Legend
Please note that a short description of a certain column can be displayed when you move your mouse cursor over the column's header and hold it still. Below, a more detailed description is shown per column.
Effect: The variant's effect on the function of the gene/protein, displayed in the format 'R/C'. R is the value reported by the source (publication, submitter) and this classification may vary between records. C is the value concluded by the curator. Note that in some database the curator uses Summary records to give details on the classification of the variant.Values used: '+' indicating the variant affects function, '+?' probably affects function, '-' does not affect function, '-?' probably does not affect function, '?' effect unknown, '.' effect was not classified.
Exon: number of exon/intron containing variant; 2 = exon 2, 12i = intron 12, 2i_7i = from intron 2 to intron 7, 8i_9 = intron 8/exon 9 boundary, _1 = 5' to exon 1, 18_ = 3' of exon 18, _1_18_ = encompassing the entire 18-exon gene
DNA change (cDNA): description of variant at DNA level, based on a coding DNA reference sequence (following HGVS recommendations); e.g. c.123C>T, c.123_145del, c.123_126dup. For deletions/duplications extending beyond the reference transcript resp. {0}/{2} is used to replace del/dup. Extent of the deletion/duplication should be specified using the genomic description (g.). "-" indicates the variant described on genomic level does not affect the coding DNA reference sequence.
RNA change: description of variant at RNA level (following HGVS recommendations).
- r.123c>u
- r.? = unknown
- r.(?) = RNA not analysed but probably transcribed copy of DNA variant
- r.spl? = RNA not analysed but variant probably affects splicing
- r.(spl?) = RNA not analysed but variant may affect splicing
- r.0? = change expected to abolish transcription
Protein: description of variant at protein level (following HGVS recommendations).
- p.(Arg345Pro) = change predicted from DNA (RNA not analysed)
- p.Arg345Pro = change derived from RNA analysis
- p.? = unknown effect
- p.0? = probably no protein produced
Allele: On which allele is the variant located? Does not necessarily imply inheritance! 'Paternal' (confirmed or inferred), 'Maternal' (confirmed or inferred), 'Parent #1' or #2 for compound heterozygosity without having screened the parents, 'Unknown' for heterozygosity without having screened the parents, 'Both' for homozygozity.
Classification method: The method used for the clinical classification of this variant.
All options:
- ACMG
- ACGS
- EAHAD-CFDB
- ENIGMA
- IARC
- InSiGHT
- kConFab
- other
Clinical classification: Clinical classification of variant, preferably based on standardised criteria (e.g. ACMG), directed on the clinical consequences as published/submitted, indicated using an enriched system including inheritance: e.g. pathogenic, pathogenic (dominant), pathogenic (recessive), pathogenic (!), pathogenic (maternal), pathogenic (paternal). Standard inheritance is covered by dominant/recessive, imprinting by maternal/paternal. A '!' warns for exceptional circumstances to be explained in the 'Remarks' field (low penetrance, variants pathogenic in heterozygous state only, hypomorphic/hypermorphic variants, protective variants, etc.). Non-disease consequences (e.g. drug metabolism (pharmacogenetics), risk factor, blood group, tasting bitter) are indicated using additions to the benign classification; benign (dominant), benign (recessive), benign (!), etc. The value 'association' is used for variants associated with a phenotype and 'NA' for variants from in vitro/in silico records. NOTE: classification may differ from the opinion of the curator as given in a variant SUMMARY-record or the 'Functional effect concluded'). NOTE: pathogenic/likely pathogenic should go together with "variant (probably) affects function" In ClassFunctional.
All options:
- pathogenic
- pathogenic (dominant)
- pathogenic (recessive)
- pathogenic (!)
- pathogenic (maternal)
- pathogenic (paternal)
- likely pathogenic
- likely pathogenic (dominant)
- likely pathogenic (recessive)
- likely pathogenic (!)
- likely pathogenic (maternal)
- likely pathogenic (paternal)
- VUS
- VUS (!)
- likely benign
- likely benign (dominant)
- likely benign (recessive)
- likely benign (!)
- likely benign (maternal)
- likely benign (paternal)
- benign
- benign (dominant)
- benign (recessive)
- benign (!)
- benign (maternal)
- benign (paternal)
- conflicting
- association
- NA
DNA change (genomic) (hg19): HGVS description of variant at DNA level, based on the genomic (chromosomal) DNA reference sequence; e.g. g.12345678C>T, g.12345679del, g.12345678_12345890dup
DNA change (hg38): HGVS description of variant at DNA level, based on the hg38 genomic (chromosomal) eference sequence; e.g. g.12345678C>T, g.12345679del, g.12345678_12345890dup
Published as: listed only when different from "DNA change"; variant as reported originally (e.g. 521delT). Variants seen in animal models, tested in vitro, predicted from RNA analysis, etc. are described between brackets like c.(456C>G)
ISCN: description of the variant according to ISCN nomenclature
DB-ID: database ID of variant, grouping multiple observations of the same variant together, starting with the HGNC gene symbol, followed by an underscore (_) and a six digit number (e.g. DMD_012345). _000000 is used for variants where DNA was not analysed (change predicted from RNA analysis), variants seen in animal models or variants not seen in humans but functionally tested in vitro
Variant remarks: remarks regarding variant described, e.g. germline mosaicism in mother, 345 kb deletion, muscle RNA analysed, not in 200 control chromosomes tested, on founder haplotype, etc.
Reference: publication describing the variant submitted, incl. links to OMIM, PubMed or other source, e.g. "den Dunnen ASHG2003 P2346"
ClinVar ID: ID of variant in ClinVar database
dbSNP ID: the dbSNP ID
Origin: Origin of variant/record: Germline = in all cells, De novo = in all cells, but not in either parent, Germline/De novo (untested) = in all cells, parents not tested (use only when De novo is likely, e.g. isolated/sporadic cases with dominant disease), Somatic = present in a subset of cells, but not in either parent, Uniparental disomy = from parental disomy (maternal or paternal), CLASSIFICATION record = submitter only sharing variant classification (note another report may share Individual data), SUMMARY record = master summary record from curator (may link to another database), In vitro (cloned) = data resulting from in vitro functional assays, animal model = data from animal model, Artefact = false positive variant call, DUPLICATE record = variant already described on another chromosome (e.g. unbalanced translocation, duplicating transposition, 2nd fusion transcript, etc.)
All options:
- Germline
- De novo
- Germline/De novo (untested)
- Somatic
- Uniparental disomy
- Uniparental disomy, maternal allele
- Uniparental disomy, paternal allele
- CLASSIFICATION record
- SUMMARY record
- In vitro (cloned)
- In silico
- animal model
- Artefact
- DUPLICATE record
- Unknown
- Not applicable
Segregation: Indicates whether the variant segregates with the phenotype (yes), does not segregate with the phenotype (no) or segregation is unknown (?)
All options:
- ? = unknown
- yes = segregates with phenotype
- no = does not segregate with phenotype
- - = not applicable
Frequency: frequency in which the variant was found; e.g 5/760 chromosomes (in 5 of 760 chromosomes tested), 1/33 patients (in 1 of 33 patients analysed in study), 0.05 controls (in 5% of control cases tested)
Re-site: restriction enzyme recognition site created (+) or destroyed (-); e.g. BglII+;BamHI-
VIP: variant VIP-status was requested for matchmaking - need collaboration(s) to crack the case - please contact the submitter/curator.
NOTE: to get VIP status ask the curator.
Methylation: result of methylation test; GOM (gain of methylation), LOM (loss of methylation), 30% (30% methylated). NOTE: when several tests were done mention the method as well (e.g. MS-PCR 75%)

 Effect
|

 Exon
|

 DNA change (cDNA)
|

 RNA change
|

 Protein
|

 Classification method
|

 Clinical classification
|

 DNA change (genomic) (hg19)
|

 DNA change (hg38)
|

 Published as
|

 ISCN
|

 DB-ID
|
 Variant remarks
|

 Reference
|

 ClinVar ID
|

 dbSNP ID
|

 Origin
|

 Segregation
|

 Frequency
|

 Re-site
|

 VIP
|

 Methylation
|

 Owner
|
-/. |
1 |
c.6C>T |
r.(?) |
p.(=) |
- |
benign |
g.44589215C>T |
g.43169105C>T |
- |
- |
CRYAA_000010 |
- |
PubMed: Litt 1998 |
- |
rs872331 |
Germline |
- |
- |
- |
- |
- |
Lars Hansen |
-/. |
1 |
c.6C>T |
r.(?) |
p.(=) |
- |
benign |
g.44589215C>T |
g.43169105C>T |
- |
- |
CRYAA_000010 |
- |
PubMed: Beby 2007 |
- |
rs872331 |
Germline |
- |
- |
- |
- |
- |
Lars Hansen |
-/. |
1 |
c.6C>T |
r.(?) |
p.(=) |
- |
benign |
g.44589215C>T |
g.43169105C>T |
D2D |
- |
CRYAA_000010 |
10/11 families tested |
PubMed: Santana 2009 |
- |
rs872331 |
Germline |
- |
10/11/15 |
- |
- |
- |
Johan den Dunnen |
?/. |
1 |
c.6C>T |
r.(?) |
p.(=) |
- |
VUS |
g.44589215C>T |
g.43169105C>T |
6G>A |
- |
CRYAA_000010 |
144 controls |
PubMed: Bhagyalaxmi 2010 |
- |
rs872331 |
Germline |
- |
24/144 |
- |
- |
- |
Johan den Dunnen |
?/. |
1 |
c.6C>T |
r.(?) |
p.(=) |
- |
VUS |
g.44589215C>T |
g.43169105C>T |
6G>A |
- |
CRYAA_000010 |
144 controls |
PubMed: Bhagyalaxmi 2010 |
- |
rs872331 |
Germline |
- |
3/144 |
- |
- |
- |
Johan den Dunnen |
?/. |
1 |
c.6C>T |
r.(?) |
p.(=) |
- |
VUS |
g.44589215C>T |
g.43169105C>T |
6G>A |
- |
CRYAA_000010 |
910 cataract cases |
PubMed: Bhagyalaxmi 2010 |
- |
rs872331 |
Germline |
- |
121/455 |
- |
- |
- |
Johan den Dunnen |
?/. |
1 |
c.6C>T |
r.(?) |
p.(=) |
- |
VUS |
g.44589215C>T |
g.43169105C>T |
6G>A |
- |
CRYAA_000010 |
910 cataract cases |
PubMed: Bhagyalaxmi 2010 |
- |
rs872331 |
Germline |
- |
28/455 |
- |
- |
- |
Johan den Dunnen |
-/. |
1 |
c.6C>T |
r.(?) |
p.(=) |
- |
benign |
g.44589215C>T |
g.43169105C>T |
D2D |
- |
CRYAA_000010 |
- |
PubMed: Santana 2009 |
- |
rs872331 |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
-/. |
1 |
c.6C>T |
r.(?) |
p.(=) |
- |
benign |
g.44589215C>T |
g.43169105C>T |
D2D |
- |
CRYAA_000010 |
- |
PubMed: Santana 2009 |
- |
rs872331 |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
-/. |
1 |
c.6C>T |
r.(?) |
p.(=) |
- |
benign |
g.44589215C>T |
g.43169105C>T |
D2D |
- |
CRYAA_000010 |
variant in family |
PubMed: Santana 2009 |
- |
rs872331 |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
-/. |
1 |
c.6C>T |
r.(?) |
p.(=) |
- |
benign |
g.44589215C>T |
g.43169105C>T |
- |
- |
CRYAA_000010 |
in controls and cataract patients |
PubMed: Pang 2002 |
- |
rs872331 |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
-/. |
1 |
c.6C>T |
r.(?) |
p.(=) |
- |
benign |
g.44589215C>T |
g.43169105C>T |
- |
- |
CRYAA_000010 |
- |
PubMed: Beby 2007 |
- |
rs872331 |
Germline |
- |
- |
- |
- |
- |
Lars Hansen |
-/. |
1 |
c.6C>T |
r.(?) |
p.(=) |
- |
benign |
g.44589215C>T |
g.43169105C>T |
6T>C |
- |
CRYAA_000010 |
- |
PubMed: Graw 2006 |
- |
rs872331 |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
?/. |
1 |
c.6C>T |
r.(?) |
p.(=) |
- |
VUS |
g.44589215C>T |
g.43169105C>T |
6G>A |
- |
CRYAA_000010 |
144 controls |
PubMed: Bhagyalaxmi 2010 |
- |
rs872331 |
Germline |
- |
3/144 |
- |
- |
- |
Johan den Dunnen |
?/. |
1 |
c.6C>T |
r.(?) |
p.(=) |
- |
VUS |
g.44589215C>T |
g.43169105C>T |
6G>A |
- |
CRYAA_000010 |
910 cataract cases |
PubMed: Bhagyalaxmi 2010 |
- |
rs872331 |
Germline |
- |
28/455 |
- |
- |
- |
Johan den Dunnen |
-/. |
1 |
c.6C>T |
r.(?) |
p.(=) |
- |
benign |
g.44589215C>T |
g.43169105C>T |
- |
- |
CRYAA_000010 |
not seggregating with disease; 14 heterozygotes in family |
PubMed: Richter 2008 |
- |
rs872331 |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
-?/. |
- |
c.6C>T |
r.(?) |
p.(Asp2=) |
- |
likely benign |
g.44589215C>T |
g.43169105C>T |
- |
- |
CRYAA_000010 |
- |
PubMed: Li 2019 |
- |
rs872331 |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
1 |
c.27G>A |
r.(?) |
p.(Trp9*) |
- |
pathogenic |
g.44589236G>A |
g.43169126G>A |
- |
- |
CRYAA_000001 |
not in 140 control chromosomes |
PubMed: Pras 2000, OMIM:var0002 |
- |
rs74315440 |
Germline |
- |
- |
HinfI+ |
- |
- |
Johan den Dunnen |
+/. |
1 |
c.27G>A |
r.(?) |
p.(Trp9*) |
- |
pathogenic |
g.44589236G>A |
g.43169126G>A |
- |
- |
CRYAA_000001 |
not in 140 control chromosomes |
PubMed: Pras 2000, OMIM:var0002 |
- |
rs74315440 |
Germline |
- |
- |
HinfI+ |
- |
- |
Johan den Dunnen |
+/. |
1 |
c.34C>T |
r.(?) |
p.(Arg12Cys) |
- |
pathogenic (dominant) |
g.44589243C>T |
g.43169133C>T |
- |
- |
CRYAA_000002 |
not in 340 control chromosomes |
PubMed: Hansen 2007, PubMed: Hansen 2009 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Lars Hansen |
+/. |
1 |
c.34C>T |
r.(?) |
p.(Arg12Cys) |
- |
pathogenic |
g.44589243C>T |
g.43169133C>T |
- |
- |
CRYAA_000002 |
- |
PubMed: Zhang 2009 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
1 |
c.34C>T |
r.(?) |
p.(Arg12Cys) |
- |
pathogenic |
g.44589243C>T |
g.43169133C>T |
104C>T |
- |
CRYAA_000002 |
not in 100 control chromosomes |
PubMed: Devi 2008 |
- |
- |
Germline |
- |
- |
ApaLI+ |
- |
- |
Johan den Dunnen |
+/. |
1 |
c.34C>T |
r.(?) |
p.(Arg12Cys) |
- |
pathogenic |
g.44589243C>T |
g.43169133C>T |
- |
- |
CRYAA_000002 |
not in 100 control chromosomes |
PubMed: Santana 2009 |
- |
- |
Germline |
- |
- |
HhaI- |
- |
- |
Johan den Dunnen |
+/. |
- |
c.34C>T |
r.(?) |
p.(Arg12Cys) |
- |
pathogenic |
g.44589243C>T |
g.43169133C>T |
CRYAA(NM_000394.2):c.34C>T (p.(Arg12Cys)), CRYAA(NM_000394.4):c.34C>T (p.R12C) |
- |
CRYAA_000002 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Groningen |
+/. |
- |
c.34C>T |
r.(?) |
p.(Arg12Cys) |
- |
pathogenic |
g.44589243C>T |
- |
CRYAA(NM_000394.2):c.34C>T (p.(Arg12Cys)), CRYAA(NM_000394.4):c.34C>T (p.R12C) |
- |
CRYAA_000002 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Nijmegen |
+?/. |
- |
c.34C>T |
r.(?) |
p.(Arg12Cys) |
- |
likely pathogenic |
g.44589243C>T |
g.43169133C>T |
c.34C-->T; p.Arg12Cys |
- |
CRYAA_000002 |
no Sanger sequencing; heterozygous |
PubMed: Patel 2019 |
- |
- |
Germline |
? |
- |
- |
- |
- |
LOVD |
+?/. |
- |
c.34C>T |
r.(?) |
p.(Arg12Cys) |
ACMG |
likely pathogenic |
g.44589243C>T |
g.43169133C>T |
CRYAA c.34C>T p.(Arg12Cys) het |
- |
CRYAA_000002 |
heterozygous |
PubMed: Lenassi 2020 |
- |
- |
Germline |
? |
- |
- |
- |
- |
LOVD |
+/. |
- |
c.34C>T |
r.(?) |
p.(Arg12Cys) |
- |
pathogenic (dominant) |
g.44589243C>T |
g.43169133C>T |
- |
- |
CRYAA_000002 |
- |
PubMed: Zhang 2018 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
- |
c.34C>T |
r.(?) |
p.(Arg12Cys) |
- |
pathogenic (dominant) |
g.44589243C>T |
g.43169133C>T |
- |
- |
CRYAA_000002 |
- |
PubMed: Reis 2013 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
- |
c.34C>T |
r.(?) |
p.(Arg12Cys) |
- |
pathogenic |
g.44589243C>T |
- |
CRYAA(NM_000394.2):c.34C>T (p.(Arg12Cys)), CRYAA(NM_000394.4):c.34C>T (p.R12C) |
- |
CRYAA_000002 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Leiden |
+?/. |
1 |
c.35G>T |
r.(?) |
p.(Arg12Leu) |
- |
likely pathogenic |
g.44589244G>T |
g.43169134G>T |
- |
- |
CRYAA_000021 |
- |
- |
- |
- |
Germline |
yes |
- |
- |
- |
- |
Zongfu Cao |
+?/. |
- |
c.35G>T |
r.(?) |
p.(Arg12Leu) |
ACMG |
likely pathogenic (dominant) |
g.44589244G>T |
g.43169134G>T |
- |
- |
CRYAA_000021 |
ACMG PM1, PM2, PP1, PP2, PP3 |
PubMed: Zhuang 2019 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
1 |
c.61C>T |
r.(?) |
p.(Arg21Trp) |
- |
pathogenic |
g.44589270C>T |
g.43169160C>T |
- |
- |
CRYAA_000003 |
not in 340 control chromosomes |
PubMed: Hansen 2009 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Lars Hansen |
+/. |
1 |
c.61C>T |
r.(?) |
p.(Arg21Trp) |
- |
pathogenic |
g.44589270C>T |
g.43169160C>T |
- |
- |
CRYAA_000003 |
not in 340 control chromosomes |
PubMed: Hansen 2007, PubMed: Hansen 2009 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Lars Hansen |
+/. |
1 |
c.61C>T |
r.(?) |
p.(Arg21Trp) |
- |
pathogenic |
g.44589270C>T |
g.43169160C>T |
- |
- |
CRYAA_000003 |
not in 340 control chromosomes |
PubMed: Hansen 2009 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Lars Hansen |
+/. |
1 |
c.61C>T |
r.(?) |
p.(Arg21Trp) |
- |
pathogenic |
g.44589270C>T |
g.43169160C>T |
130C>T |
- |
CRYAA_000003 |
not in 100 control chromosomes |
PubMed: Devi 2008 |
- |
- |
Germline |
- |
- |
MspI- |
- |
- |
Johan den Dunnen |
+/. |
1 |
c.61C>T |
r.(?) |
p.(Arg21Trp) |
- |
pathogenic |
g.44589270C>T |
g.43169160C>T |
- |
- |
CRYAA_000003 |
- |
PubMed: Kondo 2013 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
Jamie Zeegers |
+/. |
1 |
c.61C>T |
r.(?) |
p.(Arg21Trp) |
- |
pathogenic |
g.44589270C>T |
g.43169160C>T |
- |
- |
CRYAA_000003 |
- |
PubMed: Kondo 2013 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
Jamie Zeegers |
+/. |
- |
c.61C>T |
r.(?) |
p.(Arg21Trp) |
- |
pathogenic |
g.44589270C>T |
g.43169160C>T |
CRYAA(NM_000394.4):c.61C>T (p.R21W) |
- |
CRYAA_000003 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Nijmegen |
+?/. |
- |
c.61C>T |
r.(?) |
p.(Arg21Trp) |
ACMG |
likely pathogenic |
g.44589270C>T |
g.43169160C>T |
CRYAA c.61C>T p.(Arg21Trp) het |
- |
CRYAA_000003 |
heterozygous |
PubMed: Lenassi 2020 |
- |
- |
Germline |
? |
- |
- |
- |
- |
LOVD |
+?/. |
- |
c.61C>T |
r.(?) |
p.(Arg21Trp) |
ACMG |
likely pathogenic |
g.44589270C>T |
g.43169160C>T |
CRYAA c.61C>T p.(Arg21Trp) het |
- |
CRYAA_000003 |
heterozygous |
PubMed: Lenassi 2020 |
- |
- |
Germline |
? |
- |
- |
- |
- |
LOVD |
+/. |
- |
c.61C>T |
r.(?) |
p.(Arg21Trp) |
- |
pathogenic |
g.44589270C>T |
- |
CRYAA(NM_000394.4):c.61C>T (p.R21W) |
- |
CRYAA_000003 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_VUmc |
+/. |
- |
c.61C>T |
r.(?) |
p.(Arg21Trp) |
- |
pathogenic (dominant) |
g.44589270C>T |
g.43169160C>T |
- |
- |
CRYAA_000003 |
- |
PubMed: Fernandez-Alcalde 2021 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
- |
c.61C>T |
r.(?) |
p.(Arg21Trp) |
- |
pathogenic (dominant) |
g.44589270C>T |
g.43169160C>T |
- |
- |
CRYAA_000003 |
- |
PubMed: Zhang 2018 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
+?/. |
- |
c.61C>T |
r.(?) |
p.(Arg21Trp) |
ACMG |
likely pathogenic (dominant) |
g.44589270C>T |
g.43169160C>T |
- |
- |
CRYAA_000003 |
ACMG PM1, PM2, PP1, PP2, PP3, PP5 |
PubMed: Zhuang 2019 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
- |
c.61C>T |
r.(?) |
p.(Arg21Trp) |
- |
pathogenic (dominant) |
g.44589270C>T |
g.43169160C>T |
- |
- |
CRYAA_000003 |
- |
PubMed: Sun 2017 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
- |
c.61C>T |
r.(?) |
p.(Arg21Trp) |
- |
pathogenic (dominant) |
g.44589270C>T |
g.43169160C>T |
- |
- |
CRYAA_000003 |
- |
PubMed: Li 2018 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
- |
c.61C>T |
r.(?) |
p.(Arg21Trp) |
- |
pathogenic (dominant) |
g.44589270C>T |
g.43169160C>T |
- |
- |
CRYAA_000003 |
- |
PubMed: Fan 2020 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
- |
c.61C>T |
r.(?) |
p.(Arg21Trp) |
- |
pathogenic (dominant) |
g.44589270C>T |
g.43169160C>T |
- |
- |
CRYAA_000003 |
- |
PubMed: Ma 2016 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
1 |
c.61C>T |
r.(?) |
p.(Arg21Trp) |
- |
pathogenic (dominant) |
g.44589270C>T |
g.43169160C>T |
- |
- |
CRYAA_000003 |
- |
PubMed: Liu 2023 |
rs397515625 |
rs397515625 |
Germline |
yes |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
1 |
c.61C>T |
r.(?) |
p.(Arg21Trp) |
- |
pathogenic (dominant) |
g.44589270C>T |
g.43169160C>T |
- |
- |
CRYAA_000003 |
- |
PubMed: Liu 2023 |
rs397515625 |
rs397515625 |
Germline |
yes |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
1 |
c.62G>A |
r.(?) |
p.(Arg21Gln) |
- |
pathogenic |
g.44589271G>A |
g.43169161G>A |
- |
- |
CRYAA_000014 |
- |
PubMed: Laurie 2013, Journal: Laurie 2013 |
- |
- |
Germline |
yes |
- |
PvuII+ |
- |
- |
Kathryn Burdon |
+/. |
1 |
c.62G>A |
r.(?) |
p.(Arg21Gln) |
- |
pathogenic |
g.44589271G>A |
g.43169161G>A |
- |
- |
CRYAA_000014 |
- |
PubMed: Javadiyan 2016, Journal: Javadiyan 2016 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
Jamie Zeegers |
+/. |
1 |
c.62G>A |
r.(?) |
p.(Arg21Gln) |
- |
pathogenic |
g.44589271G>A |
g.43169161G>A |
- |
- |
CRYAA_000014 |
- |
PubMed: Javadiyan 2016, Journal: Javadiyan 2016 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
Jamie Zeegers |
+/. |
1 |
c.62G>A |
r.(?) |
p.(Arg21Gln) |
- |
pathogenic |
g.44589271G>A |
g.43169161G>A |
- |
- |
CRYAA_000014 |
- |
PubMed: Javadiyan 2016, Journal: Javadiyan 2016 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
Jamie Zeegers |
+/. |
1 |
c.62G>A |
r.(?) |
p.(Arg21Gln) |
- |
pathogenic |
g.44589271G>A |
g.43169161G>A |
- |
- |
CRYAA_000014 |
- |
PubMed: Javadiyan 2016, Journal: Javadiyan 2016 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
Jamie Zeegers |
+/. |
1 |
c.62G>A |
r.(?) |
p.(Arg21Gln) |
- |
pathogenic |
g.44589271G>A |
g.43169161G>A |
- |
- |
CRYAA_000014 |
- |
PubMed: Javadiyan 2016, Journal: Javadiyan 2016 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
Jamie Zeegers |
+/. |
1 |
c.62G>A |
r.(?) |
p.(Arg21Gln) |
- |
pathogenic |
g.44589271G>A |
g.43169161G>A |
- |
- |
CRYAA_000014 |
- |
PubMed: Javadiyan 2016, Journal: Javadiyan 2016 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
Jamie Zeegers |
+/. |
1 |
c.62G>T |
r.(?) |
p.(Arg21Leu) |
- |
pathogenic |
g.44589271G>T |
g.43169161G>T |
62C>T |
- |
CRYAA_000008 |
not in 192 control chromosomes |
PubMed: Graw 2006 |
- |
- |
Germline |
- |
- |
HpaII- |
- |
- |
Johan den Dunnen |
+?/. |
- |
c.62G>T |
r.(?) |
p.(Arg21Leu) |
ACMG |
likely pathogenic |
g.44589271G>T |
g.43169161G>T |
CRYAA c.62G>T p.(Arg21Leu) het |
- |
CRYAA_000008 |
heterozygous |
PubMed: Lenassi 2020 |
- |
- |
Germline |
? |
- |
- |
- |
- |
LOVD |
+?/. |
- |
c.142T>G |
r.(?) |
p.(Tyr48Asp) |
- |
likely pathogenic (dominant) |
g.44589351T>G |
g.43169241T>G |
- |
- |
CRYAA_000034 |
- |
PubMed: Ma 2016 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
1 |
c.145C>T |
r.(?) |
p.(Arg49Cys) |
- |
pathogenic |
g.44589354C>T |
g.43169244C>T |
- |
- |
CRYAA_000004 |
mapped by linkage (LOD 3.3) |
PubMed: Mackay 2003, OMIM:var0003 |
- |
rs74315441 |
Germline |
- |
- |
AciI- |
- |
- |
Johan den Dunnen |
+/. |
1 |
c.145C>T |
r.(?) |
p.(Arg49Cys) |
- |
pathogenic |
g.44589354C>T |
g.43169244C>T |
155C>T |
- |
CRYAA_000004 |
- |
PubMed: Hansen 2009 |
- |
rs74315441 |
Germline |
- |
- |
AciI- |
- |
- |
Lars Hansen |
+/. |
1 |
c.145C>T |
r.(145c>u) |
p.Arg49Cys |
- |
NA |
g.44589354C>T |
g.43169244C>T |
- |
- |
CRYAA_000004 |
expression cloning lens epithelial cells abnormal localization to nucleus, failed to protect from staurosporine-induced apoptotic cell death |
PubMed: Mackay 2003 |
- |
rs74315441 |
In vitro (cloned) |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
- |
c.145C>T |
r.(?) |
p.(Arg49Cys) |
- |
pathogenic |
g.44589354C>T |
g.43169244C>T |
- |
- |
CRYAA_000004 |
1 heterozygous, no homozygous; Clinindb (India) |
PubMed: Narang 2020, Journal: Narang 2020 |
- |
rs74315441 |
Germline |
- |
1/2795 individuals |
- |
- |
- |
Mohammed Faruq |
+/. |
- |
c.145C>T |
r.(?) |
p.(Arg49Cys) |
- |
pathogenic (recessive) |
g.44589354C>T |
g.43169244C>T |
- |
- |
CRYAA_000004 |
- |
PubMed: Patel 2017 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
+?/. |
- |
c.145C>T |
r.(?) |
p.(Arg49Cys) |
- |
likely pathogenic (dominant) |
g.44589354C>T |
g.43169244C>T |
- |
- |
CRYAA_000004 |
- |
PubMed: Berry 2020 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
Johan den Dunnen |
-?/. |
- |
c.154C>T |
r.(?) |
p.(Leu52Phe) |
- |
likely benign |
g.44589363C>T |
- |
CRYAA(NM_000394.3):c.154C>T (p.L52F) |
- |
CRYAA_000023 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Rotterdam |
+/. |
1 |
c.(160C>T) |
r.160c>u |
p.Arg54Cys |
- |
pathogenic |
g.44589369C>T |
g.43169259C>T |
mouse |
- |
CRYAA_000000 |
nm3365 mouse, spontaneous mutant |
PubMed: Xia 2006 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
1 |
c.(160C>T) |
r.160c>u |
p.Arg54Cys |
- |
pathogenic |
g.44589369C>T |
g.43169259C>T |
mouse |
- |
CRYAA_000000 |
nm3365 mouse, spontaneous mutant |
PubMed: Xia 2006 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
1 |
c.160C>T |
r.(?) |
p.(Arg54Cys) |
- |
pathogenic (recessive) |
g.44589369C>T |
g.43169259C>T |
- |
- |
CRYAA_000009 |
- |
PubMed: Khan 2007, PubMed: Khan 2015 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
1 |
c.160C>T |
r.(?) |
p.(Arg54Cys) |
- |
pathogenic (recessive) |
g.44589369C>T |
g.43169259C>T |
- |
- |
CRYAA_000009 |
- |
PubMed: Khan 2007, PubMed: Khan 2015 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
1 |
c.160C>T |
r.(?) |
p.(Arg54Cys) |
- |
pathogenic (recessive) |
g.44589369C>T |
g.43169259C>T |
- |
- |
CRYAA_000009 |
- |
PubMed: Khan 2007, PubMed: Khan 2015 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
1 |
c.160C>T |
r.(?) |
p.(Arg54Cys) |
- |
pathogenic (recessive) |
g.44589369C>T |
g.43169259C>T |
- |
- |
CRYAA_000009 |
- |
PubMed: Khan 2007PubMed: Khan 2007, PubMed: Khan 2015 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
1 |
c.160C>T |
r.(?) |
p.(Arg54Cys) |
- |
pathogenic |
g.44589369C>T |
g.43169259C>T |
230C>T |
- |
CRYAA_000009 |
not in 100 control chromosomes |
PubMed: Devi 2008 |
- |
- |
Germline |
- |
- |
HpyCH4V+ |
- |
- |
Johan den Dunnen |
+/. |
- |
c.160C>T |
r.(?) |
p.(Arg54Cys) |
ACMG |
pathogenic (dominant) |
g.44589369C>T |
g.43169259C>T |
- |
- |
CRYAA_000000 |
- |
PubMed: Javadiyan 2017 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
1 |
c.160C>T |
r.(?) |
p.(Arg54Cys) |
- |
pathogenic (!) |
g.44589369C>T |
g.43169259C>T |
- |
- |
CRYAA_000000 |
incomplete penetrance |
PubMed: Liu 2023 |
rs397515623 |
rs397515623 |
Germline |
yes |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
1 |
c.160C>T |
r.(?) |
p.(Arg54Cys) |
- |
pathogenic (!) |
g.44589369C>T |
g.43169259C>T |
- |
- |
CRYAA_000000 |
incomplete penetrance |
PubMed: Liu 2023 |
rs397515623 |
rs397515623 |
Germline |
yes |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
1 |
c.(161G>A) |
r.(?) |
p.(Arg54His) |
- |
pathogenic |
g.44589370G>A |
g.43169260G>A |
mouse |
- |
CRYAA_000000 |
lop18 mouse |
PubMed: Chang 1999 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
1 |
c.(161G>A) |
r.(?) |
p.(Arg54His) |
- |
pathogenic |
g.44589370G>A |
g.43169260G>A |
mouse |
- |
CRYAA_000000 |
lop18 mouse |
PubMed: Chang 1999 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
?/. |
- |
c.161G>A |
r.(?) |
p.(Arg54His) |
- |
VUS |
g.44589370G>A |
- |
CRYAA(NM_000394.3):c.161G>A (p.R54H) |
- |
CRYAA_000000 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Rotterdam |
+/. |
1 |
c.161G>C |
r.(?) |
p.(Arg54Pro) |
- |
pathogenic |
g.44589370G>C |
g.43169260G>C |
- |
- |
CRYAA_000019 |
- |
PubMed: Dongmei 2012 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
Jamie Zeegers |
+/. |
1 |
c.161G>C |
r.(?) |
p.(Arg54Pro) |
- |
pathogenic |
g.44589370G>C |
g.43169260G>C |
- |
- |
CRYAA_000019 |
- |
PubMed: Dongmei 2012 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
Jamie Zeegers |
+/. |
1 |
c.161G>C |
r.(?) |
p.(Arg54Pro) |
- |
pathogenic |
g.44589370G>C |
g.43169260G>C |
- |
- |
CRYAA_000019 |
- |
PubMed: Dongmei 2012 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
Jamie Zeegers |
+/. |
1 |
c.161G>C |
r.(?) |
p.(Arg54Pro) |
- |
pathogenic |
g.44589370G>C |
g.43169260G>C |
- |
- |
CRYAA_000019 |
- |
PubMed: Dongmei 2012 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
Jamie Zeegers |
+/. |
- |
c.161G>C |
r.(?) |
p.(Arg54Pro) |
- |
pathogenic (recessive) |
g.44589370G>C |
g.43169260G>C |
- |
- |
CRYAA_000019 |
- |
PubMed: Patel 2017 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
Johan den Dunnen |
?/. |
- |
c.188A>T |
r.(?) |
p.(Glu63Val) |
- |
VUS |
g.44589397A>T |
- |
CRYAA(NM_000394.2):c.188A>T (p.(Glu63Val)) |
- |
CRYAA_000039 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Leiden |
-/. |
1i |
c.189+11dup |
r.(=) |
p.(=) |
- |
benign |
g.44589409dup |
g.43169299dup |
IVS1+10insG |
- |
CRYAA_000016 |
- |
PubMed: Santhiya 2006 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
?/. |
1i |
c.189+57G>A |
r.(=) |
p.(=) |
- |
VUS |
g.44589455G>A |
g.43169345G>A |
189+56G>A |
- |
CRYAA_000012 |
- |
PubMed: Pang 2002 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
?/. |
1i |
c.189+171C>T |
r.(=) |
p.(=) |
- |
VUS |
g.44589569C>T |
g.43169459C>T |
189+170C>T |
- |
CRYAA_000011 |
not in controls |
PubMed: Pang 2002 |
- |
rs11700709 |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
-/. |
1i |
c.190-36dup |
r.(=) |
p.(=) |
- |
benign |
g.44590591dup |
g.43170481dup |
IVS1-36insC |
- |
CRYAA_000017 |
- |
PubMed: Santhiya 2006 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
- |
c.193C>T |
r.(?) |
p.(Arg65Ter) |
- |
pathogenic |
g.44590630C>T |
g.43170520C>T |
- |
- |
CRYAA_000022 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Nijmegen |
+/. |
2 |
c.213C>A |
r.(?) |
p.Phe71Leu |
- |
NA |
g.44590650C>A |
g.43170540C>A |
- |
- |
CRYAA_000015 |
tested in vitro in several assays |
PubMed: Bhagyalaxmi 2009 |
- |
- |
In vitro (cloned) |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
2 |
c.213C>A |
r.(?) |
p.(Phe71Leu) |
- |
pathogenic |
g.44590650C>A |
g.43170540C>A |
- |
- |
CRYAA_000015 |
not in 530 control chromosomes; 3/711 ARC patients |
PubMed: Bhagyalaxmi 2009 |
- |
- |
Germline |
- |
3/1422 |
- |
- |
- |
Johan den Dunnen |
+/. |
2 |
c.213C>A |
r.(?) |
p.(Phe71Leu) |
- |
pathogenic |
g.44590650C>A |
g.43170540C>A |
- |
- |
CRYAA_000015 |
- |
PubMed: Bhagyalaxmi 2009 |
- |
- |
Germline |
- |
3/1422 |
- |
- |
- |
Johan den Dunnen |
-?/. |
- |
c.213C>T |
r.(?) |
p.(Phe71=) |
- |
likely benign |
g.44590650C>T |
- |
CRYAA(NM_000394.3):c.213C>T (p.F71=) |
- |
CRYAA_000024 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Rotterdam |
?/. |
- |
c.235C>A |
r.(?) |
p.(His79Asn) |
- |
VUS |
g.44590672C>A |
- |
CRYAA(NM_000394.3):c.235C>A (p.H79N) |
- |
CRYAA_000025 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Rotterdam |
+?/. |
- |
c.253C>T |
r.(?) |
p.(Leu85Phe) |
ACMG |
likely pathogenic (dominant) |
g.44590690C>T |
g.43170580C>T |
- |
- |
CRYAA_000036 |
ACMG PM1, PM2, PP1, PP3 |
PubMed: Khidiyatova 2023 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
+?/. |
- |
c.275A>G |
r.(?) |
p.(Asp92Gly) |
- |
likely pathogenic |
g.44590712A>G |
g.43170602A>G |
c.275A-->G; p.Asp92Gly |
- |
CRYAA_000027 |
no Sanger sequencing; heterozygous |
PubMed: Patel 2019 |
- |
- |
Germline |
? |
- |
- |
- |
- |
LOVD |
+?/. |
- |
c.291C>G |
r.(?) |
p.(His97Gln) |
ACMG |
likely pathogenic (dominant) |
g.44590728C>G |
g.43170618C>G |
- |
- |
CRYAA_000037 |
ACMG PM1, PM2, PP2 ,PP3 |
PubMed: Khidiyatova 2023 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |