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.
Reported: The number of times this variant has been reported in the database.
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
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
|

 Reported
|

 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 |
01-09a |
c.(?-12444)_996+?del |
r.? |
p.? |
- |
pathogenic |
g.(?_100013161)_(100133463_?)del |
- |
1 more item |
- |
VPS13B_000006 |
1 COH1 family (hom) |
PubMed: Balikova et al. 2011 |
- |
- |
SUMMARY record |
yes |
- |
- |
- |
- |
Anne Polvi |
+?/. |
1 |
- |
c.-111_(762+1_763-1){0} |
r.0? |
p.0? |
- |
likely pathogenic (recessive) |
g.(?_100025494)_(100123508_100127927)del |
g.(?_99013266)_(99111280_99115699)del |
ex1-6 deletion |
- |
VPS13B_000380 |
- |
PubMed: Taylor 2017 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
+/. |
1 |
- |
c.7220_7221A |
r.(?) |
p.(?) |
ACMG |
pathogenic |
g.? |
g.? |
VPS13B c.7220_7221A, p.(?) |
- |
RP1_000000 |
error in annotation, actual variant unknown, heterozygous |
PubMed: Kim 2021 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
LOVD |
+/+ |
1 |
01-20 |
c.-79752_2516-7756del |
r.? |
p? |
- |
pathogenic |
g.99945853_100278670del |
g.98933625_99266442del |
315 kb del: exons 1-17: chr8(hg18):100015029...100347846; c.1-2515del |
- |
VPS13B_000008 |
1 more item |
Rivera-Brugués 2011 |
- |
- |
SUMMARY record |
yes |
0/1612 CON |
- |
- |
- |
Anne Polvi |
+/+ |
1 |
01-09a |
c.-23990_1207-5855del |
r.? |
p.? |
- |
pathogenic |
g.100001615_100141005del |
g.98989387_99128777del |
Del promotor - 8: 100070791 - 100210181 bp (hg18) |
- |
VPS13B_000007 |
2 Belgian COH1 families (hom); Deletion of exons 1-9a |
PubMed: Balikova et al. 2009 |
- |
- |
SUMMARY record |
yes |
- |
- |
- |
- |
Anne Polvi |
+?/. |
1 |
- |
c.(?_-1)_(937+1_938-1)del |
r.0? |
p.0? |
- |
likely pathogenic |
g.(?_100026016)_(100128103_100133404)del |
g.(?_99013788)_(99115875_99121176)del |
g.100025966_100128152del |
- |
VPS13B_000425 |
- |
PubMed: Ellingford 2017 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/., +?/. |
4 |
56 |
c.? |
r.? |
p.? |
- |
likely pathogenic, likely pathogenic (recessive), pathogenic (recessive) |
g.100789002G>A, g.100866389_100866391del, g.? |
g.99776774G>A, g.99854161_99854163del |
del ex17-19, del ex8, NM_017890.3:c.10847_10849del, NM_017890.3:c.7323-1G>A |
- |
RP1_000000 |
- |
PubMed: Nambot 2018, PubMed: Stone 2017 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
1 |
2 |
c.3G>A |
r.(?) |
p.0? |
- |
pathogenic |
g.100026019G>A |
g.99013791G>A |
NM_152564: c.G3A; p.M1I |
- |
VPS13B_000172 |
- |
PubMed: Karaca 2015 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
?/. |
1 |
- |
c.8A>T |
r.(?) |
p.(Glu3Val) |
- |
VUS |
g.100026024A>T |
g.99013796A>T |
VPS13B(NM_017890.4):c.8A>T (p.E3V) |
- |
COX6C_000002 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Rotterdam |
+/+ |
1 |
02 |
c.22_23delCCinsA |
r.22_23delccinsa |
p.Pro8Lysfs*3 |
- |
pathogenic |
g.100026038_100026039delinsA |
g.99013810_99013811delinsA |
c.22_23delCCinsA: p.Pro8fsX3 |
- |
VPS13B_000009 |
1 Danish COH1 patient (com-het) |
PubMed: Kolehmainen et al. 2004 |
- |
- |
SUMMARY record |
yes |
- |
- |
- |
- |
Anne Polvi |
-/. |
1 |
- |
c.147+90A>G |
r.(=) |
p.(=) |
- |
benign |
g.100026253A>G |
g.99014025A>G |
VPS13B(NM_017890.4):c.147+90A>G |
- |
VPS13B_000174 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Utrecht |
-/. |
1 |
- |
c.147+112T>C |
r.(=) |
p.(=) |
- |
benign |
g.100026275T>C |
g.99014047T>C |
VPS13B(NM_017890.4):c.147+112T>C |
- |
VPS13B_000175 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Utrecht |
?/. |
1 |
- |
c.148-8608G>C |
- |
p.(=) |
- |
VUS |
g.100042043G>C |
g.99029815G>C |
- |
- |
VPS13B_000004 |
- |
- |
- |
- |
Germline |
- |
- |
- |
- |
- |
Yu Sun |
-/. |
1 |
- |
c.174A>G |
r.(?) |
p.(Leu58=) |
- |
benign |
g.100050677A>G |
g.99038449A>G |
VPS13B(NM_017890.4):c.174A>G (p.L58=) |
- |
VPS13B_000365 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Utrecht |
?/. |
1 |
- |
c.203A>G |
r.(?) |
p.(His68Arg) |
- |
VUS |
g.100050706A>G |
g.99038478A>G |
VPS13B(NM_015243.2):c.203A>G (p.(His68Arg)) |
- |
VPS13B_000176 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Leiden |
+/+, +/. |
2 |
3 |
c.219_220delinsT |
r.(219_220delinsu) |
p.(Lys73Asnfs*8) |
- |
pathogenic (recessive) |
g.100050722_100050723delinsT |
g.99038494_99038495delinsT |
219_20delACinsT |
- |
VPS13B_000010 |
1 COH1 family (com-het) |
PubMed: Parri 2010 |
- |
- |
Germline, SUMMARY record |
yes |
- |
- |
- |
- |
Johan den Dunnen, Anne Polvi |
+/+ |
1 |
04-17 |
c.291+6437_2334-3424del |
r.? |
p.? |
- |
pathogenic |
g.100057231_100201680del |
g.99045003_99189452del |
Del 4-16: 100126407 - 100270856 bp (hg18) |
- |
VPS13B_000011 |
1 COH1 family (com-het); Deletion of exons 4-17 |
PubMed: Balikova et al. 2009 |
- |
- |
SUMMARY record |
yes |
- |
- |
- |
- |
Anne Polvi |
?/. |
1 |
- |
c.291+28132C>A |
r.(?) |
p.(=) |
- |
association |
g.100078926C>A |
g.99066698C>A |
- |
- |
VPS13B_000378 |
associated with facial morphology (nose) |
Huang 2020 (submitted) |
- |
rs11988731 |
Germline |
- |
- |
- |
- |
- |
Yin Huang |
-?/. |
1 |
- |
c.292-3T>C |
r.spl? |
p.? |
- |
likely benign |
g.100108537T>C |
- |
VPS13B(NM_017890.5):c.292-3T>C |
- |
COX6C_000126 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_AMC |
+/+, +/. |
2 |
3i_13i |
c.(291+1_292-1)_(1843+1_1844-1)dup |
r.(292_1843dup), r.? |
p.? |
- |
pathogenic (recessive) |
g.(100050795_100108539)_(100155394_100160068)dup |
- |
dup ex4-13 |
- |
VPS13B_000013 |
1 COH1 family (com-het) |
PubMed: Parri 2010 |
- |
- |
Germline, SUMMARY record |
yes |
- |
- |
- |
- |
Johan den Dunnen, Anne Polvi |
+/+, +/. |
3 |
3i_16i |
c.(291+1_292-1)_(2333+1_2334-1)del |
r.(292_2333del), r.? |
p.(Asp99Glnfs*9), p.? |
- |
pathogenic (recessive) |
g.(100050795_100108539)_(100182392_100205103)del |
- |
del ex4-16 |
- |
VPS13B_000015 |
1 COH1 family (com-het) |
PubMed: Parri 2010 |
- |
- |
Germline, SUMMARY record |
yes |
- |
- |
- |
- |
Johan den Dunnen, Anne Polvi |
-?/. |
1 |
- |
c.358A>G |
r.(?) |
p.(Ile120Val) |
- |
likely benign |
g.100108606A>G |
g.99096378A>G |
VPS13B(NM_017890.4):c.358A>G (p.I120V) |
- |
VPS13B_000178 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Rotterdam |
+/+, +/. |
2 |
4 |
c.401_402insT |
r.(404dupu), r.(?) |
p.(Leu135Phefs*11), p.(Pro136Thrfs*10) |
- |
pathogenic (recessive) |
g.100108649_100108650insT |
g.99096421_99096422insT |
402insT |
- |
VPS13B_000014 |
1 COH1 family (com-het) |
PubMed: Parri 2010 |
- |
- |
Germline, SUMMARY record |
yes |
- |
- |
- |
- |
Johan den Dunnen, Anne Polvi |
+/. |
1 |
4i |
c.412+1G>T |
r.spl |
p.? |
- |
pathogenic |
g.100108661G>T |
g.99096433G>T |
NM_152564: c.412+1G>T |
- |
VPS13B_000173 |
- |
PubMed: Karaca 2015 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
./. |
1 |
- |
c.412+3161_580+208del |
r.(?) |
p.(Gly138_Ser193del) |
- |
pathogenic |
g.100111821_100115556del |
g.99099593_99103328del |
- |
- |
VPS13B_000169 |
decreased gene dosage |
PubMed: DDDS 2015, Journal: DDDS 2015 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/+?, +/. |
3 |
4i |
c.413-2A>G |
r.spl, r.spl? |
p.?, p? |
- |
likely pathogenic (recessive), pathogenic (recessive) |
g.100115179A>G |
g.99102951A>G |
IVS4-2A>G |
- |
VPS13B_000012 |
1 COH1 family (com-het) |
PubMed: Parri 2010 |
- |
- |
Germline, SUMMARY record |
yes |
- |
- |
- |
- |
Johan den Dunnen, Anne Polvi |
+/+ |
1 |
06-15 |
c.413-?_2013+?del |
r.(413_2013del) |
p.(Gly138Glufs*4) |
- |
pathogenic |
g.(?_100115181)_(100160238_?)del |
- |
c.413_2013del: p.Gly138GlufsX4 (exon 5-14) |
- |
VPS13B_000016 |
1 Iranian COH1 family (hom); Deletion of exons 6-15 |
PubMed: Sheifert et al. 2008 |
- |
- |
SUMMARY record |
yes |
0/50 CON |
- |
- |
- |
Anne Polvi |
+/+, +?/. |
2 |
06 |
c.436C>T |
r.(?) |
p.(Arg146*), p.(Arg146Ter) |
- |
likely pathogenic, pathogenic |
g.100115204C>T |
g.99102976C>T |
EX5, VPS13B(NM_017890.5):c.436C>T (p.R146*) |
- |
VPS13B_000138 |
1 French COH1 patient (com-het), VKGL data sharing initiative Nederland |
PubMed: El Chehadeh et al. 2010 |
- |
- |
CLASSIFICATION record, SUMMARY record |
yes |
- |
- |
- |
- |
Anne Polvi, VKGL-NL_VUmc |
+/+ |
1 |
06 |
c.467_470delATAA |
r.467_470delauaa |
p.Asn156Ilefs*4 |
- |
pathogenic |
g.100115235_100115238del |
g.99103007_99103010del |
c.463_466delATAA: p.Asn156fsX4 |
- |
VPS13B_000018 |
1 British COH1 patient (hom) |
PubMed: Kolehmainen et al. 2004 |
- |
- |
SUMMARY record |
yes |
- |
- |
- |
- |
Anne Polvi |
+?/. |
1 |
- |
c.468_471del |
r.(?) |
p.(Asn157Serfs*3) |
- |
likely pathogenic |
g.100115236_100115239del |
g.99103008_99103011del |
VPS13B;NM_017890.4, c.[468_471del];[10156dup];p.[(Asn157Serfs*3)];[(Thr3386Asnfs*3)] |
- |
VPS13B_000414 |
compound heterozygous |
PubMed: Jiman 2020 |
- |
- |
Unknown |
? |
- |
- |
- |
- |
LOVD |
+/+ |
1 |
06 |
c.478_481delCTAA |
r.(?) |
p.(Leu160Asnfs*21) |
- |
pathogenic |
g.100115246_100115249del |
g.99103018_99103021del |
c.477_480delACTA (p.I159fsX21) |
- |
VPS13B_000141 |
1 French COH1 patient (com-het) |
PubMed: El Chehadeh et al. 2010 |
- |
- |
SUMMARY record |
yes |
- |
- |
- |
- |
Anne Polvi |
?/. |
1 |
- |
c.505C>A |
r.(?) |
p.(Leu169Ile) |
- |
VUS |
g.100115273C>A |
g.99103045C>A |
VPS13B(NM_017890.4):c.505C>A (p.L169I) |
- |
COX6C_000096 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Rotterdam |
-?/., ?/. |
3 |
- |
c.560G>A |
r.(?) |
p.(Arg187His) |
- |
likely benign, VUS |
g.100115328G>A |
g.99103100G>A |
VPS13B(NM_017890.4):c.560G>A (p.R187H), VPS13B(NM_017890.5):c.560G>A (p.R187H) |
- |
VPS13B_000179 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Rotterdam, VKGL-NL_Groningen, VKGL-NL_Nijmegen |
?/. |
2 |
- |
c.565T>C |
r.(?) |
p.(Phe189Leu) |
- |
VUS |
g.100115333T>C |
g.99103105T>C |
VPS13B(NM_017890.4):c.565T>C (p.F189L), VPS13B(NM_017890.5):c.565T>C (p.F189L) |
- |
VPS13B_000180 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Rotterdam, VKGL-NL_Groningen |
-/. |
1 |
- |
c.581-17T>C |
r.(=) |
p.(=) |
- |
benign |
g.100123309T>C |
g.99111081T>C |
VPS13B(NM_017890.4):c.581-17T>C |
- |
VPS13B_000181 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Utrecht |
-?/. |
2 |
- |
c.581-10T>C |
r.(=) |
p.(=) |
- |
likely benign |
g.100123316T>C |
g.99111088T>C |
VPS13B(NM_017890.4):c.581-10T>C |
- |
COX6C_000110 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Rotterdam, VKGL-NL_Utrecht |
-?/. |
1 |
- |
c.581-8C>T |
r.(=) |
p.(=) |
- |
likely benign |
g.100123318C>T |
g.99111090C>T |
VPS13B(NM_015243.2):c.581-8C>T (p.(=)) |
- |
COX6C_000004 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Leiden |
+/+, +/. |
6 |
5i_16i |
c.(580+1_581-1)_(2333+1_2334-1)del |
r.(581_2333del), r.? |
p.(Ala194Glyfs*9), p.? |
- |
pathogenic (recessive) |
g.(100115349_100123325)_(100182392_100205103)del |
- |
del ex6-16 |
- |
VPS13B_000017 |
deletion on shared haplotype, 1 more item |
PubMed: Bugiani 2008, PubMed: Parri 2010, PubMed: Parri 2010 |
- |
- |
Germline, SUMMARY record |
yes |
- |
- |
- |
- |
Johan den Dunnen, Anne Polvi |
?/. |
4 |
- |
c.584C>G |
r.(?) |
p.(Thr195Ser) |
- |
VUS |
g.100123329C>G |
- |
1 more item |
- |
COX6C_000127 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Leiden, VKGL-NL_Rotterdam, VKGL-NL_Groningen, VKGL-NL_AMC |
+/+ |
1 |
07 |
c.626_627delCA |
r.(626_627delca) |
p.(Thr209Serfs*4) |
- |
pathogenic |
g.100123371_100123372del |
g.99111143_99111144del |
c.625_626delAC: p.Thr209SerfsX4 (exon 6) |
- |
VPS13B_000019 |
1 Belgian/ Italian COH1 family (com-het) |
PubMed: Sheifert et al. 2008 |
- |
- |
SUMMARY record |
yes |
0/50 CON |
- |
- |
- |
Anne Polvi |
-?/. |
1 |
- |
c.690C>T |
r.(?) |
p.(Tyr230=) |
- |
likely benign |
g.100123435C>T |
g.99111207C>T |
VPS13B(NM_017890.4):c.690C>T (p.Y230=) |
- |
VPS13B_000182 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Rotterdam |
-?/. |
1 |
- |
c.750A>G |
r.(?) |
p.(Pro250=) |
- |
likely benign |
g.100123495A>G |
- |
VPS13B(NM_017890.5):c.750A>G (p.P250=) |
- |
COX6C_000128 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_AMC |
-/. |
1 |
- |
c.762+19del |
r.(=) |
p.(=) |
- |
benign |
g.100123526del |
g.99111298del |
VPS13B(NM_017890.4):c.762+19delT |
- |
VPS13B_000183 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Utrecht |
-/. |
1 |
- |
c.763-89C>A |
r.(=) |
p.(=) |
- |
benign |
g.100127839C>A |
g.99115611C>A |
VPS13B(NM_017890.4):c.763-89C>A |
- |
COX6C_000005 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Utrecht |
-/. |
1 |
- |
c.763-78C>G |
r.(=) |
p.(=) |
- |
benign |
g.100127850C>G |
g.99115622C>G |
VPS13B(NM_017890.4):c.763-78C>G |
- |
VPS13B_000184 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Utrecht |
-?/. |
1 |
- |
c.874T>G |
r.(?) |
p.(Phe292Val) |
- |
likely benign |
g.100128039T>G |
g.99115811T>G |
VPS13B(NM_015243.2):c.874T>G (p.(Phe292Val)) |
- |
COX6C_000006 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Leiden |
?/. |
1 |
- |
c.885C>T |
r.(?) |
p.(Gly295=) |
- |
VUS |
g.100128050C>T |
g.99115822C>T |
VPS13B(NM_017890.4):c.885C>T (p.G295=) |
- |
COX6C_000007 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Rotterdam |
+/. |
1 |
- |
c.901_904del |
r.(?) |
p.(Thr301ValfsTer7) |
- |
pathogenic |
g.100128066_100128069del |
- |
VPS13B(NM_017890.5):c.901_904delACTT (p.T301Vfs*7) |
- |
VPS13B_000353 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_VUmc |
+/+ |
1 |
08 |
c.916_917delGA |
r.(916_917delga) |
p.(Asp306Tyrfs*9) |
- |
pathogenic |
g.100128081_100128082del |
g.99115853_99115854del |
c.916_917delGA: p.Asp306TyrfsX9 (exon 7) |
- |
VPS13B_000020 |
1 Belgian COH1 family (com-het), 2 French siblings with COH1 (com-het) |
PubMed: Sheifert et al. 2008, PubMed: El Chehadeh et al. 2010 |
- |
- |
SUMMARY record |
yes |
0/50 CON |
- |
- |
- |
Anne Polvi |
-?/. |
1 |
- |
c.921G>A |
r.(?) |
p.(Met307Ile) |
- |
likely benign |
g.100128086G>A |
g.99115858G>A |
VPS13B(NM_017890.4):c.921G>A (p.M307I) |
- |
VPS13B_000185 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Rotterdam |
+?/. |
1 |
- |
c.938-8A>G |
r.(=) |
p.(=) |
- |
likely pathogenic |
g.100133397A>G |
g.99121169A>G |
- |
- |
VPS13B_000354 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Nijmegen |
+?/. |
2 |
- |
c.979C>T |
r.(?) |
p.(Gln327*) |
- |
likely pathogenic |
g.100133446C>T |
g.99121218C>T |
VPS13B, variant 1: c.979C>T/p.Q327*, variant 2: c.7753G>A/p.E2585K |
- |
VPS13B_000419 |
possibly solved, compound heterozygous |
PubMed: Weisschuh 2020 |
- |
- |
Unknown |
? |
- |
- |
- |
- |
LOVD |
-?/. |
3 |
- |
c.983A>G |
r.(?) |
p.(His328Arg) |
- |
likely benign |
g.100133450A>G |
g.99121222A>G |
VPS13B(NM_017890.4):c.983A>G (p.H328R), VPS13B(NM_017890.5):c.983A>G (p.H328R) |
- |
COX6C_000008 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Rotterdam, VKGL-NL_Utrecht, VKGL-NL_AMC |
?/. |
1 |
- |
c.1000T>C |
r.(?) |
p.(Tyr334His) |
- |
VUS |
g.100133467T>C |
g.99121239T>C |
- |
- |
VPS13B_000355 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Nijmegen |
+/+ |
1 |
9a |
c.1006C>T |
r.(?) |
p.(Gln336*) |
- |
pathogenic |
g.100133473C>T |
g.99121245C>T |
c.1006C>T (p.Q336X) |
- |
VPS13B_000142 |
1 French COH1 family (com-het) |
PubMed: El Chehadeh et al. 2010 |
- |
- |
SUMMARY record |
yes |
- |
- |
- |
- |
Anne Polvi |
-?/. |
2 |
- |
c.1041A>G |
r.(?) |
p.(Ser347=) |
- |
likely benign |
g.100133508A>G |
g.99121280A>G |
VPS13B(NM_017890.4):c.1041A>G (p.S347=), VPS13B(NM_017890.5):c.1041A>G (p.S347=) |
- |
VPS13B_000366 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Rotterdam, VKGL-NL_AMC |
-?/. |
1 |
- |
c.1081G>A |
r.(?) |
p.(Asp361Asn) |
- |
likely benign |
g.100133548G>A |
g.99121320G>A |
VPS13B(NM_017890.4):c.1081G>A (p.D361N) |
- |
COX6C_000097 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Rotterdam |
?/. |
1 |
- |
c.1108G>C |
r.(?) |
p.(Asp370His) |
- |
VUS |
g.100133575G>C |
g.99121347G>C |
VPS13B(NM_017890.4):c.1108G>C (p.D370H) |
- |
VPS13B_000186 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Rotterdam |
+/+ |
1 |
10-13 |
c.1206+5_1652-27dup |
r.spl? |
p.? |
- |
pathogenic |
g.100133678_100155175dup |
g.99121450_99142947dup |
DupEX9-12 (g.100202854_100224351dup) |
- |
VPS13B_000148 |
2 French siblings with COH1 (com-het) |
PubMed: El Chehadeh-Djebbar et al. 2011 |
- |
- |
SUMMARY record |
yes |
- |
- |
- |
- |
Anne Polvi |
-/. |
3 |
- |
c.1206+33T>G |
r.(=) |
p.(=) |
- |
benign |
g.100133706T>G |
g.99121478T>G |
VPS13B(NM_181661.2):c.1239T>G (p.Y413*), VPS13B(NM_181661.3):c.1239T>G (p.Y413*) |
- |
VPS13B_000187 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Rotterdam, VKGL-NL_Groningen, VKGL-NL_Utrecht |
+/+ |
1 |
10-21 |
c.1207-2_2824+52509del |
r.(1207_2824del) |
p.(Leu403Valfs*11) |
- |
pathogenic |
g.100146858_100339991del |
g.99134630_99327763del |
193 kb del: exons 9-19: chr8(hg18): 100216034...100409167; c.1207-2824del/p.L403fsX11 |
- |
VPS13B_000021 |
1 more item |
Rivera-Brugués 2011 |
- |
- |
SUMMARY record |
yes |
0/1612 CON |
- |
- |
- |
Anne Polvi |
+/+ |
1 |
10 |
c.1219C>T |
r.(1219c>u) |
p.(Gln407*) |
- |
pathogenic |
g.100146872C>T |
g.99134644C>T |
1219C>T: Q407X |
- |
VPS13B_000022 |
1 Saudi Arabian COH1 family (hom) |
PubMed: Mochida et al. 2004 |
- |
- |
SUMMARY record |
yes |
0/82 CON |
- |
- |
- |
Anne Polvi |
+/+ |
1 |
10 |
c.1221delA |
r.(?) |
p.(Val408Leufs*11) |
- |
pathogenic |
g.100146874del |
g.99134646del |
c.1220delA (p.Q407fsX418) |
- |
VPS13B_000140 |
1 French COH1 patient (com-het) |
PubMed: El Chehadeh et al. 2010 |
- |
- |
SUMMARY record |
yes |
- |
- |
- |
- |
Anne Polvi |
+/+ |
1 |
10 |
c.1225G>T |
r.(1225g>u) |
p.(Glu409*) |
- |
pathogenic |
g.100146878G>T |
g.99134650G>T |
c.1225g.t ( p.Glu409X ) |
- |
VPS13B_000023 |
1 Palestinian COH1 family (het) |
PubMed: Taban et al. 2007 |
- |
- |
SUMMARY record |
yes |
- |
- |
- |
- |
Anne Polvi |
+?/., ?/. |
5 |
- |
c.1248G>T |
r.(?) |
p.(Gln416His) |
ACMG |
likely pathogenic, VUS |
g.100146901G>T |
g.99134673G>T |
VPS13B(NM_017890.4):c.1248G>T (p.Q416H), VPS13B(NM_152564.4):c.1248G>T (p.(Gln416His)), 1 more item |
- |
COX6C_000009 |
conflicting interpretations of pathogenicity; 5 heterozygous, no homozygous; Clinindb (India), 1 more item |
PubMed: Narang 2020, Journal: Narang 2020, PubMed: Perea-Romero 2021 |
- |
rs143024324 |
CLASSIFICATION record, Germline |
yes |
5/2794 individuals |
- |
- |
- |
VKGL-NL_Leiden, VKGL-NL_Rotterdam, VKGL-NL_Nijmegen, Mohammed Faruq |
+/+ |
1 |
10 |
c.1269_1273delATTGT |
r.(1269_1273delauugu) |
p.(Cys425Glyfs*8) |
- |
pathogenic |
g.100146922_100146926del |
g.99134694_99134698del |
c.1269_1273delATTGT: p.Cys425GlyfsX8 (exon 9) |
- |
VPS13B_000024 |
1 Belgian/ Italian COH1 family (com-het) |
PubMed: Sheifert et al. 2008 |
- |
- |
SUMMARY record |
yes |
0/50 CON |
- |
- |
- |
Anne Polvi |
-/., -?/. |
3 |
- |
c.1293T>G |
r.(?) |
p.(Thr431=) |
- |
benign, likely benign |
g.100146946T>G |
g.99134718T>G |
VPS13B(NM_017890.4):c.1293T>G (p.T431=), VPS13B(NM_152564.5):c.1293T>G (p.(Thr431=)) |
- |
VPS13B_000188 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Leiden, VKGL-NL_Rotterdam, VKGL-NL_Utrecht |
-?/. |
1 |
- |
c.1302+12A>G |
r.(=) |
p.(=) |
- |
likely benign |
g.100146967A>G |
g.99134739A>G |
VPS13B(NM_017890.4):c.1302+12A>G |
- |
VPS13B_000189 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Rotterdam |
-?/. |
3 |
- |
c.1303-9G>A |
r.(=) |
p.(=) |
- |
likely benign |
g.100147234G>A |
g.99135006G>A |
VPS13B(NM_017890.4):c.1303-9G>A, VPS13B(NM_017890.5):c.1303-9G>A |
- |
COX6C_000098 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Rotterdam, VKGL-NL_Utrecht, VKGL-NL_AMC |
?/. |
1 |
- |
c.1343G>A |
r.(?) |
p.(Gly448Glu) |
- |
VUS |
g.100147283G>A |
- |
VPS13B(NM_017890.4):c.1343G>A (p.G448E) |
- |
COX6C_000129 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Rotterdam |
?/. |
1 |
- |
c.1354T>G |
r.(?) |
p.(Cys452Gly) |
- |
VUS |
g.100147294T>G |
g.99135066T>G |
VPS13B(NM_015243.2):c.1354T>G (p.(Cys452Gly)) |
- |
VPS13B_000190 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Leiden |
+/. |
1 |
10i_17i |
c.1426-32_2516-16303del |
r.(del) |
p.(Glu476Valfs*2) |
- |
pathogenic |
g.100147792_100270123del |
g.99135564_99257895del |
- |
- |
VPS13B_000167 |
- |
PubMed: Gilissen 2014 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
Johan den Dunnen |
-?/. |
2 |
- |
c.1440C>T |
r.(?) |
p.(Phe480=) |
- |
likely benign |
g.100147838C>T |
g.99135610C>T |
VPS13B(NM_017890.4):c.1440C>T (p.F480=) |
- |
COX6C_000010 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Rotterdam, VKGL-NL_Utrecht |
+?/. |
1 |
- |
c.1443_1444del |
r.(?) |
p.(Ile481Metfs*3) |
- |
likely pathogenic |
g.100147841_100147842del |
- |
VPS13B(NM_017890.5):c.1443_1444delTT (p.I481Mfs*3) |
- |
COX6C_000130 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_AMC |
-?/. |
2 |
- |
c.1463C>T |
r.(?) |
p.(Thr488Met) |
- |
likely benign |
g.100147861C>T |
g.99135633C>T |
VPS13B(NM_015243.2):c.1463C>T (p.(Thr488Met)), VPS13B(NM_017890.5):c.1463C>T (p.T488M) |
- |
VPS13B_000191 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Leiden, VKGL-NL_AMC |
+/+, +?/. |
2 |
12 |
c.1504C>T |
r.(?), r.1504c>u |
p.(Arg502*), p.Arg502* |
- |
likely pathogenic (recessive), pathogenic |
g.100147902C>T |
g.99135674C>T |
c.1504C>T: p.Arg502X |
- |
VPS13B_000025 |
1 Brazilian COH1 family (het), 1 English/Scottish COH1 family (com-het) |
PubMed: Hennies et al. 2004, PubMed: Seifert et al. 2006, PubMed: Tsangaris 2011 |
- |
rs180177354 |
Germline, SUMMARY record |
yes |
0/75 CON |
- |
- |
- |
Johan den Dunnen, Anne Polvi |
+/. |
1 |
- |
c.1512del |
r.(?) |
p.(Glu505Lysfs*23) |
- |
pathogenic (recessive) |
g.100147910del |
g.99135682del |
- |
- |
VPS13B_000386 |
- |
PubMed: Sanchez-Navarro 2018 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
?/. |
1 |
- |
c.1520A>G |
r.(?) |
p.(Asn507Ser) |
- |
VUS |
g.100147918A>G |
- |
VPS13B(NM_017890.4):c.1520A>G (p.N507S) |
- |
COX6C_000131 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Rotterdam |
?/. |
1 |
- |
c.1528C>T |
r.(?) |
p.(Arg510Cys) |
- |
VUS |
g.100147926C>T |
g.99135698C>T |
- |
- |
VPS13B_000371 |
1 heterozygous, no homozygous; Clinindb (India) |
PubMed: Narang 2020, Journal: Narang 2020 |
- |
rs139141291 |
Germline |
- |
1/2795 individuals |
- |
- |
- |
Mohammed Faruq |
-?/. |
2 |
- |
c.1552A>T |
r.(?) |
p.(Thr518Ser) |
- |
likely benign |
g.100147950A>T |
g.99135722A>T |
VPS13B(NM_015243.2):c.1552A>T (p.(Thr518Ser)), VPS13B(NM_017890.4):c.1552A>T (p.T518S) |
- |
COX6C_000012 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Leiden, VKGL-NL_Rotterdam |
?/. |
3 |
- |
c.1559A>G |
r.(?) |
p.(His520Arg) |
- |
VUS |
g.100147957A>G |
g.99135729A>G |
1 more item |
- |
VPS13B_000192 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Leiden, VKGL-NL_Rotterdam, VKGL-NL_Groningen |
+/+, +?/. |
2 |
12 |
c.1563G>A |
r.(?), r.[1563G>A, spl] |
p.(Lys521=), p.[=, Lys521fs*20] |
- |
likely pathogenic, pathogenic |
g.100147961G>A |
g.99135733G>A |
VPS13B, variant 1: c.1563G>A/p.?, variant 2 :Deletion exon 46-50, 1 more item |
- |
VPS13B_000026 |
1 German/English COH1 family (com-het); Splice site mutation, solved, compound heterozygous |
PubMed: Seifert et al. 2006, PubMed: Weisschuh 2020 |
- |
rs180177355 |
Germline, SUMMARY record |
yes |
- |
- |
- |
- |
Anne Polvi |
-?/. |
1 |
- |
c.1639A>G |
r.(?) |
p.(Thr547Ala) |
- |
likely benign |
g.100148968A>G |
- |
VPS13B(NM_017890.5):c.1639A>G (p.T547A) |
- |
COX6C_000132 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_AMC |
-/. |
1 |
- |
c.1656C>A |
r.(?) |
p.(Ser552=) |
- |
benign |
g.100155206C>A |
- |
VPS13B(NM_017890.5):c.1656C>A (p.S552=) |
- |
COX6C_000133 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_AMC |
?/. |
1 |
- |
c.1700G>A |
r.(?) |
p.(Gly567Glu) |
- |
VUS |
g.100155250G>A |
g.99143022G>A |
VPS13B(NM_017890.4):c.1700G>A (p.G567E) |
- |
VPS13B_000193 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Rotterdam |
+/+ |
1 |
14 |
c.1734delT |
r.(?) |
p.(Ile579*) |
- |
pathogenic |
g.100155284del |
g.99143056del |
c.1733delT |
- |
VPS13B_000152 |
One COH1 patient (com-het) |
PubMed: El Chehadeh-Djebbar et al. 2013 |
- |
- |
SUMMARY record |
? |
- |
- |
- |
- |
Anne Polvi |
+?/+?, -?/., ?/. |
4 |
14 |
c.1768G>A |
r.(1768g>a), r.(?) |
p.(Ala590Thr) |
- |
likely benign, likely pathogenic, VUS |
g.100155318G>A |
g.99143090G>A |
c.1768G>A: p.A590T (exon 13), 1 more item |
- |
VPS13B_000027 |
1 heterozygous, no homozygous; Clinindb (India), 1 Italian COH1 family (com-het), 1 more item |
PubMed: Katzaki et al. 2007, PubMed: Narang 2020, Journal: Narang 2020 |
- |
rs140601319 |
CLASSIFICATION record, Germline, SUMMARY record |
yes |
0/50 CON, 1/2795 individuals |
- |
- |
- |
Anne Polvi, VKGL-NL_Rotterdam, VKGL-NL_AMC, Mohammed Faruq |
-/. |
1 |
- |
c.1786A>C |
r.(?) |
p.(Lys596Gln) |
- |
benign |
g.100155336A>C |
g.99143108A>C |
VPS13B(NM_017890.4):c.1786A>C (p.K596Q) |
- |
VPS13B_000194 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Utrecht |
-?/. |
4 |
- |
c.1832G>A |
r.(?) |
p.(Arg611Lys) |
- |
likely benign |
g.100155382G>A |
g.99143154G>A |
1 more item |
- |
VPS13B_000195 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Leiden, VKGL-NL_Rotterdam, VKGL-NL_Utrecht, VKGL-NL_AMC |
-?/. |
1 |
- |
c.1843+10T>C |
r.(=) |
p.(=) |
- |
likely benign |
g.100155403T>C |
- |
VPS13B(NM_017890.4):c.1843+10T>C |
- |
VPS13B_000393 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Rotterdam |
-/. |
1 |
- |
c.1843+16A>G |
r.(=) |
p.(=) |
- |
benign |
g.100155409A>G |
- |
VPS13B(NM_017890.5):c.1843+16A>G |
- |
COX6C_000134 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_AMC |
-?/., ?/. |
3 |
- |
c.1864A>G |
r.(?) |
p.(Thr622Ala) |
- |
likely benign, VUS |
g.100160089A>G |
- |
1 more item |
- |
COX6C_000135 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Leiden, VKGL-NL_Rotterdam, VKGL-NL_AMC |
?/. |
1 |
- |
c.1888G>A |
r.(?) |
p.(Ala630Thr) |
- |
VUS |
g.100160113G>A |
g.99147885G>A |
VPS13B(NM_017890.4):c.1888G>A (p.A630T) |
- |
COX6C_000013 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Rotterdam |
+?/. |
2 |
- |
c.1915C>T |
r.(?) |
p.(Arg639*) |
- |
likely pathogenic, likely pathogenic (recessive) |
g.100160140C>T |
g.99147912C>T |
NM_152564.4:c.1915C>T |
- |
VPS13B_000387 |
- |
PubMed: Nambot 2018, PubMed: Stone 2017 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
-?/. |
2 |
- |
c.1981G>A |
r.(?) |
p.(Asp661Asn) |
- |
likely benign |
g.100160206G>A |
- |
VPS13B(NM_017890.4):c.1981G>A (p.D661N), VPS13B(NM_017890.5):c.1981G>A (p.D661N) |
- |
COX6C_000136 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Utrecht, VKGL-NL_AMC |
-/. |
1 |
- |
c.2013+60_2013+61del |
r.(=) |
p.(=) |
- |
benign |
g.100160298_100160299del |
g.99148070_99148071del |
VPS13B(NM_017890.4):c.2013+60_2013+61delTT |
- |
VPS13B_000197 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Utrecht |
-/. |
1 |
- |
c.2013+61del |
r.(=) |
p.(=) |
- |
benign |
g.100160299del |
g.99148071del |
VPS13B(NM_017890.4):c.2013+61delT |
- |
COX6C_000014 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Utrecht |
-/. |
1 |
- |
c.2013+100G>T |
r.(=) |
p.(=) |
- |
benign |
g.100160338G>T |
g.99148110G>T |
VPS13B(NM_017890.4):c.2013+100G>T |
- |
VPS13B_000198 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Utrecht |
-/. |
1 |
- |
c.2014-97C>G |
r.(=) |
p.(=) |
- |
benign |
g.100168680C>G |
g.99156452C>G |
VPS13B(NM_017890.4):c.2014-97C>G |
- |
VPS13B_000199 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Utrecht |
+/., +?/+? |
3 |
14i |
c.2014-2A>G |
r.spl, r.spl? |
p.?, p? |
- |
likely pathogenic (recessive), pathogenic, pathogenic (recessive) |
g.100168775A>G |
g.99156547A>G |
IVS14-2A>G, VPS13B(NM_017890.4):c.2014-2A>G |
- |
VPS13B_000028 |
1 COH1 family (com-het), VKGL data sharing initiative Nederland |
PubMed: Parri 2010 |
- |
- |
CLASSIFICATION record, Germline, SUMMARY record |
yes |
- |
- |
- |
- |
Johan den Dunnen, Anne Polvi, VKGL-NL_Utrecht |