12345678_12345890dup
Published as : listed only when different from "DNA change"; variant as reported originally (e.g. 521delT). Variants seen in animal models, tested in vitro, predicted from RNA analysis, etc. are described between brackets like c.(456C>G)
ISCN : description of the variant according to ISCN nomenclature
DB-ID : database ID of variant, grouping multiple observations of the same variant together, starting with the HGNC gene symbol, followed by an underscore (_) and a six digit number (e.g. DMD_012345). _000000 is used for variants where DNA was not analysed (change predicted from RNA analysis), variants seen in animal models or variants not seen in humans but functionally tested in vitro
Variant remarks : remarks regarding variant described, e.g. germline mosaicism in mother, 345 kb deletion, muscle RNA analysed, not in 200 control chromosomes tested, on founder haplotype, etc.
Reference : publication describing the variant submitted, incl. links to OMIM, PubMed or other source, e.g. "den Dunnen ASHG2003 P2346"
ClinVar ID : ID of variant in ClinVar database
dbSNP ID : the dbSNP ID
Origin : Origin of variant/record: Germline = in all cells, De novo = in all cells, but not in either parent, Germline/De novo (untested) = in all cells, parents not tested (use only when De novo is likely, e.g. isolated/sporadic cases with dominant disease), Somatic = present in a subset of cells, but not in either parent, Uniparental disomy = from parental disomy (maternal or paternal), CLASSIFICATION record = submitter only sharing variant classification (note another report may share Individual data), SUMMARY record = master summary record from curator (may link to another database), In vitro (cloned) = data resulting from in vitro functional assays, animal model = data from animal model, Artefact = false positive variant call, DUPLICATE record = variant already described on another chromosome (e.g. unbalanced translocation, duplicating transposition, 2nd fusion transcript, etc.)
All options:
Germline
De novo
Germline/De novo (untested)
Somatic
Uniparental disomy
Uniparental disomy, maternal allele
Uniparental disomy, paternal allele
CLASSIFICATION record
SUMMARY record
In vitro (cloned)
In silico
animal model
Artefact
DUPLICATE record
Unknown
Not applicable
Segregation : Indicates whether the variant segregates with the phenotype (yes), does not segregate with the phenotype (no) or segregation is unknown (?)
All options:
? = unknown
yes = segregates with phenotype
no = does not segregate with phenotype
- = not applicable
Frequency : frequency in which the variant was found; e.g 5/760 chromosomes (in 5 of 760 chromosomes tested), 1/33 patients (in 1 of 33 patients analysed in study), 0.05 controls (in 5% of control cases tested)
Re-site : restriction enzyme recognition site created (+) or destroyed (-); e.g. BglII+;BamHI-
VIP : variant VIP-status was requested for matchmaking - need collaboration(s) to crack the case - please contact the submitter/curator.
NOTE: to get VIP status ask the curator.
Methylation : result of methylation test; GOM (gain of methylation), LOM (loss of methylation), 30% (30% methylated). NOTE: when several tests were done mention the method as well (e.g. MS-PCR 75%)
Effect
Exon
DNA 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_2
c.-906_130delinsCGCATCCAAGA
r.0?
p.0?
-
pathogenic
g.4805975_4807264delinsTCTTGGATGCG
g.4902680_4903969delinsTCTTGGATGCG
-
-
CHRNE_000081
1.29 Kb deletion promoter-exon 2; fusion transcripts containing 5' GP1BA sequences
PubMed: Abicht 2002
-
-
Germline
-
-
-
-
-
Angela Abicht
+/.
1_2
c.-906_130delinsCGCATCCAAGA
r.?
p.?
-
pathogenic
g.4805975_4807264delinsTCTTGGATGCG
g.4902680_4903969delinsTCTTGGATGCG
-
-
CHRNE_000081
1.29 Kb deletion promoter-exon 2
PubMed: Abicht 2002
-
-
Germline
-
-
-
-
-
Angela Abicht
+/.
1
c.-96C>T
r.(?)
p.(=)
-
pathogenic
g.4806454G>A
g.4903159G>A
-156C>T
-
CHRNE_000071
affects promoter N-box
PubMed: Nichols 1999
-
-
Germline
-
-
MspI-
-
-
Johan den Dunnen
+/.
1
c.-96C>T
r.(?)
p.(=)
-
pathogenic
g.4806454G>A
g.4903159G>A
-156C>T
-
CHRNE_000071
affects promoter N-box
PubMed: Nichols 1999
-
-
Germline
-
-
MspI-
-
-
Johan den Dunnen
+/.
1
c.-96C>T
r.(?)
p.(=)
-
pathogenic
g.4806454G>A
g.4903159G>A
-156C>T
-
CHRNE_000071
affects promoter N-box, no CHRNE mRNA expression
PubMed: Nichols 1999
-
-
Germline
-
-
MspI-
-
-
Johan den Dunnen
+/.
1
c.-96C>T
r.(?)
p.(=)
-
pathogenic
g.4806454G>A
g.4903159G>A
-156C>T
-
CHRNE_000071
affects promoter N-box, no CHRNE mRNA expression
PubMed: Nichols 1999
-
-
Germline
-
-
MspI-
-
-
Johan den Dunnen
+/.
1
c.-96C>T
r.(?)
p.(=)
-
pathogenic
g.4806454G>A
g.4903159G>A
-156C>T
-
CHRNE_000071
affects promoter N-box
PubMed: Nichols 1999
-
-
Germline
-
-
MspI-
-
-
Johan den Dunnen
+?/.
-
c.-96C>T
r.(?)
p.(?)
ACMG
likely pathogenic
g.4806454G>A
g.4903159G>A
-
-
CHRNE_000071
ACMG: PS3,PM2
-
-
rs748144899
Germline
-
-
-
-
-
Andreas Laner
+/.
1
c.-95G>A
r.(?)
p.(=)
-
pathogenic
g.4806453C>T
g.4903158C>T
-155G>A
-
CHRNE_000074
not in 182 CMS/200 control chromosomes; affects Ets binding site promoter mouse (Duclert 1996)
PubMed: Ohno 1999
-
-
Germline
-
-
MspI-
-
-
Johan den Dunnen
+/.
1
c.-95G>A
r.(?)
p.(=)
-
pathogenic
g.4806453C>T
g.4903158C>T
-155G>A
-
CHRNE_000074
not in 182 CMS/200 control chromosomes; affects Ets binding site promoter mouse (Duclert 1996)
PubMed: Ohno 1999
-
-
Germline
-
-
MspI-
-
-
Johan den Dunnen
+/.
1
c.-95G>A
r.(?)
p.(=)
-
pathogenic
g.4806453C>T
g.4903158C>T
-155G>A
-
CHRNE_000074
not in 182 CMS/200 control chromosomes; affects Ets binding site promoter mouse (Duclert 1996)
PubMed: Ohno 1999
-
-
Germline
-
-
MspI-
-
-
Johan den Dunnen
+/.
1
c.-95G>A
r.(?)
p.(=)
-
pathogenic
g.4806453C>T
g.4903158C>T
-155G>A
-
CHRNE_000074
not in 182 CMS/200 control chromosomes; affects Ets binding site promoter mouse (Duclert 1996)
PubMed: Ohno 1999
-
-
Germline
-
-
MspI-
-
-
Johan den Dunnen
+/.
1
c.-95G>A
r.(?)
p.(=)
-
pathogenic
g.4806453C>T
g.4903158C>T
-155G>A
-
CHRNE_000074
not in 182 CMS/200 control chromosomes; affects Ets binding site promoter mouse (Duclert 1996)
PubMed: Ohno 1999
-
-
Germline
-
-
MspI-
-
-
Johan den Dunnen
+/.
1
c.-95G>A
r.(?)
p.(=)
-
pathogenic
g.4806453C>T
g.4903158C>T
-
-
CHRNE_000074
-
-
-
-
Germline
-
-
-
-
-
Angela Abicht
+?/.
-
c.-95G>A
r.(?)
p.?
ACMG
likely pathogenic
g.4806453C>T
g.4903158C>T
-
-
CHRNE_000074
ACMG PS4, PM2
PubMed: Molaei 2025
SCV006074789.1
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
-
c.-94G>A
r.(=)
p.(=)
-
pathogenic
g.4806452C>T
g.4903157C>T
-
-
CHRNE_000136
promoter mutation published as -154G>A in N-bo
-
-
-
Germline
-
-
-
-
-
Andreas Laner
+/.
1
c.-94G>A
r.(?)
p.(=)
-
NA
g.4806452C>T
g.4903157C>T
-154G>A
-
CHRNE_000080
reporter gene construct rat soleus muscle not induced by agrin
PubMed: Abicht 2002
-
-
In vitro (cloned)
-
-
-
-
-
Angela Abicht
+/.
1
c.-94G>A
r.(?)
p.(=)
-
pathogenic
g.4806452C>T
g.4903157C>T
-154G>A
-
CHRNE_000080
not in 84 CMS/104 control chromosomes; severely reduced expression
PubMed: Abicht 2002
-
-
Germline
-
-
MspI-
-
-
Angela Abicht
+/.
1
c.-94G>A
r.(?)
p.(=)
-
pathogenic
g.4806452C>T
g.4903157C>T
-154G>A
-
CHRNE_000080
-
PubMed: Abicht 2002
-
-
Germline
-
-
MspI-
-
-
Angela Abicht
+/.
1
c.-94G>A
r.(?)
p.(=)
-
pathogenic
g.4806452C>T
g.4903157C>T
-
-
CHRNE_000080
-
-
-
-
Germline
-
-
MspI-
-
-
Angela Abicht
+/.
1
c.-94G>A
r.(?)
p.(=)
-
pathogenic
g.4806452C>T
g.4903157C>T
-
-
CHRNE_000080
-
-
-
-
Germline
-
-
MspI-
-
-
Angela Abicht
-/.
1
c.-60C>T
r.(?)
p.(=)
-
benign
g.4806418G>A
g.4903123G>A
C362T (P121L)
-
CHRNE_000079
-
PubMed: Ohno 1996
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-/.
1
c.-60C>T
r.(?)
p.(=)
-
benign
g.4806418G>A
g.4903123G>A
C362T (P121L)
-
CHRNE_000079
-
PubMed: Ohno 1996
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
8
c.917G<T
r.spl?
p.(Arg306Met)
-
pathogenic
g.?
-
-
-
CHRNE_000110
-
-
-
-
Germline
-
-
-
-
-
Angela Abicht
+/.
8
c.917G<T
r.spl?
p.(Arg306Met)
-
pathogenic
g.?
-
-
-
CHRNE_000110
-
-
-
-
Germline
-
-
-
-
-
Angela Abicht
+/.
8
c.917G<T
r.spl?
p.(Arg306Met)
-
pathogenic
g.?
-
-
-
CHRNE_000110
-
-
-
-
Germline
-
-
-
-
-
Angela Abicht
+/.
8
c.917G<T
r.spl?
p.(Arg306Met)
-
pathogenic
g.?
-
-
-
CHRNE_000110
-
-
-
-
Germline
-
-
-
-
-
Angela Abicht
?/.
1_12
c.?
r.?
p.?
-
VUS
g.?
-
-
-
MYH2_000008
unknown variant 2nd chromosome
-
-
-
Germline
-
-
-
-
-
Angela Abicht
?/.
1_12
c.?
r.?
p.?
-
VUS
g.?
-
-
-
MYH2_000008
unknown variant 2nd chromosome
-
-
-
Germline
-
-
-
-
-
Angela Abicht
?/.
1_12
c.?
r.?
p.?
-
VUS
g.?
-
-
-
MYH2_000008
unknown variant 2nd chromosome
-
-
-
Germline
-
-
-
-
-
Angela Abicht
+?/.
-
c.?
r.?
p.?
-
likely pathogenic
g.?
g.?
exon 2-8 deletion
-
MYH2_000008
-
PubMed: Liu 2026
-
-
Germline
-
1/7496 chromosomes
-
-
-
Johan den Dunnen
-?/.
-
c.6A>G
r.(?)
p.(Ala2=)
-
likely benign
g.4806353T>C
-
CHRNE(NM_000080.4):c.6A>G (p.A2=)
-
C17orf107_000012
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
?/.
-
c.16C>G
r.(?)
p.(Leu6Val)
-
VUS
g.4806343G>C
g.4903048G>C
-
-
CHRNE_000135
alamut: benign
-
-
-
Germline
-
-
-
-
-
Andreas Laner
+/.
1
c.23T>A
r.(?)
p.(Val8Asp)
-
pathogenic
g.4806336A>T
g.4903041A>T
V-13D
-
CHRNE_000025
-
PubMed: Middleton 1999
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
c.23T>A
r.(?)
p.(Val8Asp)
-
pathogenic
g.4806336A>T
g.4903041A>T
V-13D
-
CHRNE_000025
-
PubMed: Middleton 1999
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+?/.
-
c.26T>C
r.(?)
p.(Leu9Pro)
ACMG
likely pathogenic
g.4806333A>G
g.4903038A>G
-
-
CHRNE_000191
ACMG PM2, PP2, PP3_mod, PP4,
PubMed: Molaei 2025
SCV006074788.1
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
c.26T>G
r.(?)
p.(Leu9Arg)
-
pathogenic
g.4806333A>C
g.4903038A>C
-
-
CHRNE_000078
-
-
-
-
Germline
-
-
-
-
-
Angela Abicht
+/.
1
c.26T>G
r.(?)
p.(Leu9Arg)
-
pathogenic
g.4806333A>C
g.4903038A>C
-
-
CHRNE_000078
-
-
-
-
Germline
-
-
-
-
-
Angela Abicht
+/.
1
c.37G>A
r.(?)
p.Gly13Arg
-
NA
g.4806322C>T
g.4903027C>T
-
-
CHRNE_000005
expression cloning human cDNA HEK293 cells; reduced expression CHRNE, 0.10 assembly with CHRNA
PubMed: Ohno 1996
-
-
In vitro (cloned)
-
-
-
-
-
Johan den Dunnen
+/.
1
c.37G>A
r.(?)
p.(Gly13Arg)
-
pathogenic
g.4806322C>T
g.4903027C>T
G-24A (G-8R)
-
CHRNE_000005
not in 84 CMS/200 control chromosomes; residue not conserved
PubMed: Ohno 1996
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
c.37G>A
r.(?)
p.(Gly13Arg)
-
pathogenic
g.4806322C>T
g.4903027C>T
G-24A (G-8R)
-
CHRNE_000005
-
PubMed: Ohno 1996
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
c.37G>A
r.(?)
p.(Gly13Arg)
-
pathogenic
g.4806322C>T
g.4903027C>T
G-24A (G-8R)
-
CHRNE_000005
residue not conserved
PubMed: Ohno 1996
-
-
Germline
-
-
-
-
-
Johan den Dunnen
?/.
-
c.37G>A
r.(?)
p.(Gly13Arg)
-
VUS
g.4806322C>T
g.4903027C>T
-
-
CHRNE_000005
2 heterozygous, no homozygous; Clinindb (India)
PubMed: Narang 2020 , Journal: Narang 2020
-
rs372635387
Germline
-
2/2765 individuals
-
-
-
Mohammed Faruq
+?/.
-
c.37G>A
r.(?)
p.(Gly13Arg)
-
likely pathogenic
g.4806322C>T
-
CHRNE(NM_000080.4):c.37G>A (p.(Gly13Arg))
-
CHRNE_000005
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
-/.
-
c.45C>T
r.(?)
p.(Leu15=)
-
benign
g.4806314G>A
g.4903019G>A
CHRNE(NM_000080.3):c.45C>T (p.(Leu15=))
-
CHRNE_000164
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
-?/.
-
c.45C>T
r.(?)
p.(Leu15=)
-
likely benign
g.4806314G>A
-
CHRNE(NM_000080.3):c.45C>T (p.(Leu15=))
-
CHRNE_000164
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
?/.
-
c.47-8dup
r.(=)
p.(=)
-
VUS
g.4806069dup
-
CHRNE(NM_000080.4):c.47-8dup
-
C17orf107_000035
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
-/.
-
c.53G>T
r.(?)
p.(Gly18Val)
-
benign
g.4806052C>A
g.4902757C>A
CHRNE(NM_000080.3):c.53G>T (p.(Gly18Val))
-
C17orf107_000002
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
-/.
2
c.53G>T
r.53g>u
p.Gly18Val
-
benign
g.4806052C>A
g.4902757C>A
G-8T (G-3V)
-
CHRNE_000092
-
PubMed: Engel 1996
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-/.
2
c.53G>T
r.(?)
p.(Gly18Val)
-
benign
g.4806052C>A
g.4902757C>A
G-8T (G-3V)
-
CHRNE_000092
-
PubMed: Ohno 1996
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-/.
2
c.53G>T
r.(?)
p.(Gly18Val)
-
benign
g.4806052C>A
g.4902757C>A
G-8T (G-3V)
-
CHRNE_000092
-
PubMed: Ohno 1996
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-/.
2
c.53G>T
r.53g>u
p.Gly18Val
-
benign
g.4806052C>A
g.4902757C>A
G-8T (G-3V)
-
CHRNE_000092
control chromosomes
PubMed: Engel 1996
-
-
Germline
-
6/252
-
-
-
Johan den Dunnen
-?/.
-
c.53G>T
r.(?)
p.(Gly18Val)
-
likely benign
g.4806052C>A
g.4902757C>A
CHRNE(NM_000080.3):c.53G>T (p.(Gly18Val))
-
C17orf107_000002
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
-?/.
-
c.53G>T
r.(?)
p.(Gly18Val)
-
likely benign
g.4806052C>A
g.4902757C>A
-
-
C17orf107_000002
47 heterozygous, no homozygous; Clinindb (India)
PubMed: Narang 2020 , Journal: Narang 2020
-
rs4790235
Germline
-
47/2795 individuals
-
-
-
Mohammed Faruq
?/.
-
c.70G>A
r.(?)
p.(Glu24Lys)
-
VUS
g.4806035C>T
g.4902740C>T
-
-
CHRNE_000134
path.
-
-
-
Germline
-
-
-
-
-
Andreas Laner
+/.
2
c.92T>C
r.(?)
p.(Leu31Pro)
-
pathogenic
g.4806013A>G
g.4902718A>G
-
-
CHRNE_000093
-
-
-
-
Germline
-
-
-
-
-
Angela Abicht
+/.
2
c.92T>C
r.(?)
p.(Leu31Pro)
-
pathogenic
g.4806013A>G
g.4902718A>G
-
-
CHRNE_000093
-
-
-
-
Germline
-
-
-
-
-
Angela Abicht
+/.
2
c.103T>C
r.(?)
p.(Tyr35His)
-
pathogenic
g.4806002A>G
g.4902707A>G
Y15H
-
CHRNE_000037
-
PubMed: Ealing 2002
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
-
c.103T>C
r.(?)
p.(Tyr35His)
-
pathogenic
g.4806002A>G
-
CHRNE(NM_000080.3):c.103T>C (p.(Tyr35His))
-
CHRNE_000037
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+/.
2
c.105T>A
r.(?)
p.(Tyr35*)
-
pathogenic
g.4806000A>T
g.4902705A>T
Y15X
-
CHRNE_000034
-
PubMed: Croxen 2002
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
2
c.105T>A
r.(?)
p.(Tyr35*)
-
pathogenic
g.4806000A>T
g.4902705A>T
Y15X
-
CHRNE_000034
-
PubMed: Croxen 2002
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
2
c.115_119dup
r.(?)
p.(Pro41Alafs*19)
-
pathogenic
g.4805986_4805990dup
g.4902691_4902695dup
115_119dupAGCCG
-
CHRNE_000019
-
PubMed: Ohno 1998
-
-
Germline
-
-
MwoI
-
-
Johan den Dunnen
+/.
2
c.115_119dup
r.(?)
p.(Pro41Alafs*19)
-
pathogenic
g.4805986_4805990dup
g.4902691_4902695dup
115_119dupAGCCG
-
CHRNE_000019
-
PubMed: Ohno 1998
-
-
Germline
-
-
MwoI
-
-
Johan den Dunnen
+/.
2
c.118C>T
r.(?)
p.(Arg40Trp)
-
pathogenic
g.4805987G>A
g.4902692G>A
-
-
CHRNE_000058
-
-
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
2
c.126dup
r.(?)
p.(Glu44Glyfs*3)
-
pathogenic
g.4805979dup
g.4902684dup
70insG
-
CHRNE_000021
-
PubMed: Ohno 1998
-
-
Germline
-
-
BdI
-
-
Johan den Dunnen
+/.
2
c.126dup
r.(?)
p.(Glu44Glyfs*3)
-
pathogenic
g.4805979dup
g.4902684dup
70insG
-
CHRNE_000021
-
PubMed: Ohno 1998
-
-
Germline
-
-
BdI
-
-
Johan den Dunnen
?/.
2
c.126dup
r.(?)
p.(Glu44Glyfs*3)
-
VUS
g.4805979dup
g.4902684dup
70insG
-
CHRNE_000021
-
PubMed: Croxen 2002
-
-
Germline
-
-
BdI
-
-
Johan den Dunnen
+/.
2
c.126dup
r.(?)
p.(?)
-
pathogenic
g.4805979dup
g.4902684dup
126dupG
-
CHRNE_000061
-
-
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
2
c.126dup
r.(?)
p.(Glu44Glyfs*3)
-
pathogenic
g.4805979dup
g.4902684dup
70insG
-
CHRNE_000021
-
PubMed: Ohno 1998
-
-
Germline
-
-
BdI
-
-
Johan den Dunnen
+/.
2
c.126dup
r.(?)
p.(Glu44Glyfs*3)
-
pathogenic
g.4805979dup
g.4902684dup
130dupG
-
CHRNE_000021
-
-
-
-
Germline
-
-
-
-
-
Angela Abicht
+/.
2
c.126dup
r.(?)
p.(Glu44Glyfs*3)
-
pathogenic
g.4805979dup
g.4902684dup
130dupG
-
CHRNE_000021
-
-
-
-
Germline
-
-
-
-
-
Angela Abicht
+/.
2
c.126dup
r.(?)
p.(Glu44Glyfs*3)
-
pathogenic
g.4805979dup
g.4902684dup
130dupG
-
CHRNE_000021
-
-
-
-
Germline
-
-
-
-
-
Angela Abicht
+/.
2
c.126dup
r.(?)
p.(Glu44Glyfs*3)
-
pathogenic
g.4805979dup
g.4902684dup
130dupG
-
CHRNE_000021
-
-
-
-
Germline
-
-
-
-
-
Angela Abicht
+/.
2
c.126dup
r.(?)
p.(Glu44Glyfs*3)
-
pathogenic
g.4805979dup
g.4902684dup
130dupG
-
CHRNE_000021
-
-
-
-
Germline
-
-
-
-
-
Angela Abicht
+/.
2
c.126dup
r.(?)
p.(Glu44Glyfs*3)
-
pathogenic
g.4805979dup
g.4902684dup
130dupG
-
CHRNE_000021
-
-
-
-
Germline
-
-
-
-
-
Angela Abicht
?/.
2
c.126dup
r.(?)
p.(Glu44Glyfs*3)
-
VUS
g.4805979dup
g.4902684dup
70insG
-
CHRNE_000021
-
PubMed: Croxen 2002
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
-
c.130dup
r.(?)
p.(Glu44Glyfs*3)
-
pathogenic
g.4805977dup
g.4902682dup
-
-
CHRNE_000169
-
Ohno et al. 1998. Ann Neurol 44: 234
-
rs762368691
Germline
-
-
-
-
-
Andreas Laner
+/.
-
c.130dup
r.(?)
p.(Glu44GlyfsTer3)
ACMG
pathogenic
g.4805977dup
g.4902682dup
-
-
CHRNE_000169
ACMG PVS1, PM2, PP5
PubMed: Molaei 2025
SCV001755187
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
2
c.158_161dup
r.(?)
p.(Lys34Asnfs*13)
-
pathogenic
g.4805944_4805947dup
g.4902649_4902652dup
-
-
CHRNE_000091
-
-
-
-
Germline
-
-
-
-
-
Angela Abicht
+/.
2
c.158_161dup
r.(?)
p.(Lys34Asnfs*13)
-
pathogenic
g.4805944_4805947dup
g.4902649_4902652dup
-
-
CHRNE_000091
-
-
-
-
Germline
-
-
-
-
-
Angela Abicht
+?/.
-
c.164T>A
r.(?)
p.(Val55Asp)
ACMG
likely pathogenic
g.4805941A>T
g.4902646A>T
-
-
CHRNE_000190
ACMG PM1, PM2, PP3, PP4
PubMed: Molaei 2025
SCV001755317
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
2
c.183_187dup
r.183_187dup
p.Leu63Profs*3
-
pathogenic
g.4805918_4805922dup
g.4902623_4902627dup
127ins5
-
CHRNE_000010
not in 100 control chromosomes
PubMed: Ohno 1997
-
-
Germline
-
-
BslI-
-
-
Johan den Dunnen
+/.
2
c.183_187dup
r.(?)
p.(Leu63Profs*3)
-
pathogenic
g.4805918_4805922dup
g.4902623_4902627dup
-
-
CHRNE_000010
-
-
-
-
Germline
-
-
-
-
-
Angela Abicht
+/.
2
c.183_187dup
r.183_187dup
p.Leu63Profs*3
-
pathogenic
g.4805918_4805922dup
g.4902623_4902627dup
127ins5
-
CHRNE_000010
not in 100 control chromosomes
PubMed: Ohno 1997
-
-
Germline
-
-
BslI-
-
-
Johan den Dunnen
+/.
2
c.183_187dup
r.(?)
p.(Leu63ProfsTer3)
-
pathogenic
g.4805918_4805922dup
g.4902623_4902627dup
-
-
CHRNE_000010
-
PubMed: Ganapathy 2019
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
2
c.183_187dup
r.(?)
p.(Leu63ProfsTer3)
-
pathogenic
g.4805918_4805922dup
g.4902623_4902627dup
-
-
CHRNE_000010
-
PubMed: Ganapathy 2019
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
-
c.183_187dup
r.(?)
p.(Leu63ProfsTer3)
ACMG
pathogenic
g.4805918_4805922dup
g.4902623_4902627dup
-
-
CHRNE_000010
ACMG PVS1, PS3, sup, PM2, PM3
PubMed: Molaei 2025
SCV001755366
-
Germline
-
-
-
-
-
Johan den Dunnen
?/.
-
c.202G>A
r.(?)
p.(Glu68Lys)
-
VUS
g.4805777C>T
-
CHRNE(NM_000080.4):c.202G>A (p.(Glu68Lys))
-
C17orf107_000033
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+/.
-
c.209_210del
r.(?)
p.(Leu70Hisfs*3)
-
pathogenic
g.4805772_4805773del
g.4902477_4902478del
-
-
CHRNE_000133
-
-
-
-
Germline
-
-
-
-
-
Andreas Laner
+/.
3
c.211A>C
r.(?)
p.(Thr71Pro)
-
pathogenic
g.4805768T>G
g.4902473T>G
T51P
-
CHRNE_000024
-
PubMed: Middleton 1999
-
-
Germline
-
-
HphI
-
-
Johan den Dunnen
+/.
3
c.211A>C
r.(?)
p.(Thr71Pro)
-
pathogenic
g.4805768T>G
g.4902473T>G
T51P
-
CHRNE_000024
-
PubMed: Middleton 1999
-
-
Germline
-
-
HphI
-
-
Johan den Dunnen
+/.
3
c.211A>C
r.(?)
p.(Thr71Pro)
-
pathogenic
g.4805768T>G
g.4902473T>G
T51P
-
CHRNE_000024
-
PubMed: Middleton 1999
-
-
Germline
-
-
HphI
-
-
Johan den Dunnen
?/.
-
c.221T>G
r.(?)
p.(Val74Gly)
-
VUS
g.4805758A>C
g.4902463A>C
-
-
C17orf107_000007
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
-/.
3i
c.234+54C>A
r.(?)
p.(=)
-
benign
g.4805691G>T
g.4902396G>T
IVS3+54C>A
-
CHRNE_000094
-
PubMed: Abicht 2002
-
-
Germline
-
-
-
-
-
Angela Abicht
-/.
3i
c.234+54C>A
r.(?)
p.(=)
-
benign
g.4805691G>T
g.4902396G>T
IVS3+54C>A
-
CHRNE_000094
-
PubMed: Abicht 2002
-
-
Germline
-
-
-
-
-
Angela Abicht
+/.
4
c.250C>T
r.(?)
p.(Arg84*)
-
pathogenic
g.4805606G>A
g.4902311G>A
C190T (R64X)
-
CHRNE_000011
not in 100 control chromosomes
PubMed: Ohno 1997 , OMIM:var0004
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
4
c.250C>T
r.(?)
p.(Arg84*)
-
pathogenic
g.4805606G>A
g.4902311G>A
R64X
-
CHRNE_000011
-
PubMed: Sieb 2000
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
4
c.250C>T
r.(?)
p.(Arg84*)
-
pathogenic
g.4805606G>A
g.4902311G>A
C190T (R64X)
-
CHRNE_000011
not in 100 control chromosomes
PubMed: Ohno 1997 , OMIM:var0004
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
-
c.250C>T
r.(?)
p.(Arg84*)
-
pathogenic
g.4805606G>A
-
CHRNE(NM_000080.4):c.250C>T (p.(Arg84*))
-
CHRNE_000011
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+/.
4
c.293T>C
r.(?)
p.(Leu98Pro)
-
pathogenic
g.4805563A>G
g.4902268A>G
L78P (233 T>C)
-
CHRNE_000001
-
PubMed: Croxen 2002 a
-
rs28929768
Germline
-
-
-
-
-
Johan den Dunnen