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
|
-/. |
- |
c.53>C>T |
r.(?) |
p.(Ile178=) |
- |
benign |
g.10393629G>A |
g.10412981G>A |
MKKS 534C>T |
- |
MKKS_000062 |
association study; frequency difference obese/healthy individuals non-significant |
PubMed: Rouskas 2008 |
- |
- |
Unknown |
? |
CC 171/220 obese subjects, CC 275/330 non-obese controls |
- |
- |
- |
LOVD |
+/. |
- |
c.-7485893_*1185894del |
r.0? |
p.0? |
- |
pathogenic |
g.9200001_17900000del |
g.9200001_17900000del |
JAG1 del20p12.1-p12.2, |
- |
JAG1_000783 |
heterozygous |
PubMed: Thorsteinsson 2021 |
- |
- |
Unknown |
? |
- |
- |
- |
- |
LOVD |
?/. |
1 |
c.-3436C>T |
r.(?) |
p.? |
- |
VUS |
g.10417543G>A |
- |
BBS6: c.-3436C>T |
- |
MKKS_000099 |
- |
PubMed: M'hamdi-2014 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
-?/. |
- |
c.-649+2430T>G |
r.(=) |
p.(=) |
- |
likely benign |
g.10412326A>C |
- |
MKKS(NM_018848.3):c.-649+6T>G |
- |
MKKS_000141 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Utrecht |
-?/. |
- |
c.-477G>T |
r.(?) |
p.(=) |
- |
likely benign |
g.10401235C>A |
- |
MKKS(NM_170784.2):c.-477G>T (p.?) |
- |
MKKS_000164 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Leiden |
-?/. |
- |
c.-417-13dup |
r.(=) |
p.(=) |
- |
likely benign |
g.10394599dup |
- |
MKKS(NM_018848.3):c.-417-13dupT |
- |
MKKS_000140 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Utrecht |
?/. |
_3_6_ |
c.(?_-417-1)_*467{0} |
r.0 |
p.0 |
- |
VUS |
g.(?_2639084)_(10394167_?)del |
- |
chr20:2639084-10394167 |
- |
MKKS_000000 |
- |
PubMed: Ellingsford 2018 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
+/. |
1 |
c.? |
r.(?) |
p.? |
- |
pathogenic |
g.10417543G>A |
- |
c.-3436C >T |
- |
MKKS_000099 |
- |
PubMed: M'hamdi 2014 |
- |
- |
Unknown |
- |
- |
- |
- |
- |
LOVD |
+?/. |
- |
c.? |
r.(?) |
p.? |
- |
likely pathogenic (recessive) |
g.? |
- |
BBS6:C499S |
- |
DNMT3B_000000 |
- |
PubMed: Katsanis-2001 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
+?/. |
- |
c.? |
r.(?) |
p.? |
- |
likely pathogenic (recessive) |
g.? |
- |
BBS6:Q147X |
- |
DNMT3B_000000 |
- |
PubMed: Katsanis-2001 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
?/. |
- |
c.? |
r.(?) |
p.? |
- |
VUS |
g.10393205T>A |
- |
g.10393205T>A |
- |
MKKS_000099 |
Variant reference (T) does not agree with reference sequence (G) |
PubMed: Anasagasti-2013 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
LOVD |
?/. |
- |
c.? |
r.(?) |
p.? |
- |
VUS |
g.10393205T>A |
- |
g.10393205T>A |
- |
MKKS_000099 |
Variant reference (T) does not agree with reference sequence (G) |
PubMed: Anasagasti-2013 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
LOVD |
+?/. |
- |
c.? |
r.(?) |
p.? |
- |
likely pathogenic |
g.? |
- |
BBS6:Q147X |
- |
DNMT3B_000000 |
- |
PubMed: Eichers-2009, Katsanis 2001 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
+/. |
- |
c.? |
r.(?) |
p.F94SfsX9 |
- |
pathogenic |
g.? |
- |
[F94SfsX9]+[F94SfsX9] |
- |
DNMT3B_000000 |
- |
PubMed: Billingsley-2010 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
+?/. |
- |
c.? |
r.(?) |
p.? |
- |
likely pathogenic |
g.? |
- |
[p.T501M];[p.R525H] |
- |
DNMT3B_000000 |
- |
PubMed: Deveault-2011 |
- |
- |
Unknown |
- |
- |
- |
- |
- |
LOVD |
?/. |
3 |
c.3G>H |
r.? |
p.? |
- |
VUS |
g.10394160C>A |
- |
BBS6:p.[M1I];[Y37C] |
- |
MKKS_000127 |
- |
PubMed: Scheidecker 2015 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
?/. |
- |
c.7C>T |
r.(?) |
p.(Arg3Cys) |
- |
VUS |
g.10394156G>A |
- |
MKKS(NM_170784.3):c.7C>T (p.(Arg3Cys)) |
- |
MKKS_000086 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Leiden |
-/. |
3 |
c.16G>A |
r.(?) |
p.(Ala6Thr) |
- |
benign |
g.10394147C>T |
g.10413499C>T |
MKKS(NM_018848.3):c.16G>A (p.A6T) |
- |
MKKS_000069 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Utrecht |
-/. |
- |
c.16G>A |
r.(?) |
p.(Ala6Thr) |
- |
benign |
g.10394147C>T |
- |
MKKS(NM_018848.3):c.16G>A (p.A6T) |
- |
MKKS_000069 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_AMC |
?/. |
- |
c.59A>G |
r.(?) |
p.(Glu20Gly) |
- |
VUS |
g.10394104T>C |
- |
MKKS(NM_018848.3):c.59A>G (p.E20G) |
- |
MKKS_000093 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Rotterdam |
./. |
3 |
c.67A>G |
r.(?) |
p.(Arg23Gly) |
- |
likely benign |
g.10394096T>C |
g.10413448T>C |
c.67T>C / g.10394096A>G |
- |
MKKS_000048 |
- |
Journal: Lim 2014 |
- |
- |
Germline |
no |
- |
- |
- |
- |
Marianne Vos (LOVD-team) |
-?/. |
3 |
c.67A>G |
r.(?) |
p.(Arg23Gly) |
- |
likely benign |
g.10394096T>C |
g.10413448T>C |
MKKS(NM_018848.3):c.67A>G (p.(Arg23Gly), p.R23G) |
- |
MKKS_000048 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Utrecht |
-?/. |
- |
c.67A>G |
r.(?) |
p.(Arg23Gly) |
- |
likely benign |
g.10394096T>C |
- |
MKKS(NM_018848.3):c.67A>G (p.(Arg23Gly), p.R23G) |
- |
MKKS_000048 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Leiden |
+/. |
3 |
c.110A>G |
r.(?) |
p.(Tyr37Cys) |
- |
pathogenic |
g.10394053T>C |
g.10413405T>C |
MKKS(NM_018848.3):c.110A>G (p.Y37C) |
- |
MKKS_000005 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Nijmegen |
+/. |
- |
c.110A>G |
r.(?) |
p.(Tyr37Cys) |
- |
pathogenic |
g.10394053T>C |
g.10413405T>C |
MKKS(NM_018848.3):c.110A>G (p.Y37C) |
- |
MKKS_000005 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_AMC |
+/. |
- |
c.110A>G |
r.(?) |
p.(Tyr37Cys) |
- |
pathogenic |
g.10394053T>C |
g.10413405T>C |
MKKS(NM_018848.3):c.110A>G (p.Y37C) |
- |
MKKS_000005 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Utrecht |
+/. |
- |
c.110A>G |
r.(?) |
p.(Tyr37Cys) |
- |
pathogenic |
g.10394053T>C |
g.10413405T>C |
MKKS(NM_018848.3):c.110A>G (p.Y37C) |
- |
MKKS_000005 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_VUmc |
+?/. |
- |
c.110A>G |
r.(?) |
p.(Tyr37Cys) |
- |
likely pathogenic |
g.10394053T>C |
g.10413405T>C |
- |
- |
MKKS_000005 |
- |
PubMed: Stone 2017 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
+?/. |
3 |
c.110A>G |
r.(?) |
p.(Tyr37Cys) |
- |
likely pathogenic (recessive) |
g.10394053T>C |
- |
BBS6:Y37C |
- |
MKKS_000005 |
- |
PubMed: Katsanis-2001 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
+?/. |
3 |
c.110A>G |
r.(?) |
p.(Tyr37Cys) |
- |
likely pathogenic |
g.10394053T>C |
- |
BBS6:c.110A>G |
- |
MKKS_000005 |
- |
PubMed: Muller-2010, Stone 2000 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
+?/. |
3 |
c.110A>G |
r.(?) |
p.(Tyr37Cys) |
- |
likely pathogenic |
g.10394053T>C |
- |
BBS6:c.110A>G |
- |
MKKS_000005 |
- |
PubMed: Muller-2010, Stone 2000 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
+?/. |
3 |
c.110A>G |
r.(?) |
p.(Tyr37Cys) |
- |
likely pathogenic |
g.10394053T>C |
- |
BBS6:Y37C |
- |
MKKS_000005 |
- |
PubMed: Eichers-2009, Katsanis 2002 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
+?/. |
3 |
c.110A>G |
r.(?) |
p.(Tyr37Cys) |
- |
likely pathogenic |
g.10394053T>C |
- |
BBS6:c.110A>G |
- |
MKKS_000005 |
- |
PubMed: Feuillan-2011 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
+?/. |
3 |
c.110A>G |
r.(?) |
p.(Tyr37Cys) |
- |
likely pathogenic |
g.10394053T>C |
- |
BBS6:c.110A>G |
- |
MKKS_000005 |
- |
PubMed: Feuillan-2011 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
+?/. |
3 |
c.110A>G |
r.(?) |
p.(Tyr37Cys) |
- |
likely pathogenic |
g.10394053T>C |
- |
BBS6:c.110A>G |
- |
MKKS_000005 |
- |
PubMed: Feuillan-2011 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
+?/. |
3 |
c.110A>G |
r.(?) |
p.(Tyr37Cys) |
- |
likely pathogenic |
g.10394053T>C |
- |
c.110A>G |
- |
MKKS_000005 |
- |
PubMed: Schaefer-2011 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
?/. |
3 |
c.110A>G |
r.(?) |
p.(Tyr37Cys) |
- |
VUS |
g.10394053T>C |
- |
BBS6:p.[M1I];[Y37C] |
- |
MKKS_000005 |
- |
PubMed: Scheidecker 2015 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
+?/. |
- |
c.110A>G |
r.(?) |
p.(Tyr37Cys) |
- |
likely pathogenic |
g.10394053T>C |
g.10413405T>C |
MKKS 997A->G, Y37C |
- |
MKKS_000005 |
obsolete nucletotide annotation, it should be c.110A>G and not 997A->G; heterozygous |
PubMed: Stone 2000 |
- |
- |
Germline |
yes |
0/200 chromosomes from the non-Amish control group |
- |
- |
- |
LOVD |
+?/. |
- |
c.110A>G |
r.(?) |
p.(Tyr37Cys) |
- |
likely pathogenic |
g.10394053T>C |
g.10413405T>C |
MKKS Y37C |
- |
MKKS_000005 |
homozygous |
PubMed: Katsanis 2000 |
- |
- |
Germline |
yes |
0/188 European or 24 Newfoundland unrelated control chromosomes |
- |
- |
- |
LOVD |
+?/. |
- |
c.110A>G |
r.(?) |
p.(Tyr37Cys) |
- |
likely pathogenic |
g.10394053T>C |
g.10413405T>C |
MKKS Y37C |
- |
MKKS_000005 |
homozygous |
PubMed: Katsanis 2000 |
- |
- |
Germline |
yes |
0/188 European or 24 Newfoundland unrelated control chromosomes |
- |
- |
- |
LOVD |
+/. |
- |
c.110A>G |
r.(?) |
p.(Tyr37Cys) |
ACMG |
pathogenic (recessive) |
g.10394053T>C |
g.10413405T>C |
- |
- |
MKKS_000005 |
ACMG PP3, PM2, PP5_STRONG |
PubMed: Weisschuh 2024 |
5309 |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
-?/. |
3 |
c.114C>T |
r.(?) |
p.(Gly38=) |
- |
likely benign |
g.10394049G>A |
g.10413401G>A |
MKKS(NM_018848.3):c.114C>T (p.G38=) |
- |
MKKS_000068 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Rotterdam |
+?/. |
3 |
c.116C>T |
r.(?) |
p.(Pro39Leu) |
- |
likely pathogenic |
g.10394047G>A |
- |
c.116C>T p.(P39L) |
- |
MKKS_000123 |
- |
PubMed: Abu-Safieh-2012 |
- |
- |
Germline |
- |
0/96 ethnically matched controls |
- |
- |
- |
LOVD |
+?/. |
- |
c.116C>T |
r.(?) |
p.(Pro39Leu) |
- |
likely pathogenic |
g.10394047G>A |
g.10413399G>A |
MKKS c.116C>T, p.(Pro39Leu) |
- |
MKKS_000123 |
homozygous |
PubMed: Aldahmesh 2014 |
- |
- |
Germline |
? |
- |
- |
- |
- |
LOVD |
+?/. |
- |
c.116C>T |
r.(?) |
p.(Pro39Leu) |
- |
likely pathogenic |
g.10394047G>A |
g.10413399G>A |
MKKS c.116C>T, p.(Pro39Leu) |
- |
MKKS_000123 |
homozygous |
PubMed: Aldahmesh 2014 |
- |
- |
Germline |
? |
- |
- |
- |
- |
LOVD |
-/. |
3 |
c.117C>T |
r.(?) |
p.(Pro39=) |
- |
benign |
g.10394046G>A |
g.10413398G>A |
MKKS(NM_018848.3):c.117C>T (p.P39=) |
- |
MKKS_000067 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Rotterdam |
-/. |
3 |
c.117C>T |
r.(?) |
p.(Pro39=) |
- |
benign |
g.10394046G>A |
g.10413398G>A |
MKKS(NM_018848.3):c.117C>T (p.P39=) |
- |
MKKS_000067 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Nijmegen |
+/. |
3 |
c.117C>T |
r.(=) |
p.(=) |
- |
pathogenic |
g.10394046G>A |
- |
BBS6:c.117C>T |
- |
MKKS_000067 |
- |
PubMed: Hoskins-2003 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
-/. |
- |
c.117C>T |
r.(?) |
p.(=) |
- |
benign |
g.10394046G>A |
g.10413398G>A |
- |
- |
MKKS_000067 |
- |
PubMed: Smaoui 2006 |
- |
rs17852626 |
Germline |
- |
4/19 families BBS |
- |
- |
- |
Johan den Dunnen |
+/. |
3 |
c.119C>G |
r.(?) |
p.(Ser40*) |
- |
pathogenic |
g.10394044G>C |
- |
c.119C>G |
- |
MKKS_000130 |
- |
PubMed: Ullah-2017 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
LOVD |
+/. |
3 |
c.119C>G |
r.(?) |
p.(Ser40*) |
- |
pathogenic |
g.10394044G>C |
- |
c.119C>G |
- |
MKKS_000130 |
- |
PubMed: Ullah-2017 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
LOVD |
+/. |
3 |
c.119C>G |
r.(?) |
p.(Ser40*) |
- |
pathogenic |
g.10394044G>C |
- |
c.119C>G |
- |
MKKS_000130 |
- |
PubMed: Ullah-2017 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
LOVD |
+/. |
3 |
c.121G>C |
r.(?) |
p.(Gly41Arg) |
- |
pathogenic |
g.10394042C>G |
- |
[G41R]+[G41R] |
- |
MKKS_000122 |
- |
PubMed: Billingsley-2010 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
+?/. |
3 |
c.121G>C |
r.(?) |
p.(Gly41Arg) |
- |
likely pathogenic |
g.10394042C>G |
- |
[p.G539D];[p.P632FfsX7] |
- |
MKKS_000122 |
- |
PubMed: Deveault-2011 |
- |
- |
Unknown |
- |
- |
- |
- |
- |
LOVD |
+?/. |
- |
c.155G>A |
r.(?) |
p.(Gly52Asp) |
- |
likely pathogenic |
g.10394008C>T |
- |
MKKS 1042G->A, G52D |
- |
MKKS_000151 |
obsolete nucletotide annotation, it should be c.155G>A and not 1042G->A; heterozygous |
PubMed: Slavotinek 2000 |
- |
- |
Germline |
yes |
0/102 chromosomes from Hispanic controls |
- |
- |
- |
LOVD |
?/. |
3 |
c.166A>G |
r.(?) |
p.(Thr56Ala) |
- |
VUS |
g.10393997T>C |
g.10413349T>C |
MKKS(NM_018848.3):c.166A>G (p.T56A) |
- |
MKKS_000066 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Rotterdam |
?/. |
3 |
c.169A>G |
r.(?) |
p.(Thr57Ala) |
- |
VUS |
g.10393994T>C |
g.10413346T>C |
MKKS(NM_018848.3):c.169A>G (p.T57A) |
- |
MKKS_000006 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Rotterdam |
+?/. |
3 |
c.169A>G |
r.(?) |
p.(Thr57Ala) |
- |
likely pathogenic |
g.10393994T>C |
- |
BBS6:c.169A>G |
- |
MKKS_000006 |
- |
PubMed: Muller-2010, Katsanis 2000 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
+?/. |
- |
c.169A>G |
r.(?) |
p.(Thr57Ala) |
- |
likely pathogenic |
g.10393994T>C |
g.10413346T>C |
MKKS T57A |
- |
MKKS_000006 |
single heterozygous, no second allele found |
PubMed: Katsanis 2000 |
- |
- |
Germline |
yes |
0/192 European or 24 Newfoundland unrelated control chromosomes |
- |
- |
- |
LOVD |
-/. |
3 |
c.207C>T |
r.(?) |
p.(Val69=) |
- |
benign |
g.10393956G>A |
g.10413308G>A |
MKKS(NM_018848.3):c.207C>T (p.V69=) |
- |
MKKS_000065 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_AMC |
+?/. |
- |
c.250C>T |
r.(?) |
p.(His84Tyr) |
- |
likely pathogenic |
g.10393913G>A |
g.10413265G>A |
MKKS 1137C->T, H84Y |
- |
MKKS_000150 |
obsolete nucletotide annotation, it should be c.250C>T and not 1137C->T; predicted to interfere with ATP hydrolysis; linked in this family to a likely benign p.A242S variant; homozygous |
PubMed: Stone 2000 |
- |
- |
Germline |
yes |
0/200 chromosomes from the non-Amish control group |
- |
- |
- |
LOVD |
+?/. |
3 |
c.269A>G |
r.(?) |
p.(Asp90Gly) |
- |
likely pathogenic |
g.10393894T>C |
g.10413246T>C |
- |
- |
MKKS_000046 |
- |
PubMed: González-del Pozo 2014 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
María González-del Pozo |
+/. |
3 |
c.269A>G |
r.(?) |
p.(Asp90Gly) |
- |
pathogenic |
g.10393894T>C |
- |
c.269A>G; p.D90G |
- |
MKKS_000046 |
- |
PubMed: Gonzalez del Pozo 2013 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
LOVD |
+/. |
3 |
c.269A>G |
r.(?) |
p.(Asp90Gly) |
- |
pathogenic |
g.10393894T>C |
- |
c.269A>G; p.D90G |
- |
MKKS_000046 |
- |
PubMed: Gonzalez del Pozo 2013 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
LOVD |
+/. |
3 |
c.269A>G |
r.(?) |
p.(Asp90Gly) |
- |
pathogenic |
g.10393894T>C |
- |
c.269A>G; p.D90G |
- |
MKKS_000046 |
- |
PubMed: Gonzalez del Pozo 2013 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
LOVD |
+?/. |
- |
c.281del |
r.(?) |
p.(Phe94Serfs*9) |
- |
likely pathogenic |
g.10393883del |
- |
MKKS 1167 delT |
- |
MKKS_000149 |
obsolete nucletotide annotation, it should be c.281del and not 1167 delT; homozygous |
PubMed: Slavotinek 2000 |
- |
- |
Germline |
yes |
0/102 Northern European control chromosomes |
- |
- |
- |
LOVD |
+?/. |
- |
c.281del |
r.(?) |
p.(Phe94Serfs*9) |
- |
likely pathogenic |
g.10393883del |
- |
MKKS 1167 delT |
- |
MKKS_000149 |
obsolete nucletotide annotation, it should be c.281del and not 1167 delT; No DNA was available from the father, so might be a deletion on the other allele; homozygous |
PubMed: Slavotinek 2000 |
- |
- |
Germline |
yes |
0/102 Northern European control chromosomes |
- |
- |
- |
LOVD |
+?/. |
- |
c.281del |
r.(?) |
p.(Phe94Serfs*9) |
- |
likely pathogenic |
g.10393883del |
- |
MKKS 1167 delT |
- |
MKKS_000149 |
obsolete nucletotide annotation, it should be c.281del and not 1167 delT; No DNA was available from the father, so might be a deletion on the other allele; homozygous |
PubMed: Slavotinek 2000 |
- |
- |
Germline |
yes |
0/102 Northern European control chromosomes |
- |
- |
- |
LOVD |
+?/. |
- |
c.281del |
r.(?) |
p.(Phe94Serfs*9) |
- |
likely pathogenic |
g.10393883del |
g.10413235del |
MKKS fs2=280delT (F94fsX103) |
- |
MKKS_000149 |
heterozygous |
PubMed: Katsanis 2000 |
- |
- |
Germline |
yes |
0/168 European or 84 Newfoundland unrelated control chromosomes |
- |
- |
- |
LOVD |
+?/. |
- |
c.281del |
r.(?) |
p.(Phe94Serfs*9) |
- |
likely pathogenic |
g.10393883del |
g.10413235del |
MKKS fs2=280delT (F94fsX103) |
- |
MKKS_000149 |
heterozygous |
PubMed: Katsanis 2000 |
- |
- |
Germline |
yes |
0/168 European or 84 Newfoundland unrelated control chromosomes |
- |
- |
- |
LOVD |
+?/. |
- |
c.281del |
r.(?) |
p.(Phe94Serfs*9) |
- |
likely pathogenic |
g.10393883del |
g.10413235del |
MKKS fs2=280delT (F94fsX103) |
- |
MKKS_000149 |
heterozygous |
PubMed: Katsanis 2000 |
- |
- |
Germline |
yes |
0/168 European or 84 Newfoundland unrelated control chromosomes |
- |
- |
- |
LOVD |
+?/. |
- |
c.281del |
r.(?) |
p.(Phe94Serfs*9) |
- |
likely pathogenic |
g.10393883del |
g.10413235del |
MKKS fs2=280delT (F94fsX103) |
- |
MKKS_000149 |
homozygous |
PubMed: Katsanis 2000 |
- |
- |
Germline |
yes |
0/168 European or 84 Newfoundland unrelated control chromosomes |
- |
- |
- |
LOVD |
+?/. |
- |
c.281del |
r.(?) |
p.(Phe94Serfs*9) |
- |
likely pathogenic |
g.10393883del |
g.10413235del |
MKKS fs2=280delT (F94fsX103) |
- |
MKKS_000149 |
homozygous |
PubMed: Katsanis 2000 |
- |
- |
Germline |
yes |
0/168 European or 84 Newfoundland unrelated control chromosomes |
- |
- |
- |
LOVD |
+?/. |
- |
c.281del |
r.(?) |
p.(Phe94Serfs*9) |
- |
likely pathogenic |
g.10393883del |
g.10413235del |
MKKS fs2=280delT (F94fsX103) |
- |
MKKS_000149 |
homozygous |
PubMed: Katsanis 2000 |
- |
- |
Germline |
yes |
0/168 European or 84 Newfoundland unrelated control chromosomes |
- |
- |
- |
LOVD |
+?/. |
- |
c.287C>T |
r.(?) |
p.(Ala96Val) |
- |
likely pathogenic |
g.10393876G>A |
g.10413228G>A |
MKKS c.287C>T, p.Ala96Val |
- |
MKKS_000155 |
homozygous |
PubMed: Ullah 2018 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
LOVD |
+?/. |
- |
c.287C>T |
r.(?) |
p.(Ala96Val) |
- |
likely pathogenic |
g.10393876G>A |
g.10413228G>A |
MKKS c.287C>T, p.Ala96Val |
- |
MKKS_000155 |
homozygous |
PubMed: Ullah 2018 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
LOVD |
+?/. |
- |
c.295T>C |
r.(?) |
p.(Cys99Arg) |
- |
likely pathogenic |
g.10393868A>G |
g.10413220A>G |
- |
- |
MKKS_000097 |
- |
PubMed: Patel 2016 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
+/. |
3 |
c.295T>C |
r.(?) |
p.(Cys99Arg) |
- |
pathogenic |
g.10393868A>G |
- |
[C99R]+[C99R] |
- |
MKKS_000097 |
- |
PubMed: Billingsley-2010 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
+?/. |
3 |
c.295T>C |
r.(?) |
p.(Cys99Arg) |
- |
likely pathogenic |
g.10393868A>G |
- |
[c.197+1G>T];[p.L600S] |
- |
MKKS_000097 |
- |
PubMed: Deveault-2011 |
- |
- |
Unknown |
- |
- |
- |
- |
- |
LOVD |
+?/. |
- |
c.295T>C |
r.(?) |
p.(Cys99Arg) |
- |
likely pathogenic |
g.10393868A>G |
g.10413220A>G |
MKKS c.29ST >C p.(Cys99Arg) |
- |
MKKS_000097 |
homozygous |
PubMed: Méjécase 2020 |
- |
- |
Unknown |
? |
- |
- |
- |
- |
LOVD |
+/. |
3 |
c.295T>C |
r.(?) |
p.(Cys99Arg) |
- |
pathogenic (recessive) |
g.10393868A>G |
- |
BBS6:c.[295T>C];[492T>A] |
- |
MKKS_000097 |
- |
PubMed: Mary-2019 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
-?/. |
- |
c.336A>G |
r.(?) |
p.(Thr112=) |
- |
likely benign |
g.10393827T>C |
g.10413179T>C |
MKKS(NM_018848.3):c.336A>G (p.T112=) |
- |
MKKS_000084 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Rotterdam |
?/. |
- |
c.363A>C |
r.(?) |
p.(Lys121Asn) |
- |
VUS |
g.10393800T>G |
g.10413152T>G |
MKKS(NM_018848.3):c.363A>C (p.K121N) |
- |
MKKS_000083 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Rotterdam |
+?/. |
3 |
c.380del |
r.(?) |
p.(Cys127Serfs*32) |
- |
likely pathogenic |
g.10393783del |
- |
BBS6:c.380delG |
- |
MKKS_000121 |
- |
PubMed: Feuillan-2011 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
+?/. |
- |
c.415C>T |
r.(?) |
p.(Arg139*) |
- |
likely pathogenic |
g.10393748G>A |
g.10413100G>A |
BBS6 c.415C>T, p.Arg139X |
- |
MKKS_000148 |
heterozygous |
PubMed: Putoux 2010 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
LOVD |
-?/. |
3 |
c.416G>A |
r.(?) |
p.(Arg139Gln) |
- |
likely benign |
g.10393747C>T |
g.10413099C>T |
MKKS(NM_018848.3):c.416G>A (p.R139Q) |
- |
MKKS_000064 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Utrecht |
-/. |
- |
c.416G>A |
r.(?) |
p.(Arg139Gln) |
- |
benign |
g.10393747C>T |
g.10413099C>T |
- |
- |
MKKS_000064 |
2 heterozygous, no homozygous; Clinindb (India) |
PubMed: Narang 2020, Journal: Narang 2020 |
- |
rs145045986 |
Germline |
- |
2/2795 individuals |
- |
- |
- |
Mohammed Faruq |
+?/. |
- |
c.416G>A |
r.(?) |
p.(Arg139Gln) |
- |
likely pathogenic |
g.10393747C>T |
g.10413099C>T |
MKKS/BBS6 c.416G>A, p.Arg139Gln |
- |
MKKS_000064 |
single heterozygous variant in a recessive disease; no second causative allele found |
PubMed: Kang 2016 |
- |
- |
Unknown |
? |
- |
- |
- |
- |
LOVD |
-?/. |
- |
c.420C>T |
r.(?) |
p.(Ile140=) |
- |
likely benign |
g.10393743G>A |
g.10413095G>A |
MKKS(NM_018848.3):c.420C>T (p.I140=) |
- |
MKKS_000082 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Rotterdam |
?/. |
3 |
c.429delCT |
r.(?) |
p.? |
- |
VUS |
g.10393734delAG |
- |
[p.G119S; p.Y263H] |
- |
MKKS_000120 |
normal 2nd chromosome |
PubMed: Imhoff-2011 |
- |
- |
Unknown |
- |
- |
- |
- |
- |
LOVD |
?/. |
3 |
c.429_433delinsTT |
r.(?) |
p.(Phe144_Ser145delinsCys) |
- |
VUS |
g.10393730_10393734delinsAA |
- |
c.429delCT433delAG |
- |
MKKS_000105 |
- |
PubMed: Hichri-2005 |
- |
- |
Unknown |
- |
- |
- |
- |
- |
LOVD |
+?/. |
- |
c.429_434delinsTT |
r.(?) |
p.(Phe144Leufs*14) |
- |
likely pathogenic |
g.10393729_10393734delinsAA |
- |
MKKS 1316delC and 1324-1326delGTA |
- |
MKKS_000147 |
obsolete nucletotide annotation, it should be c.429_434delinsTT and not 1316delC with 1324-1326delGTA; homozygous |
PubMed: Slavotinek 2000 |
- |
- |
Germline |
yes |
0/102 Northern European control chromosomes |
- |
- |
- |
LOVD |
+?/. |
- |
c.429_434delinsTT |
r.(?) |
p.(Phe144Leufs*14) |
- |
likely pathogenic |
g.10393729_10393734delinsAA |
- |
MKKS 1316delC and 1324-1326delGTA |
- |
MKKS_000147 |
obsolete nucletotide annotation, it should be c.429_434delinsTT and not 1316delC with 1324-1326delGTA; homozygous |
PubMed: Slavotinek 2000 |
- |
- |
Germline |
yes |
0/102 Northern European control chromosomes |
- |
- |
- |
LOVD |
+?/. |
- |
c.429_434delinsTT |
r.(?) |
p.(Phe144Leufs*14) |
- |
likely pathogenic |
g.10393729_10393734delinsAA |
g.10413081_10413086delinsAA |
MKKS fs1=429delCT/433delAG (D143fsX157) |
- |
MKKS_000147 |
heterozygous |
PubMed: Katsanis 2000 |
- |
- |
Germline |
yes |
0/172 European or 76 Newfoundland unrelated control chromosomes |
- |
- |
- |
LOVD |
+?/. |
- |
c.429_434delinsTT |
r.(?) |
p.(Phe144Leufs*14) |
- |
likely pathogenic |
g.10393729_10393734delinsAA |
g.10413081_10413086delinsAA |
MKKS fs1=429delCT/433delAG (D143fsX157) |
- |
MKKS_000147 |
heterozygous |
PubMed: Katsanis 2000 |
- |
- |
Germline |
yes |
0/172 European or 76 Newfoundland unrelated control chromosomes |
- |
- |
- |
LOVD |
+?/. |
- |
c.429_434delinsTT |
r.(?) |
p.(Phe144Leufs*14) |
- |
likely pathogenic |
g.10393729_10393734delinsAA |
g.10413081_10413086delinsAA |
MKKS fs1=429delCT/433delAG (D143fsX157) |
- |
MKKS_000147 |
homozygous |
PubMed: Katsanis 2000 |
- |
- |
Germline |
yes |
0/172 European or 76 Newfoundland unrelated control chromosomes |
- |
- |
- |
LOVD |
+?/. |
- |
c.429_434delinsTT |
r.(?) |
p.(Phe144Leufs*14) |
- |
likely pathogenic |
g.10393729_10393734delinsAA |
g.10413081_10413086delinsAA |
MKKS fs1=429delCT/433delAG (D143fsX157) |
- |
MKKS_000147 |
homozygous |
PubMed: Katsanis 2000 |
- |
- |
Germline |
yes |
0/172 European or 76 Newfoundland unrelated control chromosomes |
- |
- |
- |
LOVD |
+?/. |
- |
c.431_441delTTAGTAGTACT |
r.(?) |
p.(Phe144Serfs*9) |
- |
likely pathogenic |
g.10393725_10393735del |
g.10413077_10413087del |
MKKS c.431_441delTTAGTAGTACT |
- |
MKKS_000146 |
homozygous |
PubMed: Slavotinek 2002 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
LOVD |
+?/. |
- |
c.432_435del |
r.(?) |
p.(Phe144Leufs*14) |
ACMG |
likely pathogenic |
g.10393729_10393732del |
g.10413081_10413084del |
MKKS, c.432_435delTAGT, p.Phe144Leufs*14, homozygous |
- |
MKKS_000134 |
- |
PubMed: Perea-Romero 2021 |
- |
- |
Unknown |
? |
- |
- |
- |
- |
LOVD |
+?/. |
3 |
c.433delAG |
r.(?) |
p.? |
- |
likely pathogenic |
g.10393730delCT |
- |
[p.L55P];[p.E274VfsX29] |
- |
MKKS_000119 |
- |
PubMed: Imhoff-2011 |
- |
- |
Unknown |
- |
- |
- |
- |
- |
LOVD |