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
|
+?/. |
- |
146C>T |
r.(?) |
p.(Thr49Met) |
- |
likely pathogenic |
g.68191267C>T |
g.67724550C>T |
RDH12 146C->T, T49M |
- |
RDH12_000001 |
heterozygous |
PubMed: Janecke 2004 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
LOVD |
+?/. |
- |
184C>T |
r.(?) |
p.(Arg62*) |
- |
likely pathogenic |
g.68191305C>T |
g.67724588C>T |
RDH12 184C->T, R62X |
- |
RDH12_000002 |
heterozygous |
PubMed: Janecke 2004 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
LOVD |
+?/. |
- |
565C>T |
r.(?) |
p.(Gln189*) |
- |
likely pathogenic |
g.68193814C>T |
g.67727097C>T |
RDH12 565C->T, Q189X |
- |
RDH12_000003 |
homozygous |
PubMed: Janecke 2004 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
LOVD |
+?/. |
- |
565C>T |
r.(?) |
p.(Gln189*) |
- |
likely pathogenic |
g.68193814C>T |
g.67727097C>T |
RDH12 565C->T, Q189X |
- |
RDH12_000003 |
homozygous |
PubMed: Janecke 2004 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
LOVD |
+?/. |
- |
677A>G |
r.(?) |
p.(Tyr226Cys) |
- |
likely pathogenic |
g.68195926A>G |
g.67729209A>G |
RDH12 677A->G, Y226C |
- |
RDH12_000004 |
homozygous |
PubMed: Janecke 2004 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
LOVD |
+?/. |
- |
677A>G |
r.(?) |
p.(Tyr226Cys) |
- |
likely pathogenic |
g.68195926A>G |
g.67729209A>G |
RDH12 677A->G, Y226C |
- |
RDH12_000004 |
homozygous |
PubMed: Janecke 2004 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
LOVD |
+?/. |
- |
677A>G |
r.(?) |
p.(Tyr226Cys) |
- |
likely pathogenic |
g.68195926A>G |
g.67729209A>G |
RDH12 677A->G, Y226C |
- |
RDH12_000004 |
homozygous |
PubMed: Janecke 2004 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
LOVD |
+?/. |
- |
677A>G |
r.(?) |
p.(Tyr226Cys) |
- |
likely pathogenic |
g.68195926A>G |
g.67729209A>G |
RDH12 677A->G, Y226C |
- |
RDH12_000004 |
homozygous |
PubMed: Janecke 2004 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
LOVD |
+?/. |
- |
677A>G |
r.(?) |
p.(Tyr226Cys) |
- |
likely pathogenic |
g.68195926A>G |
g.67729209A>G |
RDH12 677A->G, Y226C |
- |
RDH12_000004 |
homozygous |
PubMed: Janecke 2004 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
LOVD |
+?/. |
- |
677A>G |
r.(?) |
p.(Tyr226Cys) |
- |
likely pathogenic |
g.68195926A>G |
g.67729209A>G |
RDH12 677A->G, Y226C |
- |
RDH12_000004 |
homozygous |
PubMed: Janecke 2004 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
LOVD |
+?/. |
- |
677A>G |
r.(?) |
p.(Tyr226Cys) |
- |
likely pathogenic |
g.68195926A>G |
g.67729209A>G |
RDH12 677A->G, Y226C |
- |
RDH12_000004 |
homozygous |
PubMed: Janecke 2004 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
LOVD |
+?/. |
- |
677A>G |
r.(?) |
p.(Tyr226Cys) |
- |
likely pathogenic |
g.68195926A>G |
g.67729209A>G |
RDH12 677A->G, Y226C |
- |
RDH12_000004 |
homozygous |
PubMed: Janecke 2004 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
LOVD |
+?/. |
- |
677A>G |
r.(?) |
p.(Tyr226Cys) |
- |
likely pathogenic |
g.68195926A>G |
g.67729209A>G |
RDH12 677A->G, Y226C |
- |
RDH12_000004 |
homozygous |
PubMed: Janecke 2004 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
LOVD |
+?/. |
- |
677A>G |
r.(?) |
p.(Tyr226Cys) |
- |
likely pathogenic |
g.68195926A>G |
g.67729209A>G |
RDH12 677A->G, Y226C |
- |
RDH12_000004 |
homozygous |
PubMed: Janecke 2004 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
LOVD |
+?/. |
- |
677A>G |
r.(?) |
p.(Tyr226Cys) |
- |
likely pathogenic |
g.68195926A>G |
g.67729209A>G |
RDH12 677A->G, Y226C |
- |
RDH12_000004 |
homozygous |
PubMed: Janecke 2004 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
LOVD |
+?/. |
- |
677A>G |
r.(?) |
p.(Tyr226Cys) |
- |
likely pathogenic |
g.68195926A>G |
g.67729209A>G |
RDH12 677A->G, Y226C |
- |
RDH12_000004 |
homozygous |
PubMed: Janecke 2004 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
LOVD |
+?/. |
- |
677A>G |
r.(?) |
p.(Tyr226Cys) |
- |
likely pathogenic |
g.68195926A>G |
g.67729209A>G |
RDH12 677A->G, Y226C |
- |
RDH12_000004 |
homozygous |
PubMed: Janecke 2004 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
LOVD |
+?/. |
- |
677A>G |
r.(?) |
p.(Tyr226Cys) |
- |
likely pathogenic |
g.68195926A>G |
g.67729209A>G |
RDH12 677A->G, Y226C |
- |
RDH12_000004 |
homozygous |
PubMed: Janecke 2004 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
LOVD |
+?/. |
- |
677A>G |
r.(?) |
p.(Tyr226Cys) |
- |
likely pathogenic |
g.68195926A>G |
g.67729209A>G |
RDH12 677A->G, Y226C |
- |
RDH12_000004 |
homozygous |
PubMed: Janecke 2004 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
LOVD |
+?/. |
- |
806delCCCTG |
r.(?) |
p.(Ala269Glyfs*2) |
- |
likely pathogenic |
g.68196055_68196059del |
g.67729338_67729342del |
RDH12 806delCCCTG, Y226C |
- |
RDH12_000005 |
homozygous |
PubMed: Janecke 2004 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
LOVD |
+?/+? |
- |
c.? |
r.? |
p.? |
- |
likely pathogenic (recessive) |
g.? |
- |
Y194X |
- |
SERPINA1_000009 |
- |
PubMed: Jacobson 2007 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Julia Lopez |
+?/+? |
- |
c.? |
r.? |
p.? |
- |
likely pathogenic (recessive) |
g.? |
- |
Y194X |
- |
SERPINA1_000009 |
- |
PubMed: Jacobson 2007 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Julia Lopez |
+?/+? |
- |
c.? |
r.? |
p.? |
- |
likely pathogenic (recessive) |
g.? |
- |
R295X |
- |
SERPINA1_000009 |
- |
PubMed: Jacobson 2007 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Julia Lopez |
+/. |
5 |
c.? |
r.(?) |
p.? |
- |
pathogenic |
g.68191924C>A |
- |
c.296C>A |
- |
SERPINA1_000009 |
- |
PubMed: Beryozkin-2014 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
+?/. |
- |
c.? |
r.0? |
p.0? |
- |
likely pathogenic |
g.68176483_68197327del |
- |
chr14:g.68176483_68197327del |
- |
SERPINA1_000009 |
heterozygous |
PubMed: Turro 2020 |
- |
- |
Germline/De novo (untested) |
? |
- |
- |
- |
- |
LOVD |
+?/. |
1 |
c.2T>C |
r.(?) |
p.(Met1?) |
- |
likely pathogenic |
g.68189361T>C |
g.67722644T>C |
RDH12 c.2T>C, p.M1? |
- |
RDH12_000139 |
heterozygous |
PubMed: Thompson 2005 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
LOVD |
+?/. |
- |
c.2T>C |
r.0? |
p.(Met1?) |
- |
likely pathogenic (dominant) |
g.68189361T>C |
g.67722644T>C |
RDH12 M1? |
- |
RDH12_000139 |
heterozygous |
PubMed: Valverde 2009 |
- |
- |
Unknown |
? |
- |
- |
- |
- |
LOVD |
+?/. |
- |
c.38C>A |
r.(?) |
p.(Ser13*) |
- |
likely pathogenic |
g.68189397C>A |
g.67722680C>A |
RDH12 c.38C>A, Ser13X |
- |
RDH12_000159 |
homozygous |
PubMed: Fahim 2019 |
- |
- |
Unknown |
? |
- |
- |
- |
- |
LOVD |
?/. |
- |
c.59C>T |
r.(?) |
p.(Pro20Leu) |
- |
VUS |
g.68189418C>T |
g.67722701C>T |
- |
- |
RDH12_000026 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Nijmegen |
+/. |
3 |
c.63_66del |
r.(?) |
p.(Ile22Glyfs*19) |
- |
pathogenic |
g.68189422_68189425del |
g.67722705_67722708del |
- |
- |
RDH12_000011 |
- |
- |
- |
- |
Unknown |
- |
- |
- |
- |
- |
Feng Wang |
+/. |
3 |
c.63_66del |
r.(?) |
p.(Ile22Glyfs*19) |
- |
pathogenic |
g.68189422_68189425del |
- |
c.63_66del |
- |
RDH12_000011 |
- |
PubMed: Wang-2014 |
- |
- |
Unknown |
- |
- |
- |
- |
- |
LOVD |
+?/. |
- |
c.63_66del |
r.(?) |
p.(Ile22Glyfs*19) |
- |
likely pathogenic |
g.68189422_68189425del |
g.67722705_67722708del |
RDH12 c.57_60del, p.P20del |
- |
RDH12_000011 |
error in annotation, 3' nucleotide and first affected amino acid rule shift it to c.63_66del, p.I22GfsX19; heterozygous |
PubMed: Mackay 2011 |
- |
- |
Unknown |
? |
- |
- |
- |
- |
LOVD |
+?/. |
- |
c.63_66del |
r.(?) |
p.(Ile22Glyfs*19) |
- |
likely pathogenic |
g.68189422_68189425del |
g.67722705_67722708del |
RDH12 lIe22Gly |
- |
RDH12_000011 |
no nucleotide written, extrapolated from protein, databases and publications; error in annotation, in databases lIe22Gly is described as ""this sequence change creates a premature translational stop signal (p.Ile22Glyfs*19)"", and points to c.63_66del; heterozygous |
PubMed: Aleman 2018 |
- |
- |
Unknown |
? |
- |
- |
- |
- |
LOVD |
+?/. |
- |
c.63_66del |
r.(?) |
p.(Ile22Glyfs*19) |
- |
likely pathogenic |
g.68189422_68189425del |
g.67722705_67722708del |
RDH12 c.57_60delTCCA, Ala19fs |
- |
RDH12_000011 |
error in annotation, most 3' rule shifts it to c.63_66del, Ile22Glyfs*19; single heterozygous variant |
PubMed: Fahim 2019 |
- |
- |
Unknown |
? |
- |
- |
- |
- |
LOVD |
+?/. |
- |
c.68+1G>A |
r.spl |
p.? |
- |
likely pathogenic |
g.68189428G>A |
g.67722711G>A |
c.68 + 1G > A NA |
- |
RDH12_000080 |
- |
PubMed: Wang 2016 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
+/. |
- |
c.68+1G>A |
r.spl |
p.(?) |
ACMG |
pathogenic |
g.68189428G>A |
g.67722711G>A |
RDH12 NM_152443: g.20826G>A, c.68+1G>A |
- |
RDH12_000080 |
- |
PubMed: Xu 2020 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
LOVD |
-/. |
- |
c.69-19_69-18del |
r.(=) |
p.(=) |
- |
benign |
g.68191171_68191172del |
g.67724454_67724455del |
RDH12(NM_152443.3):c.69-19_69-18delTT |
- |
RDH12_000016 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_AMC |
?/. |
- |
c.85G>A |
r.(?) |
p.(Gly29Arg) |
- |
VUS |
g.68191206G>A |
g.67724489G>A |
RDH12(NM_152443.3):c.85G>A (p.G29R) |
- |
RDH12_000049 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_AMC |
?/. |
- |
c.91T>C |
r.(?) |
p.(Cys31Arg) |
- |
VUS |
g.68191212T>C |
g.67724495T>C |
RDH12(NM_152443.2):c.91T>C (p.C31R) |
- |
RDH12_000050 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Rotterdam |
+?/. |
2 |
c.99_102dupAAAT |
r.(?) |
p.(Val35Lysfs*28) |
- |
likely pathogenic |
g.68191220_68191223dup |
g.67724503_67724506dup |
RDH12 c.99_102dupAAAT, p.Val35LysfsX27 |
- |
RDH12_000140 |
reduced ability to convert all-trans retinal to all-trans retinol in the presence of NADPH , heterozygous |
PubMed: Thompson 2005 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
LOVD |
+?/. |
- |
c.99_102dupAAAT |
r.(?) |
p.(Val35Lysfs*28) |
- |
likely pathogenic (dominant) |
g.68191220_68191223dup |
g.67724503_67724506dup |
RDH12 c.99_102dupAAAT |
- |
RDH12_000140 |
heterozygous |
PubMed: Valverde 2009 |
- |
- |
Unknown |
? |
- |
- |
- |
- |
LOVD |
+?/. |
- |
c.121G>T |
r.(?) |
p.(Val41Leu) |
- |
likely pathogenic |
g.68191242G>T |
g.67724525G>T |
- |
- |
RDH12_000072 |
- |
PubMed: Huang 2017 |
- |
- |
Unknown |
- |
- |
- |
- |
- |
LOVD |
?/. |
- |
c.125T>C |
r.(?) |
p.(Val42Ala) |
ACMG |
VUS |
g.68191246T>C |
g.67724529T>C |
RDH12 c.125T>C; p.VaI42AIa |
- |
RDH12_000132 |
heterozygous |
PubMed: Sallum 2020 |
- |
- |
Unknown |
? |
- |
- |
- |
- |
LOVD |
?/. |
- |
c.125T>C |
r.(?) |
p.(Val42Ala) |
ACMG |
VUS |
g.68191246T>C |
g.67724529T>C |
RDH12 c.12ST>C; p.VaI42AIa |
- |
RDH12_000132 |
heterozygous |
PubMed: Sallum 2020 |
- |
- |
Unknown |
? |
- |
- |
- |
- |
LOVD |
+/. |
- |
c.133A>G |
r.(?) |
p.(Thr45Ala) |
- |
pathogenic |
g.68191254A>G |
g.67724537A>G |
- |
- |
RDH12_000078 |
- |
PubMed: Zolnikova 2017 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
+/. |
4 |
c.133A>G |
r.(?) |
p.(Thr45Ala) |
- |
pathogenic |
g.68191254A>G |
- |
c.133A>G |
- |
RDH12_000078 |
- |
PubMed: Eisenberger-2013 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
+?/. |
- |
c.133A>G |
r.(?) |
p.(Thr45Ala) |
- |
likely pathogenic |
g.68191254A>G |
g.67724537A>G |
RDH12 Thr45Ala |
- |
RDH12_000078 |
no nucleotide written, extrapolated from protein and databases; heterozygous |
PubMed: Aleman 2018 |
- |
- |
Unknown |
? |
- |
- |
- |
- |
LOVD |
+?/. |
- |
c.133dup |
r.(?) |
p.(Thr45Asnfs*17) |
- |
likely pathogenic |
g.68191254dup |
g.67724537dup |
133_134insA |
- |
RDH12_000073 |
- |
PubMed: Stone 2017 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
+?/. |
2 |
c.138C>T |
r.spl? |
p.(Gly46=) |
- |
likely pathogenic |
g.68191259C>T |
g.67724542C>T |
RDH12 G46G |
- |
RDH12_000141 |
single heterozygous variant in a recessive disease; no second allele |
PubMed: Sun 2007 |
- |
- |
Unknown |
? |
- |
- |
- |
- |
LOVD |
+?/. |
- |
c.139G>A |
r.(?) |
p.(Ala47Thr) |
- |
likely pathogenic (recessive) |
g.68191260G>A |
g.67724543G>A |
- |
- |
RDH12_000065 |
- |
PubMed: Taylor 2017 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
+?/. |
- |
c.139G>A |
r.(?) |
p.(Ala47Thr) |
- |
likely pathogenic |
g.68191260G>A |
g.67724543G>A |
- |
- |
RDH12_000065 |
- |
PubMed: Patel 2016 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
+?/. |
- |
c.139G>A |
r.(?) |
p.(Ala47Thr) |
- |
likely pathogenic |
g.68191260G>A |
g.67724543G>A |
- |
- |
RDH12_000065 |
- |
PubMed: Patel 2016 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
+?/. |
- |
c.139G>A |
r.(?) |
p.(Ala47Thr) |
- |
likely pathogenic |
g.68191260G>A |
g.67724543G>A |
- |
- |
RDH12_000065 |
- |
PubMed: Patel 2016 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
+?/+? |
4 |
c.139G>A |
r.(?) |
p.(Ala47Thr) |
- |
likely pathogenic (recessive) |
g.68191260G>A |
- |
A47T |
- |
RDH12_000065 |
- |
PubMed: Jacobson 2007 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Julia Lopez |
?/. |
4 |
c.139G>A |
r.(?) |
p.(Ala47Thr) |
- |
VUS |
g.68191260G>A |
- |
c.139G>A |
- |
RDH12_000065 |
- |
PubMed: Abu-Safieh-2013 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
?/. |
4 |
c.139G>A |
r.(?) |
p.(Ala47Thr) |
- |
VUS |
g.68191260G>A |
- |
c.139G>A |
- |
RDH12_000065 |
- |
PubMed: Abu-Safieh-2013 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
+/. |
- |
c.139G>A |
r.(?) |
p.(Ala47Thr) |
ACMG |
pathogenic |
g.68191260G>A |
g.67724543G>A |
RDH12 NM_152443: g.22658G>A, c.139G>A, p.A47T |
- |
RDH12_000065 |
- |
PubMed: Xu 2020 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
LOVD |
+?/. |
4 |
c.139G>A |
r.(?) |
p.(Ala47Thr) |
- |
likely pathogenic |
g.68191260G>A |
g.67724543G>A |
RDH12:NM_152443:exon4:c.139G>A:p.A47T |
- |
RDH12_000065 |
homozygous |
PubMed: Chen 2020 |
- |
- |
Germline |
? |
- |
- |
- |
- |
LOVD |
+?/. |
- |
c.139G>A |
r.(?) |
p.(Ala47Thr) |
- |
likely pathogenic |
g.68191260G>A |
g.67724543G>A |
RDH12 c.139G>A p.(Ala47Thr) |
- |
RDH12_000065 |
homozygous |
PubMed: Méjécase 2020 |
- |
- |
Unknown |
? |
- |
- |
- |
- |
LOVD |
+?/. |
4 |
c.139G>A |
r.(?) |
p.(Ala47Thr) |
- |
likely pathogenic (recessive) |
g.68191260G>A |
- |
c.139G>A |
- |
RDH12_000065 |
- |
PubMed: Liu-2020 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
+?/. |
2 |
c.139G>A |
r.(?) |
p.(Ala47Thr) |
- |
likely pathogenic |
g.68191260G>A |
g.67724543G>A |
RDH12 c.139G>A, p.A47T |
- |
RDH12_000065 |
heterozygous |
PubMed: Thompson 2005 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
LOVD |
+?/. |
- |
c.139G>A |
r.(?) |
p.(Ala47Thr) |
- |
likely pathogenic |
g.68191260G>A |
g.67724543G>A |
RDH12 c.139G>A; p.Ala47Thr |
- |
RDH12_000065 |
homozygous |
PubMed: AlBakri 2015 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
LOVD |
+?/. |
- |
c.139G>A |
r.(?) |
p.(Ala47Thr) |
- |
likely pathogenic |
g.68191260G>A |
g.67724543G>A |
RDH12 c.139G>A; p.Ala47Thr |
- |
RDH12_000065 |
homozygous |
PubMed: AlBakri 2015 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
LOVD |
+?/. |
- |
c.139G>A |
r.(?) |
p.(Ala47Thr) |
- |
likely pathogenic |
g.68191260G>A |
g.67724543G>A |
RDH12 c.139G>A, Ala47Thr |
- |
RDH12_000065 |
heterozygous |
PubMed: Fahim 2019 |
- |
- |
Unknown |
? |
- |
- |
- |
- |
LOVD |
+/. |
- |
c.146C>A |
r.(?) |
p.(Thr49Lys) |
ACMG |
pathogenic (recessive) |
g.68191267C>A |
g.67724550C>A |
c.C146A |
- |
RDH12_000079 |
- |
PubMed: Zhang 2016 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
+?/. |
- |
c.146C>A |
r.(?) |
p.(Thr49Lys) |
- |
likely pathogenic |
g.68191267C>A |
g.67724550C>A |
RDH12 c.146C>A, p.T49K |
- |
RDH12_000079 |
homozygous |
PubMed: Mackay 2011 |
- |
- |
Unknown |
? |
- |
- |
- |
- |
LOVD |
+/. |
2 |
c.146C>T |
r.(?) |
p.(Thr49Met) |
- |
pathogenic (recessive) |
g.68191267C>T |
g.67724550C>T |
- |
- |
RDH12_000003 |
- |
PubMed: Yücelyilmaz 2014 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
Didem Yücel Yılmaz |
+?/. |
2 |
c.146C>T |
r.(?) |
p.(Thr49Met) |
- |
likely pathogenic |
g.68191267C>T |
g.67724550C>T |
- |
- |
RDH12_000003 |
- |
Sharon, submitted |
- |
- |
Germline |
- |
- |
- |
- |
- |
Dror Sharon |
+/. |
- |
c.146C>T |
r.(?) |
p.(Thr49Met) |
- |
pathogenic |
g.68191267C>T |
g.67724550C>T |
- |
- |
RDH12_000003 |
- |
- |
- |
- |
Germline |
- |
- |
- |
- |
- |
Marta de Castro-Miró |
+/. |
- |
c.146C>T |
r.(?) |
p.(Thr49Met) |
- |
pathogenic |
g.68191267C>T |
g.67724550C>T |
- |
- |
RDH12_000003 |
1 heterozygous, no homozygous; Clinindb (India) |
PubMed: Narang 2020, Journal: Narang 2020 |
- |
rs28940314 |
Germline |
- |
1/2795 individuals |
- |
- |
- |
Mohammed Faruq |
+?/. |
- |
c.146C>T |
r.(?) |
p.(Thr49Met) |
ACMG |
likely pathogenic |
g.68191267C>T |
- |
- |
- |
RDH12_000003 |
- |
PubMed: Sharon 2019 |
- |
- |
Germline |
- |
1/2420 IRD families |
- |
- |
- |
Global Variome, with Curator vacancy |
+/. |
- |
c.146C>T |
r.(?) |
p.(Thr49Met) |
- |
pathogenic |
g.68191267C>T |
g.67724550C>T |
T49M |
- |
RDH12_000003 |
- |
PubMed: Sanchez-Alcudia 2014 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
+/. |
4 |
c.146C>T |
r.(?) |
p.(Thr49Met) |
ACMG |
pathogenic |
g.68191267C>T |
g.67724550C>T |
NM_152443.2:c.146C>T, NP_689656.2:p.(Thr49Met), NC_000014.8:g.68191267C>T |
- |
RDH12_000003 |
- |
PubMed: Wang 2018 |
- |
- |
Germline |
? |
- |
- |
- |
- |
LOVD |
+/. |
4 |
c.146C>T |
r.(?) |
p.(Thr49Met) |
- |
pathogenic |
g.68191267C>T |
- |
c.146C>T |
- |
RDH12_000003 |
- |
PubMed: Wang-2013 |
- |
- |
Unknown |
- |
- |
- |
- |
- |
LOVD |
+/. |
4 |
c.146C>T |
r.(?) |
p.(Thr49Met) |
- |
pathogenic |
g.68191267C>T |
- |
c.146C>T |
- |
RDH12_000003 |
- |
PubMed: Wang-2013 |
- |
- |
Unknown |
- |
- |
- |
- |
- |
LOVD |
+/. |
4 |
c.146C>T |
r.(?) |
p.(Thr49Met) |
- |
pathogenic |
g.68191267C>T |
- |
c.146C>T |
- |
RDH12_000003 |
- |
PubMed: Beryozkin-2014 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
+?/. |
- |
c.146C>T |
r.(?) |
p.(Thr49Met) |
- |
likely pathogenic |
g.68191267C>T |
g.67724550C>T |
RDH12, variant 1: c.146C>T/p.T49M, variant 2: c.146C>T/p.T49M |
- |
RDH12_000003 |
solved, homozygous |
PubMed: Weisschuh 2020 |
- |
- |
Unknown |
? |
- |
- |
- |
- |
LOVD |
+?/. |
- |
c.146C>T |
r.(?) |
p.(Thr49Met) |
- |
likely pathogenic |
g.68191267C>T |
g.67724550C>T |
RDH12, variant 1: c.146C>T/p.T49M, variant 2: c.451C>G/p.H151D |
- |
RDH12_000003 |
solved, compound heterozygous |
PubMed: Weisschuh 2020 |
- |
- |
Unknown |
? |
- |
- |
- |
- |
LOVD |
?/. |
- |
c.146C>T |
r.(?) |
p.(Thr49Met) |
ACMG |
VUS |
g.68191267C>T |
g.67724550C>T |
RDH12 c.146C>T; p.Thr49Met |
- |
RDH12_000003 |
heterozygous |
PubMed: Sallum 2020 |
- |
- |
Unknown |
? |
- |
- |
- |
- |
LOVD |
+?/. |
4 |
c.146C>T |
r.(?) |
p.(Thr49Met) |
- |
likely pathogenic (recessive) |
g.68191267C>T |
- |
c.146C>T |
- |
RDH12_000003 |
- |
PubMed: Liu-2020 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
+?/. |
4 |
c.146C>T |
r.(?) |
p.(Thr49Met) |
- |
likely pathogenic (recessive) |
g.68191267C>T |
- |
c.146C>T |
- |
RDH12_000003 |
- |
PubMed: Liu-2020 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
+?/. |
- |
c.146C>T |
r.(?) |
p.(Thr49Met) |
ACMG |
likely pathogenic |
g.68191267C>T |
g.67724550C>T |
RDH12 c.146C>T(;)806C>G, V1: c.146C>T, (p.Thr49Met) |
- |
RDH12_000003 |
alleles in cis or trans; heterozygous |
PubMed: Chen 2021 |
- |
- |
Unknown |
? |
- |
- |
- |
- |
LOVD |
+?/. |
2 |
c.146C>T |
r.(?) |
p.(Thr49Met) |
- |
likely pathogenic |
g.68191267C>T |
g.67724550C>T |
RDH12 T49M |
- |
RDH12_000003 |
dramatic reduction in the ability to produce all-trans-retinol from all-trans-retinal (~95% less); heterozygous |
PubMed: Sun 2007 |
- |
- |
Unknown |
? |
- |
- |
- |
- |
LOVD |
+?/. |
- |
c.146C>T |
r.(?) |
p.(Thr49Met) |
- |
likely pathogenic (dominant) |
g.68191267C>T |
g.67724550C>T |
RDH12 T49M |
- |
RDH12_000003 |
homozygous |
PubMed: Valverde 2009 |
- |
- |
Unknown |
? |
- |
- |
- |
- |
LOVD |
+?/. |
- |
c.146C>T |
r.(?) |
p.(Thr49Met) |
- |
likely pathogenic |
g.68191267C>T |
g.67724550C>T |
RDH12 T49M |
- |
RDH12_000003 |
mutation significantly raises the apparent Km values of RDH12 for nucleotide cofactors; statistically significant improvement in the conversion of retinaldehyde to retinol after treatment with MG132 was observed in the cells containing T49M (but not I51N) |
PubMed: Lee 2011 |
- |
- |
In vitro (cloned) |
? |
- |
- |
- |
- |
LOVD |
+?/. |
- |
c.146C>T |
r.(?) |
p.(Thr49Met) |
- |
likely pathogenic |
g.68191267C>T |
g.67724550C>T |
RDH12 c.146C>T, p.T49M |
- |
RDH12_000003 |
homozygous |
PubMed: Mackay 2011 |
- |
- |
Unknown |
? |
- |
- |
- |
- |
LOVD |
+?/. |
- |
c.146C>T |
r.(?) |
p.(Thr49Met) |
- |
likely pathogenic |
g.68191267C>T |
g.67724550C>T |
RDH12 Thr49Met |
- |
RDH12_000003 |
no nucleotide written, extrapolated from protein and databases; heterozygous |
PubMed: Aleman 2018 |
- |
- |
Unknown |
? |
- |
- |
- |
- |
LOVD |
+?/. |
- |
c.146C>T |
r.(?) |
p.(Thr49Met) |
- |
likely pathogenic |
g.68191267C>T |
g.67724550C>T |
RDH12 c.146 C>T, Thr49Met |
- |
RDH12_000003 |
heterozygous |
PubMed: Fahim 2019 |
- |
- |
Unknown |
? |
- |
- |
- |
- |
LOVD |
+?/. |
- |
c.146C>T |
r.(?) |
p.(Thr49Met) |
- |
likely pathogenic |
g.68191267C>T |
g.67724550C>T |
RDH12 c.146 C>T, Thr49Met |
- |
RDH12_000003 |
single heterozygous variant |
PubMed: Fahim 2019 |
- |
- |
Unknown |
? |
- |
- |
- |
- |
LOVD |
+?/. |
- |
c.146C>T |
r.(?) |
p.(Thr49Met) |
ACMG |
pathogenic |
g.68191267C>T |
g.67724550C>T |
RDH12 c.146C>T, p.Thr49Met |
- |
RDH12_000003 |
homozygous |
PubMed: Scott 2020 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
LOVD |
+?/. |
- |
c.146C>T |
r.(?) |
p.(Thr49Met) |
ACMG |
pathogenic |
g.68191267C>T |
g.67724550C>T |
RDH12 c.146C>T, p.Thr49Met |
- |
RDH12_000003 |
homozygous |
PubMed: Scott 2020 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
LOVD |
+?/. |
- |
c.146C>T |
r.(?) |
p.(Thr49Met) |
- |
likely pathogenic |
g.68191267C>T |
g.67724550C>T |
RDH12 c.146C>T(;)806C>G; p.(Thr49Met) |
- |
RDH12_000003 |
heterozygous |
PubMed: Chen 2021 |
- |
- |
Germline/De novo (untested) |
? |
Taiwan Biobank: 0.000330; GnomAD_exome_East: 0.0000544; GnomAD_All: 0.0000159 |
- |
- |
- |
LOVD |
+/. |
4 |
c.146C>T |
r.(?) |
p.(Thr49Met) |
- |
pathogenic |
g.68191267C>T |
- |
c.146C>T |
- |
RDH12_000003 |
- |
PubMed: Panneman 2023 |
- |
- |
Unknown |
- |
- |
- |
- |
- |
Daan Panneman |
+/. |
- |
c.148G>A |
r.(?) |
p.(Gly50Ser) |
- |
pathogenic |
g.68191269G>A |
g.67724552G>A |
- |
- |
RDH12_000039 |
- |
- |
- |
- |
Germline |
- |
- |
- |
- |
- |
Marta de Castro-Miró |
+/. |
4 |
c.148G>A |
r.(?) |
p.(Gly50Ser) |
ACMG |
pathogenic |
g.68191269G>A |
g.67724552G>A |
- |
- |
RDH12_000039 |
- |
PubMed: Hitti-Malin 2024, Journal: Hitti-Malin 2024 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Rebekkah Hitti-Malin |
+?/. |
4 |
c.151A>T |
r.(?) |
p.(Ile51Phe) |
ACMG |
VUS (!) |
g.68191272A>T |
- |
- |
- |
RDH12_000062 |
ACMG: PM2, PM5, PP3 class 3 |
- |
- |
- |
Germline |
? |
- |
- |
- |
- |
Andreas Laner |
+?/. |
2 |
c.152T>A |
r.(?) |
p.(Ile51Asn) |
- |
likely pathogenic |
g.68191273T>A |
g.67724556T>A |
RDH12 c.152T>A, p.Ile51Asn |
- |
RDH12_000142 |
heterozygous |
PubMed: Perrault 2004 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
LOVD |
+?/. |
- |
c.152T>A |
r.(?) |
p.(Ile51Asn) |
- |
likely pathogenic |
g.68191273T>A |
g.67724556T>A |
RDH12 I51N |
- |
RDH12_000142 |
mutation significantly raises the apparent Km values of RDH12 for nucleotide cofactors |
PubMed: Lee 2011 |
- |
- |
In vitro (cloned) |
? |
- |
- |
- |
- |
LOVD |
+?/. |
- |
c.152T>C |
r.(?) |
p.(Ile51Thr) |
- |
likely pathogenic |
g.68191273T>C |
g.67724556T>C |
- |
- |
RDH12_000027 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Nijmegen |
+/. |
- |
c.152T>C |
r.(?) |
p.(Ile51Thr) |
- |
pathogenic |
g.68191273T>C |
g.67724556T>C |
- |
- |
RDH12_000027 |
- |
PubMed: Haer-Wigman 2017 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |