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
|
+?/. |
3 |
- |
- |
r.? |
p.? |
- |
likely pathogenic (dominant) |
g.30859428_31701585del, g.31031082_31704996del, g.31140508_31738346del |
g.30837881_31680038del, g.31009535_31683449del, g.31118961_31716799del |
g.30837881_31680038del, g.31009535_31683449del, g.31118961_31716799del |
- |
PAX6_000878 |
del PAX6 downstream regulatory region |
PubMed: Hall 2024 |
- |
- |
Germline/De novo (untested) |
- |
- |
- |
- |
- |
Johan den Dunnen |
+?/. |
1 |
13_ |
c.*180_*5142{0} |
r.? |
p.? |
- |
likely pathogenic (dominant) |
g.(?_31675779)_(31685705_31811302)del |
g.(?_31654231)_(31664157_31789754)del |
del SIMO |
- |
PAX6_000880 |
deletion of SIMO enhancer |
PubMed: Hall 2024 |
- |
- |
Germline/De novo (untested) |
- |
- |
- |
- |
- |
Johan den Dunnen |
+?/. |
1 |
_1_13_ |
c.-533_*5142inv{1} |
r.0? |
p.0? |
- |
likely pathogenic (dominant) |
g.31723012_36615048inv |
g.31701464_36593500inv |
g.31701464_36593500inv |
- |
PAX6_000881 |
4.9Mb inversion separating PAX6 from downstream regulatory region; breakpoints in ELP4 and RAG2 |
PubMed: Hall 2024 |
- |
- |
Germline/De novo (untested) |
- |
- |
- |
- |
- |
Johan den Dunnen |
+?/. |
1 |
_1_13_ |
c.-533_*5142{0} |
r.0 |
p.0 |
- |
likely pathogenic (dominant) |
g.31735622_31926723del |
g.31714074_31905175del |
g.31714074_31905175del |
- |
PAX6_000882 |
c.-533_*5142{0} |
PubMed: Hall 2024 |
- |
- |
Germline/De novo (untested) |
- |
- |
- |
- |
- |
Johan den Dunnen |
+?/. |
1 |
11i_13_ |
c.1032+734_*5142{1} |
r.? |
p.? |
- |
likely pathogenic (dominant) |
g.24500578_31814252inv |
g.24479030_31792704inv |
g.24479030_31792704inv |
- |
PAX6_000879 |
7.3Mb inversion 11p13-11p14.3 with intragenic PAX6 breakpoint |
PubMed: Hall 2024 |
- |
- |
Germline/De novo (untested) |
- |
- |
- |
- |
- |
Johan den Dunnen |
+?/. |
1 |
4i_13_ |
c.11-907_*5142{0} |
r.? |
p.? |
- |
likely pathogenic (dominant) |
g.31784515_31825289del |
g.31762967_31803741del |
g.31762967_31803741del |
- |
PAX6_000883 |
deletion incl. ELP4 and PAX6 |
PubMed: Hall 2024 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
Johan den Dunnen |
+?/. |
1 |
8i_13_ |
c.524-1228_*12912del |
r.? |
p.? |
- |
likely pathogenic (dominant) |
g.31798570_31817564del |
g.31777022_31796016del |
g.31777022_31796016del |
- |
PAX6_000884 |
C-termini of both ELP4 and PAX6 (intron 8 onwards) |
PubMed: Hall 2024 |
- |
- |
Germline/De novo (untested) |
- |
- |
- |
- |
- |
Johan den Dunnen |
+?/. |
1 |
_1_4i |
c.-533_10+902inv{1} |
r.0? |
p.0? |
- |
likely pathogenic (dominant) |
g.[31827048_31833130inv;31833131_31863605del] |
g.[31805500_31811582inv;31811583_31842057del] |
46,XX t(1;9)(p36.1;q22) |
- |
PAX6_000890 |
N-terminus of PAX6 up to intron 4 |
PubMed: Hall 2024 |
- |
- |
De novo |
- |
- |
- |
- |
- |
Johan den Dunnen |
-?/. |
1 |
- |
c.-134A>T |
r.(?) |
p.(=) |
- |
likely benign |
g.31832381T>A |
- |
- |
- |
PAX6_000814 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Nijmegen |
+?/. |
1 |
- |
c.-133_-132del |
r.? |
p.? |
ACMG |
VUS |
g.31832379_31832380del |
g.31810831_31810832del |
-133_-132delTA |
- |
PAX6_000805 |
- |
- |
- |
- |
Germline/De novo (untested) |
- |
- |
- |
- |
- |
Jinu Han |
-/., -?/. |
4 |
- |
c.-129+9G>A |
r.(=) |
p.(=) |
- |
benign, likely benign |
g.31832367C>T |
g.31810819C>T |
PAX6(NM_001368905.2):c.-910+9G>A |
- |
PAX6_000772 |
1 homozygous; Clinindb (India), 57 heterozygous; Clinindb (India), 1 more item |
PubMed: Narang 2020, Journal: Narang 2020 |
- |
rs56139994 |
CLASSIFICATION record, Germline |
- |
1/2794 individuals, 57/2794 individuals |
- |
- |
- |
VKGL-NL_Nijmegen, VKGL-NL_AMC, Mohammed Faruq |
-?/. |
1 |
- |
c.-128-358_-128-357insAC |
r.(=) |
p.(=) |
- |
likely benign |
g.31828831_31828832insTG |
- |
PAX6(NM_001368891.2):c.-129+7_-129+8insAC |
- |
PAX6_000876 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Leiden |
-?/. |
1 |
- |
c.-128-7G>T |
r.(=) |
p.(=) |
- |
likely benign |
g.31828480C>A |
- |
- |
- |
PAX6_000858 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Nijmegen |
+/., +?/. |
3 |
2i |
c.-128-2del |
r.spl, r.spl? |
p.? |
- |
likely pathogenic (dominant), pathogenic, pathogenic (dominant) |
g.31828475del |
g.31806927del |
IVS2-2del |
- |
PAX6_000857 |
ACMG PM2, PS4 mod, PP3, PP4 mod, ACMG PM2, PS4 mod, PP3, PS2, PP4 mod, 1 more item |
PubMed: Hall 2024 |
VCV000430969.2 |
- |
CLASSIFICATION record, De novo, Germline/De novo (untested) |
- |
- |
- |
- |
- |
Johan den Dunnen, VKGL-NL_Nijmegen |
+/. |
1 |
- |
c.-52+1G>A |
r.spl? |
p.? |
- |
pathogenic |
g.31828396C>T |
- |
- |
- |
PAX6_000784 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Nijmegen |
+?/. |
1 |
3i |
c.-52+1G>C |
r.spl |
p.? |
- |
likely pathogenic (dominant) |
g.31828396C>G |
g.31806848C>G |
IVS3+1G>C |
- |
PAX6_000891 |
ACMG PM2, PS1 supp, PP3, PP4 mod* |
PubMed: Hall 2024 |
- |
- |
Germline/De novo (untested) |
- |
- |
- |
- |
- |
Johan den Dunnen |
+?/. |
1 |
3i |
c.-52+1G>T |
r.spl |
p.? |
- |
likely pathogenic (dominant) |
g.31828396C>A |
g.31806848C>A |
IVS3+1G>T |
- |
PAX6_000892 |
ACMG PM2, PS4 supp, PP3, PS1 supp, PP4 mod* |
PubMed: Hall 2024 |
- |
- |
Germline/De novo (untested) |
- |
- |
- |
- |
- |
Johan den Dunnen |
+?/. |
1 |
- |
- |
r.? |
p.? |
- |
likely pathogenic (dominant) |
g.31786968_31799611dup |
g.31765420_31778063dup |
g.31765420_31778063dup31780738_31796994del |
- |
PAX6_000878 |
variant affects PAX6 and ELP4 |
PubMed: Hall 2024 |
- |
- |
Germline/De novo (untested) |
- |
- |
- |
- |
- |
Johan den Dunnen |
+?/. |
1 |
6i_13_ |
c.524-2206_*5142{0} |
r.? |
p.? |
- |
likely pathogenic (dominant) |
g.31802286_31818542del |
g.31780738_31796994del |
g.31765420_31778063dup31780738_31796994del |
- |
PAX6_000877 |
variant affects PAX6 and ELP4 |
PubMed: Hall 2024 |
- |
- |
Germline/De novo (untested) |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
1 |
- |
c.? |
r.? |
p.? |
- |
likely pathogenic |
g.? |
- |
NM_001368900.1:c.142-3T>C |
- |
PAX6_000703 |
- |
PubMed: Ehrenberg 2021 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
1 |
- |
c.2T>G |
r.(?) |
p.(Met1?) |
- |
pathogenic |
g.31827958A>C |
g.31806410A>C |
PAX6 c.2T>G, - |
- |
PAX6_000827 |
heterozygous |
PubMed: Bell 2021 |
- |
- |
De novo |
yes |
- |
- |
- |
- |
LOVD |
+/. |
1 |
- |
c.10+1G>C |
r.spl |
p.? |
ACMG |
pathogenic |
g.31827949C>G |
- |
- |
- |
PAX6_000783 |
- |
PubMed: Sharon 2019 |
- |
- |
Germline |
- |
1/2420 IRD families |
- |
- |
- |
Global Variome, with Curator vacancy |
+?/. |
1 |
- |
c.10+5G>C |
r.spl? |
p.? |
ACMG |
VUS |
g.31827945C>G |
- |
- |
- |
PAX6_000801 |
- |
- |
- |
- |
Germline/De novo (untested) |
- |
- |
- |
- |
- |
Jinu Han |
-?/. |
1 |
- |
c.10+8C>T |
r.(=) |
p.(=) |
- |
likely benign |
g.31827942G>A |
g.31806394G>A |
PAX6(NM_000280.3):c.10+8C>T |
- |
PAX6_000727 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Rotterdam |
+?/. |
1 |
- |
c.11-2A>G |
r.spl |
p.(?) |
- |
likely pathogenic |
g.31824384T>C |
g.31802836T>C |
c.11-2A>G, p.(?) |
- |
PAX6_000821 |
- |
PubMed: Wang 2019 |
- |
- |
Germline |
? |
- |
- |
- |
- |
LOVD |
+/. |
1 |
- |
c.(10+1_11-1)_(*1_?)del |
r.? |
p.? |
- |
pathogenic |
g.(?_31811481)_(31824383_31827949)del |
g.(?_31789933)_(31802835_31806401)del |
NM_001604.5:(10+1_11-1)_(1311_?)del |
- |
PAX6_000798 |
- |
PubMed: Haer-Wigman 2017 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
-/. |
1 |
- |
c.13C>G |
r.(?) |
p.(His5Asp) |
- |
benign |
g.31824380G>C |
g.31802832G>C |
- |
- |
PAX6_000900 |
- |
PubMed: Plachy 2019 |
- |
- |
Germline/De novo (untested) |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
1 |
5 |
c.19G>T |
r.(?) |
p.(Gly7*) |
- |
pathogenic |
g.31824374C>A |
g.31802826C>A |
- |
- |
PAX6_000704 |
- |
PubMed: Han 2018 |
- |
- |
De novo |
- |
- |
- |
- |
- |
Joonhong Park |
?/. |
1 |
- |
c.26A>T |
r.(?) |
p.(Asn9Ile) |
- |
VUS |
g.31824367T>A |
- |
PAX6(NM_000280.3):c.26A>T (p.N9I) |
- |
PAX6_000766 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_VUmc |
+/+ |
1 |
5 |
c.33del |
r.(?) |
p.(Gly12Valfs*19) |
- |
pathogenic |
g.31824360del |
g.31802812del |
- |
- |
PAX6_000100 |
- |
- |
- |
- |
Germline |
- |
- |
- |
- |
- |
Andreas Laner |
+/+, +/., +?/. |
6 |
5 |
c.52G>A |
r.(?) |
p.(Gly18Arg) |
- |
likely pathogenic (dominant), pathogenic, pathogenic (dominant) |
g.31824341C>T |
g.31802793C>T |
NM_001310158.1:c.52G>A (Gly18Arg) |
- |
PAX6_000308 |
VKGL data sharing initiative Nederland |
PubMed: Fan 2020, PubMed: Liu 2023, PubMed: Reichsteiner 2021 |
- |
- |
CLASSIFICATION record, De novo, Germline |
yes |
- |
- |
- |
- |
Johan den Dunnen, Andreas Laner, VKGL-NL_Nijmegen |
?/. |
1 |
- |
c.52G>C |
r.(?) |
p.(Gly18Arg) |
- |
VUS |
g.31824341C>G |
g.31802793C>G |
PAX6(NM_001310158.1):c.52G>C (p.G18R) |
- |
PAX6_000769 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Rotterdam |
+?/. |
1 |
- |
c.55C>T |
r.(?) |
p.(Arg19Trp) |
ACMG |
likely pathogenic |
g.31824338G>A |
g.31802790G>A |
- |
- |
PAX6_000770 |
ACMG: PM2,PM5,PP1,PP3; Vincent et al. 2003. Eur J Hum Genet 11: 163 |
- |
- |
- |
Germline |
- |
- |
- |
- |
- |
Andreas Laner |
?/. |
1 |
- |
c.62T>C |
r.(?) |
p.(Leu21Pro) |
- |
VUS |
g.31824331A>G |
- |
PAX6(NM_001310158.1):c.62T>C (p.L21P) |
- |
PAX6_000840 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Rotterdam |
-?/. |
1 |
- |
c.72C>T |
r.(?) |
p.(=) |
- |
likely benign |
g.31824321G>A |
g.31802773G>A |
PAX6(NM_001310158.1):c.72C>T (p.S24=) |
- |
PAX6_000765 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Rotterdam |
+/. |
1 |
- |
c.76C>T |
r.(?) |
p.(Arg26Trp) |
- |
pathogenic (dominant) |
g.31824317G>A |
g.31802769G>A |
- |
- |
PAX6_000864 |
- |
PubMed: Patel 2017 |
- |
- |
Germline/De novo (untested) |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
1 |
- |
c.77G>A |
r.(?) |
p.(Arg26Gln) |
- |
pathogenic (dominant) |
g.31824316C>T |
g.31802768C>T |
- |
- |
PAX6_000854 |
- |
PubMed: Fernandez-Alcalde 2021 |
- |
- |
De novo |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
1 |
- |
c.80del |
r.(?) |
p.(Gln27Argfs*4) |
- |
pathogenic |
g.31824313del |
- |
- |
- |
PAX6_000791 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Nijmegen |
+?/. |
2 |
- |
c.88del |
r.(?) |
p.(Val30*) |
- |
likely pathogenic (dominant) |
g.31824305del |
g.31802757del |
NM_001310158.1:c.88del (Val30*fs*1) |
- |
PAX6_000869 |
- |
PubMed: Liu 2023 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
Johan den Dunnen |
-?/. |
1 |
- |
c.90A>G |
r.(?) |
p.(=) |
- |
likely benign |
g.31824303T>C |
g.31802755T>C |
PAX6(NM_000280.4):c.90A>G (p.(=)) |
- |
PAX6_000764 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Leiden |
+/. |
2 |
- |
c.106del |
r.(?) |
p.(Ala37Profs*17) |
- |
pathogenic (dominant) |
g.31824287del |
g.31802739del |
NM_001310158.1:c.109del (Ala37Profs31) |
- |
PAX6_000868 |
- |
PubMed: Liu 2023 |
rs1057517780 |
rs1057517780 |
Germline |
yes |
- |
- |
- |
- |
Johan den Dunnen |
+?/. |
1 |
- |
c.107G>T |
r.(?) |
p.(Gly36Val) |
- |
likely pathogenic |
g.31824286C>A |
g.31802738C>A |
c.107G>T, p.(Gly36Val) |
- |
PAX6_000820 |
different transcript: NM_001604.5(PAX6):c.107G>T, heterozygous |
PubMed: Wang 2019 |
- |
- |
Germline |
? |
- |
- |
- |
- |
LOVD |
+?/. |
1 |
- |
c.107_114dup |
r.(?) |
p.(Pro39Glyfs*18) |
- |
likely pathogenic |
g.31824280_31824287dup |
g.31802732_31802739dup |
PAX6 c.107_114dup p.(Pro39Glyfs * 18) |
- |
PAX6_000825 |
heterozygous |
PubMed: Méjécase 2020 |
- |
- |
Unknown |
? |
- |
- |
- |
- |
LOVD |
+/. |
2 |
- |
c.110del |
r.(?) |
p.(Arg38Glyfs*16), p.(Arg38GlyfsTer16) |
- |
pathogenic (dominant) |
g.31824283del |
g.31802735del |
NM_001310158.1:c.112del (Arg38Glyfs30), NM_001368894.2:c.112delG |
- |
PAX6_000728 |
- |
PubMed: Jackson 2020, PubMed: Liu 2023 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
Johan den Dunnen |
-?/. |
1 |
- |
c.111C>G |
r.(?) |
p.(=) |
- |
likely benign |
g.31824282G>C |
- |
PAX6(NM_000280.4):c.111C>G (p.(=)) |
- |
PAX6_000898 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Leiden |
+/. |
2 |
5 |
c.112C>G |
r.(?) |
p.(Arg38Gly) |
ACMG |
pathogenic (dominant) |
g.31824281G>C |
g.31802733G>C |
NM_001258462.1:c.112C>G (Arg38Gly) |
- |
PAX6_000786 |
ACMG PS4, PM1, PM2, PP3, PP4, somatic mosaicism in father |
PubMed: Lima Cunha 2020, PubMed: Marinakis 2021 |
- |
rs397514640 |
Germline |
yes |
- |
- |
- |
- |
Johan den Dunnen, Jan Traeger-Synodinos |
+/. |
2 |
6 |
c.112del |
r.(?) |
p.(Arg38Glyfs*16) |
- |
pathogenic |
g.31824283del |
g.31802735del |
112delC, c.112del, p.(Arg38Glyfs*30) |
- |
PAX6_000728 |
different transcript: NM_001604.5(PAX6):c.112del, heterozygous |
PubMed: Wang 2019 |
- |
- |
Germline |
? |
- |
- |
- |
- |
Marta de Castro-Miró |
+/. |
1 |
- |
c.112_116del |
r.(?) |
p.(Arg38Valfs*16) |
- |
pathogenic |
g.31824281_31824285del |
- |
- |
- |
PAX6_000839 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Nijmegen |
+/., +?/. |
2 |
- |
c.113G>A |
r.(?) |
p.(Arg38Gln) |
ACMG |
likely pathogenic (dominant), pathogenic (dominant) |
g.31824280C>T |
g.31802732C>T |
- |
- |
PAX6_000799 |
mother germline mosiac, 1 more item |
PubMed: Li 2018 |
- |
- |
Germline |
? |
- |
- |
- |
- |
Johan den Dunnen, Andreas Laner |
+/., +?/., ?/. |
6 |
- |
c.113G>C |
r.(?) |
p.(Arg38Pro) |
ACMG |
likely pathogenic, pathogenic (dominant), VUS |
g.31824280C>G |
g.31802732C>G |
NM_001310158.1:c.113G>C (Arg38Pro) |
- |
PAX6_000796 |
variant in affected mother from consanguineous family |
PubMed: Fan 2020, PubMed: Liu 2023, PubMed: Rim 2017 |
- |
- |
Germline, Germline/De novo (untested) |
yes |
- |
- |
- |
- |
Johan den Dunnen, Jinu Han |
+?/. |
2 |
- |
c.120C>A |
r.(?) |
p.(Cys40*) |
- |
likely pathogenic |
g.31824273G>T |
g.31802725G>T |
c.120C>A, p.(Cys40*), PAX6(NM_001258462.1):c.120C>A(p.C40*) |
- |
PAX6_000803 |
different transcript: NM_001258462.1(PAX6):c.120C>A, p.(C40*), 1 more item |
PubMed: Sun 2018, PubMed: Wang 2019 |
- |
- |
Germline, Germline/De novo (untested) |
?, yes |
112 |
- |
- |
- |
LOVD |
-/., -?/. |
3 |
- |
c.130C>A |
r.(?) |
p.(=) |
- |
benign, likely benign |
g.31824263G>T |
g.31802715G>T |
1 more item |
- |
PAX6_000763 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Leiden, VKGL-NL_Rotterdam, VKGL-NL_AMC |
+/. |
1 |
- |
c.131G>C |
r.(?) |
p.(Arg44Pro) |
- |
pathogenic (dominant) |
g.31824262C>G |
g.31802714C>G |
- |
- |
PAX6_000860 |
grandmother likely germline mosaic |
PubMed: Riera 2017 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
Johan den Dunnen |
-?/. |
1 |
- |
c.141+11C>G |
r.(=) |
p.(=) |
- |
likely benign |
g.31824241G>C |
- |
PAX6(NM_000280.4):c.141+11C>G (p.(=)) |
- |
PAX6_000838 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Leiden |
-?/. |
1 |
- |
c.141+55G>A |
r.(=) |
p.(=) |
- |
likely benign |
g.31824197C>T |
- |
PAX6(NM_001368918.2):c.196G>A (p.(Ala66Thr)) |
- |
PAX6_000875 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Leiden |
+?/. |
1 |
5i_7i |
c.142-394_402del |
r.? |
p.? |
- |
likely pathogenic (dominant) |
g.31822360_31823718del |
g.31800812_31802170del |
g.31800812_31802170del |
- |
PAX6_000888 |
del ex6-7 |
PubMed: Hall 2024 |
- |
- |
Germline/De novo (untested) |
- |
- |
- |
- |
- |
Johan den Dunnen |
?/. |
1 |
- |
c.142-131T>A |
r.(=) |
p.(=) |
- |
VUS |
g.31823455A>T |
- |
PAX6(NM_001258462.1):c.147T>A (p.(His49Gln)) |
- |
PAX6_000837 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Leiden |
-?/. |
1 |
- |
c.142-99A>G |
r.(=) |
p.(=) |
- |
likely benign |
g.31823423T>C |
- |
PAX6(NM_001310158.1):c.179A>G (p.Q60R) |
- |
PAX6_000780 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Rotterdam |
-?/. |
1 |
- |
c.142-95C>T |
r.(=) |
p.(=) |
- |
likely benign |
g.31823419G>A |
- |
PAX6(NM_001310158.1):c.183C>T (p.N61=) |
- |
PAX6_000836 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Rotterdam |
?/. |
1 |
- |
c.142-94G>A |
r.(=) |
p.(=) |
- |
VUS |
g.31823418C>T |
- |
PAX6(NM_001604.5):c.183+1G>A |
- |
PAX6_000845 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Leiden |
?/. |
1 |
- |
c.142-91A>G |
r.(=) |
p.(=) |
- |
VUS |
g.31823415T>C |
- |
PAX6(NM_001604.5):c.183+4A>G (p.?) |
- |
PAX6_000844 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Leiden |
?/. |
1 |
5i |
c.142-89C>T |
r.(=) |
p.(=) |
- |
VUS |
g.31823413G>A |
g.31801865G>A |
- |
- |
PAX6_000702 |
suspected PAX6-Mutation |
- |
- |
- |
Germline |
- |
- |
- |
- |
- |
Andreas Laner |
-/. |
1 |
- |
c.142-83A>G |
r.(=) |
p.(=) |
- |
benign |
g.31823407T>C |
- |
PAX6(NM_000280.3):c.142-83A>G |
- |
PAX6_000874 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_AMC |
+/. |
1 |
- |
c.151G>A |
r.(?) |
p.(Gly51Arg) |
- |
pathogenic |
g.31823315C>T |
g.31801767C>T |
- |
- |
PAX6_000719 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Nijmegen |
+/. |
2 |
- |
c.154T>C |
r.(?) |
p.(Cys52Arg) |
ACMG |
likely pathogenic, pathogenic |
g.31823312A>G |
g.31801764A>G |
NM_001258462.1:c.196T>C |
- |
PAX6_000703 |
- |
PubMed: Ehrenberg 2021, PubMed: Trujillano 2017 |
- |
- |
De novo, Germline |
- |
- |
- |
- |
- |
Johan den Dunnen, Daniel Trujillano |
+?/. |
1 |
- |
c.157G>A |
r.(?) |
p.(Val53Met) |
- |
likely pathogenic |
g.31823309C>T |
- |
- |
- |
PAX6_000843 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Nijmegen |
+/. |
1 |
6 |
c.163A>T |
r.(?) |
p.(Lys55*) |
- |
pathogenic |
g.31823303T>A |
g.31801755T>A |
- |
- |
PAX6_000701 |
- |
- |
- |
- |
Germline |
- |
- |
- |
- |
- |
Andreas Laner |
+?/. |
1 |
- |
c.177G>T |
r.(?) |
p.(Arg59Ser) |
- |
likely pathogenic (dominant) |
g.31823289C>A |
g.31801741C>A |
NM_001258462.2:c.219G>T |
- |
PAX6_000847 |
- |
PubMed: Fernandez-Alcalde 2021 |
- |
- |
Germline/De novo (untested) |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
1 |
- |
c.183C>G |
r.(?) |
p.(Tyr61*) |
- |
pathogenic |
g.31823283G>C |
- |
- |
- |
PAX6_000873 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Nijmegen |
?/. |
1 |
- |
c.191G>C |
r.(?) |
p.(Gly64Ala) |
- |
VUS |
g.31823275C>G |
- |
- |
- |
PAX6_000813 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Nijmegen |
?/. |
1 |
- |
c.191G>T |
r.(?) |
p.(Gly64Val) |
- |
VUS |
g.31823275C>A |
- |
PAX6(NM_001310158.1):c.233G>T (p.G78V) |
- |
PAX6_000790 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Rotterdam |
+?/. |
1 |
- |
c.197T>A |
r.(?) |
p.(Ile66Asn) |
- |
likely pathogenic (dominant) |
g.31823269A>T |
g.31801721A>T |
NM_001604.5:c.239T>A |
- |
PAX6_000863 |
- |
PubMed: Ma 2016 |
- |
- |
De novo |
- |
- |
- |
- |
- |
Johan den Dunnen |
-?/. |
1 |
- |
c.201A>G |
r.(?) |
p.(=) |
- |
likely benign |
g.31823265T>C |
g.31801717T>C |
PAX6(NM_001604.5):c.243A>G (p.R81=) |
- |
PAX6_000768 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Rotterdam |
+/. |
1 |
- |
c.214G>A |
r.(?) |
p.(Gly72Ser) |
- |
pathogenic |
g.31823252C>T |
g.31801704C>T |
- |
- |
PAX6_000762 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Nijmegen |
+/. |
1 |
6 |
c.214G>C |
r.(?) |
p.(Gly72Arg) |
- |
pathogenic (dominant) |
g.31823252C>G |
g.31801704C>G |
- |
- |
PAX6_000785 |
somatic mosaicism in father |
PubMed: Lima Cunha 2020 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
+?/. |
1 |
- |
c.227C>T |
r.(?) |
p.(Pro76Leu) |
- |
likely pathogenic |
g.31823239G>A |
g.31801691G>A |
- |
- |
PAX6_000797 |
- |
PubMed: Stone 2017 |
- |
- |
De novo |
- |
- |
- |
- |
- |
LOVD |
?/. |
1 |
- |
c.233T>C |
r.(?) |
p.(Val78Ala) |
- |
VUS |
g.31823233A>G |
- |
- |
- |
PAX6_000856 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Nijmegen |
+/. |
1 |
- |
c.233T>G |
r.(?) |
p.(Val78Gly) |
- |
likely pathogenic |
g.31823233A>C |
g.31801685A>C |
NM_1258462.2:c.275T>G |
- |
PAX6_000828 |
- |
PubMed: Ehrenberg 2021 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
1 |
- |
c.233_241del |
r.(?) |
p.(Val78_Pro81delinsAla) |
- |
likely pathogenic |
g.31823225_31823233del |
g.31801677_31801685del |
- |
- |
PAX6_000829 |
- |
PubMed: Ehrenberg 2021 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
?/. |
1 |
- |
c.241C>T |
r.(?) |
p.(Pro81Ser) |
- |
VUS |
g.31823225G>A |
- |
PAX6(NM_000280.6):c.241C>T (p.P81S) |
- |
PAX6_000897 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Groningen |
-?/. |
1 |
- |
c.255C>T |
r.(?) |
p.(=) |
- |
likely benign |
g.31823211G>A |
g.31801663G>A |
PAX6(NM_001310158.1):c.297C>T (p.S99=) |
- |
PAX6_000761 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Rotterdam |
+/., +?/. |
5 |
- |
c.275G>A |
r.(?) |
p.(Arg92Gln) |
- |
likely pathogenic, pathogenic (dominant) |
g.31823191C>T |
g.31801643C>T |
c.317G>A, p.(Arg106Gln), G275A |
- |
PAX6_000729 |
different transcript: NM_001604.3(PAX6):c.317G>A, heterozygous, 1 more item |
PubMed: Wang 2019, PubMed: Xiao 2019 |
- |
- |
Germline |
?, yes |
- |
- |
- |
- |
Johan den Dunnen |
?/. |
1 |
- |
c.284C>T |
r.(?) |
p.(Pro95Leu) |
- |
VUS |
g.31823182G>A |
g.31801634G>A |
PAX6(NM_000280.6):c.284C>T (p.P95L) |
- |
PAX6_000759 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Groningen |
+/. |
1 |
- |
c.300G>A |
r.(?) |
p.(Trp100Ter) |
- |
pathogenic (dominant) |
g.31823166C>T |
g.31801618C>T |
NM_001258463.1:342G>A |
- |
PAX6_000853 |
- |
PubMed: Zhai 2017 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
3 |
- |
c.307C>T |
r.(?) |
p.(Arg103*) |
ACMG |
pathogenic, pathogenic (dominant) |
g.31823159G>A |
g.31801611G>A |
NM_001310158.1:c.349C>T (Arg117*) |
- |
PAX6_000776 |
ACMG grading: PVS1,PM2,PP5; Glaser et al. 1994. NatGenet 7: 463 |
PubMed: Liu 2023 |
rs121907914 |
rs121907914 |
Germline |
yes |
- |
- |
- |
- |
Johan den Dunnen, Andreas Laner |
?/. |
1 |
- |
c.308G>C |
r.(?) |
p.(Arg103Pro) |
- |
VUS |
g.31823158C>G |
- |
PAX6(NM_001604.5):c.350G>C (p.(Arg117Pro)) |
- |
PAX6_000896 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Leiden |
+/. |
1 |
- |
c.325G>T |
r.(?) |
p.(Glu109Ter) |
- |
pathogenic |
g.31823141C>A |
g.31801593C>A |
NM_001604:p.Glu123* |
- |
PAX6_000852 |
- |
PubMed: Li 2016 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
-/., -?/. |
3 |
- |
c.327G>A |
r.(?) |
p.(=) |
- |
benign, likely benign |
g.31823139C>T |
g.31801591C>T |
PAX6(NM_000280.3):c.327G>A (p.E109=), PAX6(NM_000280.4):c.327G>A (p.(Glu109=)) |
- |
PAX6_000726 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Leiden, VKGL-NL_Utrecht, VKGL-NL_AMC |
+?/. |
1 |
- |
c.353C>A |
r.(?) |
p.(Pro118Gln) |
- |
likely pathogenic |
g.31823113G>T |
g.31801565G>T |
PAX6(NM_001310158.1):c.395C>A (p.P132Q) |
- |
PAX6_000725 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Rotterdam |
+/., +?/. |
3 |
- |
c.357C>A |
r.(?) |
p.(Ser119Arg) |
ACMG |
likely pathogenic, pathogenic, VUS |
g.31823109G>T |
g.31801561G>T |
PAX6(NM_000280.4):c.357C>A (p.(Ser119Arg)) |
- |
PAX6_000757 |
VKGL data sharing initiative Nederland |
PubMed: Ehrenberg 2021 |
- |
- |
CLASSIFICATION record, Germline, Germline/De novo (untested) |
- |
- |
- |
- |
- |
Johan den Dunnen, VKGL-NL_Leiden, Jinu Han |
+/. |
2 |
- |
c.357+1G>A |
r.spl, r.spl? |
p.(?), p.? |
- |
pathogenic |
g.31823108C>T |
g.31801560C>T |
c.399+1C>T, p.? |
- |
PAX6_000771 |
2 heterozygous, no homozygous; Clinindb (India), 1 more item |
PubMed: Narang 2020, Journal: Narang 2020, PubMed: Wang 2019 |
- |
rs398123295 |
Germline |
yes |
2/2795 individuals |
- |
- |
- |
Mohammed Faruq |
+/. |
1 |
- |
c.357+1G>C |
r.spl? |
p.? |
- |
pathogenic |
g.31823108C>G |
- |
- |
- |
PAX6_000895 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Nijmegen |
+?/. |
1 |
6i |
c.357+334G>A |
r.spl |
p.? |
- |
likely pathogenic (dominant) |
g.31822775C>T |
g.31801227C>T |
- |
- |
PAX6_000889 |
ACMG PM2, PS4 supp, PP3, PP4 mod |
PubMed: Hall 2024 |
VCV000559621.2 |
- |
Germline/De novo (untested) |
- |
- |
- |
- |
- |
Johan den Dunnen |
-/., -?/. |
3 |
- |
c.358-15_358-5del |
r.spl? |
p.? |
- |
benign, likely benign |
g.31822422_31822432del |
g.31800874_31800884del |
1 more item |
- |
PAX6_000755 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Leiden, VKGL-NL_Rotterdam, VKGL-NL_AMC |
-?/. |
2 |
- |
c.358-7T>C |
r.(=) |
p.(=) |
- |
likely benign |
g.31822411A>G |
g.31800863A>G |
PAX6(NM_000280.4):c.358-7T>C (p.(=)), PAX6(NM_001310160.1):c.-51-7T>C |
- |
PAX6_000754 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Leiden, VKGL-NL_Rotterdam |
+?/. |
1 |
- |
c.358G>C |
r.(?) |
p.(Val120Leu) |
ACMG |
VUS |
g.31822404C>G |
- |
- |
- |
PAX6_000809 |
- |
- |
- |
- |
Germline/De novo (untested) |
- |
- |
- |
- |
- |
Jinu Han |
+?/. |
2 |
- |
c.362C>T |
r.(?) |
p.(Ser121Leu) |
ACMG |
likely pathogenic, VUS |
g.31822400G>A |
g.31800852G>A |
- |
- |
PAX6_000808 |
- |
PubMed: Moon 2021 |
- |
- |
Germline, Germline/De novo (untested) |
- |
- |
- |
- |
- |
Johan den Dunnen, Jinu Han |
+/. |
1 |
- |
c.370_373del |
r.(?) |
p.(Asn124Glufs*22) |
- |
pathogenic |
g.31822390_31822393del |
- |
- |
- |
PAX6_000812 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Nijmegen |
+/. |
1 |
- |
c.371A>G |
r.(?) |
p.(Asn124Ser) |
- |
pathogenic (dominant) |
g.31822391T>C |
g.31800843T>C |
NM_001368894.2:c.413A>G |
- |
PAX6_000851 |
- |
PubMed: Jackson 2020 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/., +?/., ?/. |
4 |
- |
c.383G>A |
r.(?) |
p.(Arg128His) |
ACMG |
likely pathogenic, VUS |
g.31822379C>T |
g.31800831C>T |
- |
- |
PAX6_000718 |
VKGL data sharing initiative Nederland |
PubMed: Ehrenberg 2021, PubMed: Moon 2021 |
- |
- |
CLASSIFICATION record, Germline, Germline/De novo (untested) |
- |
- |
- |
- |
- |
Johan den Dunnen, VKGL-NL_Nijmegen, Jinu Han |