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

 Effect
|

 Exon
|

 DNA change (cDNA)
|

 RNA change
|

 Protein
|

 Classification method
|

 Clinical classification
|

 DNA change (genomic) (hg19)
|

 DNA change (hg38)
|

 Published as
|

 ISCN
|

 DB-ID
|
 Variant remarks
|

 Reference
|

 ClinVar ID
|

 dbSNP ID
|

 Origin
|

 Segregation
|

 Frequency
|

 Re-site
|

 VIP
|

 Methylation
|

 Owner
|
./. |
- |
c.-9273523_*2935700dup |
- |
- |
- |
pathogenic |
g.123150811_135380935dup |
- |
- |
- |
ACADSB_000015 |
increased gene dosage |
PubMed: DDDS 2015, Journal: DDDS 2015 |
- |
- |
De novo |
- |
- |
- |
- |
- |
Johan den Dunnen |
-/. |
- |
c.-94T>G |
r.(?) |
p.(=) |
- |
benign |
g.126107506A>C |
g.124418937A>C |
- |
- |
OAT_000081 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Nijmegen |
-?/. |
- |
c.-53C>T |
r.(?) |
p.(=) |
- |
likely benign |
g.126107465G>A |
g.124418896G>A |
- |
- |
OAT_000080 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Nijmegen |
?/. |
- |
c.-53C>T |
r.(=) |
p.(=) |
- |
VUS |
g.126107465G>A |
g.124418896G>A |
- |
- |
OAT_000080 |
91 heterozygous; Clinindb (India) |
PubMed: Narang 2020, Journal: Narang 2020 |
- |
rs117824913 |
Germline |
- |
91/2793 individuals |
- |
- |
- |
Mohammed Faruq |
?/. |
- |
c.-53C>T |
r.(=) |
p.(=) |
- |
VUS |
g.126107465G>A |
g.124418896G>A |
- |
- |
OAT_000080 |
3 homozygous; Clinindb (India) |
PubMed: Narang 2020, Journal: Narang 2020 |
- |
rs117824913 |
Germline |
- |
3/2793 individuals |
- |
- |
- |
Mohammed Faruq |
-?/. |
- |
c.-30+8C>T |
r.(=) |
p.(=) |
- |
likely benign |
g.126107434G>A |
g.124418865G>A |
OAT(NM_000274.3):c.-30+8C>T (p.(=)) |
- |
OAT_000082 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Leiden |
+?/. |
- |
c.? |
r.0 |
p.0 |
- |
likely pathogenic |
g.? |
g.? |
OAT partial deletion EcoRI frgment 5.7 kb to ~5kb |
- |
CYP2C9_001038 |
heterozygous; second allele unknown |
PubMed: Hotta 1989 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
LOVD |
+?/. |
- |
c.? |
r.0 |
p.0 |
- |
likely pathogenic |
g.? |
g.? |
OAT partial deletion EcoRI frgment ~5.4 Kbp; complete absence of mRNA |
- |
CYP2C9_001038 |
heterozygous |
PubMed: Inana 1988 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
LOVD |
+?/. |
- |
c.? |
r.0 |
p.0 |
- |
likely pathogenic |
g.? |
g.? |
OAT promoter ? |
- |
CYP2C9_001038 |
Northern 50% of expression, so probably a heterozygous mutation in the promoter or partial deletion |
PubMed: Mashima 1999 |
- |
- |
Germline |
? |
- |
- |
- |
- |
LOVD |
+/+? |
2 |
c.3G>A |
r.(3g>a) |
p.(Met1?) |
- |
pathogenic |
g.126100738C>T |
g.124412169C>T |
3G>A: MI1 |
- |
OAT_000039 |
1 Lebanese Maronite patient and 1 patient of unknown ethnicity. Mutation leads to production of inactive OAT protein. |
PubMed: Brody 1992, PubMed: Park 1992 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
Anne Polvi |
+/. |
- |
c.3G>A |
r.(?) |
p.(Met1?) |
- |
pathogenic |
g.126100738C>T |
g.124412169C>T |
- |
- |
OAT_000039 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Nijmegen |
+/. |
- |
c.3G>A |
r.(?) |
p.(Met1?) |
- |
pathogenic |
g.126100738C>T |
g.124412169C>T |
OAT G->A transition, changing the initiator ATG (methionine) codon to ATA |
- |
OAT_000039 |
homozygous |
PubMed: Mitchell 1988 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
LOVD |
+/. |
- |
c.3G>A |
r.(?) |
p.(Met1?) |
- |
pathogenic |
g.126100738C>T |
g.124412169C>T |
OAT G->A transition, changing the initiator ATG (methionine) codon to ATA |
- |
OAT_000039 |
homozygous |
PubMed: Mitchell 1988 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
LOVD |
+/. |
- |
c.3G>A |
r.(?) |
p.(Met1?) |
- |
pathogenic |
g.126100738C>T |
g.124412169C>T |
OAT G->A transition, changing the initiator ATG (methionine) codon to ATA |
- |
OAT_000039 |
homozygous |
PubMed: Mitchell 1988 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
LOVD |
+/. |
- |
c.3G>A |
r.(?) |
p.(Met1?) |
- |
pathogenic |
g.126100738C>T |
g.124412169C>T |
OAT G->A transition, changing the initiator ATG (methionine) codon to ATA |
- |
OAT_000039 |
homozygous |
PubMed: Mitchell 1988 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
LOVD |
-?/. |
- |
c.48C>T |
r.(?) |
p.(Arg16=) |
- |
likely benign |
g.126100693G>A |
g.124412124G>A |
OAT(NM_000274.4):c.48C>T (p.R16=) |
- |
OAT_000085 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_AMC |
+/+? |
2 |
c.152G>A |
r.(152g>a) |
p.(Gly51Asp) |
- |
pathogenic (recessive) |
g.126100589C>T |
g.124412020C>T |
- |
- |
OAT_000003 |
- |
PubMed: Doimo 2012 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
Anne Polvi |
+/+? |
2 |
c.152G>A |
r.(152g>a) |
p.(Gly51Asp) |
- |
pathogenic (recessive) |
g.126100589C>T |
g.124412020C>T |
- |
- |
OAT_000003 |
- |
PubMed: Doimo 2012 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
Anne Polvi |
+/+? |
2 |
c.152G>A |
r.(152g>a) |
p.(Gly51Asp) |
- |
pathogenic |
g.126100589C>T |
g.124412020C>T |
- |
- |
OAT_000003 |
- |
PubMed: Sergouniotis 2012 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
Anne Polvi |
+?/. |
- |
c.152G>A |
r.(?) |
p.(Gly51Asp) |
- |
likely pathogenic |
g.126100589C>T |
g.124412020C>T |
- |
- |
OAT_000003 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Nijmegen |
+/+? |
2 |
c.159del |
r.(?) |
p.(His53Glnfs*8) |
- |
pathogenic |
g.126100582del |
g.124412013del |
159delC: H53fs(-l) |
- |
OAT_000029 |
Iraqi Jew mutation |
PubMed: Brody 1992 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
Anne Polvi |
+/. |
- |
c.159del |
r.(?) |
p.(His53Glnfs*8) |
ACMG |
pathogenic |
g.126100582del |
- |
c.159delC |
- |
OAT_000029 |
- |
PubMed: Sharon 2019 |
- |
- |
Germline |
- |
2/2420 IRD families |
- |
- |
- |
Global Variome, with Curator vacancy |
+?/. |
- |
c.159delC |
r.(?) |
p.(His53Glnfs*8) |
- |
likely pathogenic |
g.126100582del |
g.124412013del |
OAT c.159delC, p.(H53Qfs7*) |
- |
OAT_000029 |
homozygous |
PubMed: Heller 2017 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
LOVD |
+/+? |
2 |
c.162C>A |
r.162c>a |
p.Asn54Lys |
- |
pathogenic (recessive) |
g.126100579G>T |
g.124412010G>T |
C>A at 162 bp, Asp54Lys |
- |
OAT_000030 |
Mutation leads to inactive OAT protein. |
PubMed: Ramesh 1988 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
Anne Polvi |
+/+? |
2 |
c.163T>C |
r.(163u>c) |
p.(Tyr55His) |
- |
pathogenic |
g.126100578A>G |
g.124412009A>G |
163T>C |
- |
OAT_000031 |
Australian/Hungarian/English mutation. Mutation leads to very low RNA expression level and production of inactive OAT protein. |
PubMed: Ramesh 1988, PubMed: Brody 1992 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
Anne Polvi |
+/+? |
2 |
c.163T>C |
r.(163u>c) |
p.(Tyr55His) |
- |
pathogenic |
g.126100578A>G |
g.124412009A>G |
- |
- |
OAT_000031 |
- |
PubMed: Sergouniotis 2012 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
Anne Polvi |
+/. |
- |
c.192_193del |
r.(?) |
p.(Gly65Lysfs*15) |
- |
pathogenic (recessive) |
g.126100553_126100554del |
g.124411984_124411985del |
192_193delAG |
- |
OAT_000032 |
- |
PubMed: Mashima 1992 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
-?/. |
- |
c.199+11_199+16dup |
r.(=) |
p.(=) |
- |
likely benign |
g.126100527_126100532dup |
- |
OAT(NM_001322966.2):c.199+11_199+16dupAATTAA |
- |
OAT_000099 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_AMC |
?/. |
- |
c.199+11_199+16dupAATTAA |
r.spl? |
p.? |
- |
VUS |
g.126100527_126100532dup |
g.124411958_124411963dup |
OAT c.199+11_199+16dupAATTAA |
- |
OAT_000099 |
heterozygous; segregation not performed |
PubMed: Kim 2013 |
- |
- |
Germline |
? |
- |
- |
- |
- |
LOVD |
?/. |
- |
c.199+11_199+16dupAATTAA |
r.spl? |
p.? |
- |
VUS |
g.126100527_126100532dup |
g.124411958_124411963dup |
OAT c.199+11_199+16dupAATTAA |
- |
OAT_000099 |
heterozygous; segregation not performed |
PubMed: Kim 2013 |
- |
- |
Germline |
? |
- |
- |
- |
- |
LOVD |
-/. |
- |
c.199+19C>T |
r.(=) |
p.(=) |
- |
benign |
g.126100523G>A |
g.124411954G>A |
OAT(NM_000274.4):c.199+19C>T |
- |
OAT_000079 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Groningen |
-/. |
- |
c.199+19C>T |
r.(=) |
p.(=) |
- |
benign |
g.126100523G>A |
g.124411954G>A |
OAT(NM_000274.4):c.199+19C>T |
- |
OAT_000079 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Nijmegen |
-/. |
- |
c.199+20G>A |
r.(=) |
p.(=) |
- |
benign |
g.126100522C>T |
g.124411953C>T |
OAT(NM_000274.4):c.199+20G>A |
- |
OAT_000078 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_AMC |
+/+? |
2i |
c.199+303C>G |
r.199_200ins199+161_199+302 |
p.(=) |
- |
pathogenic |
g.126100239G>C |
g.124411670G>C |
OAT G67ins; C>G transition in intron 3: mRNA with 142-bp insertion (new Alu exon"") between exon 3 and 4"" |
- |
OAT_000033 |
Mutation creates a new donor splice site that activates an upstream cryptic acceptor site. New 142 bp exon"" is created and included into transcript."" |
PubMed: Mitchell 1991 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
Anne Polvi |
-/. |
- |
c.200-10T>C |
r.(=) |
p.(=) |
- |
benign |
g.126097544A>G |
g.124408975A>G |
OAT(NM_001322966.1):c.200-10T>C, OAT(NM_001322966.2):c.200-10T>C |
- |
OAT_000077 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_AMC |
-/. |
- |
c.200-10T>C |
r.(=) |
p.(=) |
- |
benign |
g.126097544A>G |
g.124408975A>G |
OAT(NM_001322966.1):c.200-10T>C, OAT(NM_001322966.2):c.200-10T>C |
- |
OAT_000077 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Rotterdam |
+?/. |
3 |
c.248G>A |
r.(?) |
p.(Ser83Asn) |
- |
likely pathogenic |
g.126097486C>T |
g.124408917C>T |
OAT c.G248A:p.S83N |
- |
OAT_000098 |
homozygous |
PubMed: Huang 2018 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
LOVD |
+?/. |
3 |
c.248G>A |
r.(?) |
p.(Ser83Asn) |
- |
likely pathogenic |
g.126097486C>T |
g.124408917C>T |
OAT c.G248A:p.S83N |
- |
OAT_000098 |
homozygous |
PubMed: Huang 2018 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
LOVD |
+/+? |
3 |
c.267C>A |
r.(267c>a) |
p.(Asn89Lys) |
- |
pathogenic |
g.126097467G>T |
g.124408898G>T |
267C>A |
- |
OAT_000034 |
Finnish mutation |
PubMed: Brody 1992 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
Anne Polvi |
+/. |
- |
c.267C>A |
r.(?) |
p.(Asn89Lys) |
- |
pathogenic |
g.126097467G>T |
g.124408898G>T |
OAT N89K |
- |
OAT_000034 |
no nucleotide annotation, extrapolated from protein and databases; heterozygous |
PubMed: Peltola 2001 |
- |
- |
Unknown |
? |
- |
- |
- |
- |
LOVD |
+/+? |
3 |
c.268C>G |
r.(268c>G) |
p.(Gln90Glu) |
- |
pathogenic |
g.126097466G>C |
g.124408897G>C |
268C>G: Q90E |
- |
OAT_000035 |
Loss of mitocondrial targeting of mutant protein. |
PubMed: Kobayashi et al.1995 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
Anne Polvi |
+/+? |
3 |
c.272G>A |
r.(272g>a) |
p.(Gly91Glu) |
- |
pathogenic |
g.126097462C>T |
g.124408893C>T |
- |
- |
OAT_000068 |
- |
PubMed: Sergouniotis 2012 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
Anne Polvi |
?/. |
- |
c.272G>A |
r.(?) |
p.(Gly91Glu) |
- |
VUS |
g.126097462C>T |
- |
OAT(NM_001322966.1):c.272G>A (p.G91E) |
- |
OAT_000068 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Rotterdam |
+?/. |
- |
c.272G>A |
r.(?) |
p.(Gly91Glu) |
- |
likely pathogenic |
g.126097462C>T |
g.124408893C>T |
- |
- |
OAT_000068 |
- |
PubMed: Stone 2017 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
+/+? |
3 |
c.278G>T |
r.(278g>u) |
p.(Cys93Phe) |
- |
pathogenic |
g.126097456C>A |
g.124408887C>A |
278G>T |
- |
OAT_000036 |
German/Italian mutation |
PubMed: Brody 1992 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
Anne Polvi |
?/. |
- |
c.298G>A |
r.(?) |
p.(Ala100Thr) |
- |
VUS |
g.126097436C>T |
g.124408867C>T |
OAT(NM_001322966.1):c.298G>A (p.A100T) |
- |
OAT_000084 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Rotterdam |
+/. |
3 |
c.311A>G |
r.311a>g |
p.Gln104Arg |
- |
pathogenic |
g.126097423T>C |
g.124408854T>C |
- |
- |
OAT_000004 |
- |
- |
- |
- |
Germline |
- |
- |
- |
- |
- |
Eva Trevisson |
+/+? |
3 |
c.311A>G |
r.(311a>g) |
p.(Gln104Arg) |
- |
pathogenic |
g.126097423T>C |
g.124408854T>C |
- |
- |
OAT_000004 |
- |
PubMed: Doimo 2012 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
Anne Polvi |
-?/. |
- |
c.314T>C |
r.(?) |
p.(Val105Ala) |
- |
likely benign |
g.126097420A>G |
- |
OAT(NM_001322966.1):c.314T>C (p.V105A) |
- |
OAT_000088 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Rotterdam |
?/. |
3 |
c.314T>C |
r.(?) |
p.(Val105Ala) |
- |
VUS |
g.126097420A>G |
g.124408851A>G |
T314C |
- |
OAT_000088 |
- |
PubMed: Katagiri 2014 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
+/+? |
3 |
c.362G>A |
r.(362g>a) |
p.(Gly121Asp) |
- |
pathogenic |
g.126097372C>T |
g.124408803C>T |
- |
- |
OAT_000070 |
- |
PubMed: Sergouniotis 2012 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
Anne Polvi |
+/+? |
3 |
c.373_375del |
r.(?) |
p.(Glu125del) |
- |
pathogenic |
g.126097362_126097364del |
g.124408793_124408795del |
Codon 125 GAG Glu deletion |
- |
OAT_000037 |
- |
PubMed: Mashima 1996 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
Anne Polvi |
+/+? |
3 |
c.381dup |
r.381dupu) |
p.(Thr128Tyrfs*2) |
- |
pathogenic |
g.126097354dup |
g.124408785dup |
380-1insT: 1127fs(+l) |
- |
OAT_000038 |
Welsh mutation. Reduced RNA expression. |
PubMed: Brody 1992 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
Anne Polvi |
+?/. |
- |
c.416T>G |
r.(?) |
p.(Met139Arg) |
ACMG |
likely pathogenic |
g.126097318A>C |
g.124408749A>C |
OAT c.416T>G, p.(Met139Arg) |
- |
OAT_000090 |
homozygous |
PubMed: Dineiro 2020 |
- |
- |
Germline |
? |
- |
- |
- |
- |
LOVD |
+/+? |
3i_4 |
c.425-4_429del |
r.425_520del |
p.Gly142_Ala173del |
- |
pathogenic |
g.126097204_126097212del |
g.124408635_124408643del |
a 9-bp deletion covering the 3' splice acceptor region of intron 4: exon 5 skipping; McClatchey-1 |
- |
OAT_000041 |
- |
PubMed: McClatchey 1990 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
Anne Polvi |
+?/. |
- |
c.425-4_429del |
r.spl |
p.? |
- |
likely pathogenic |
g.126097204_126097212del |
g.124408635_124408643del |
IVS3-6del9cTGATAGGAG |
- |
OAT_000041 |
- |
PubMed: Stone 2017 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
+/+? |
3i |
c.425-2A>G |
r.425_520del |
p.Gly142_Ala173del |
- |
pathogenic (recessive) |
g.126097208T>C |
g.124408639T>C |
Exon 5 skipping |
- |
OAT_000040 |
exon 4 skipping (published exon 5) |
PubMed: Mashima 1992 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
Anne Polvi |
+/. |
- |
c.425-2A>G |
r.spl |
p.? |
- |
pathogenic |
g.126097208T>C |
g.124408639T>C |
OAT IVS4 nt-2 a->g |
- |
OAT_000040 |
exon 4 (described as exon 5) not spliced in; possible duplicate patient from {PMID:Mashima 1992:1487247}; heterozygous |
PubMed: Mashima 1999 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
LOVD |
+/. |
- |
c.425-2A>G |
r.spl |
p.? |
- |
pathogenic |
g.126097208T>C |
g.124408639T>C |
OAT IVS4 nt-2 a->g |
- |
OAT_000040 |
exon 4 (described as exon 5) not spliced in; possible duplicate patient from {PMID:Mashima 1992:1487247}; heterozygous |
PubMed: Mashima 1999 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
LOVD |
+/+? |
4 |
c.425G>A |
r.(425g>a) |
p.(Gly142Glu) |
- |
pathogenic |
g.126097206C>T |
g.124408637C>T |
Codon 142 GGA>GAA: Gly>Glu |
- |
OAT_000042 |
- |
PubMed: Mashima 1996 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
Anne Polvi |
+/. |
- |
c.425G>A |
r.(?) |
p.(Gly142Glu) |
- |
pathogenic |
g.126097206C>T |
g.124408637C>T |
OAT c.425G>A |
- |
OAT_000042 |
heterozygous; segregation not performed |
PubMed: Kim 2013 |
- |
- |
Germline |
? |
- |
- |
- |
- |
LOVD |
+/. |
- |
c.425G>A |
r.(?) |
p.(Gly142Glu) |
- |
pathogenic |
g.126097206C>T |
g.124408637C>T |
OAT c.425G>A |
- |
OAT_000042 |
heterozygous; segregation not performed |
PubMed: Kim 2013 |
- |
- |
Germline |
? |
- |
- |
- |
- |
LOVD |
?/. |
- |
c.460C>T |
r.(?) |
p.(Arg154Cys) |
- |
VUS |
g.126097171G>A |
g.124408602G>A |
- |
- |
OAT_000076 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Nijmegen |
+/+? |
4 |
c.461G>T |
r.(461g>u) |
p.(Arg154Leu) |
- |
pathogenic |
g.126097170C>A |
g.124408601C>A |
461G>T |
- |
OAT_000043 |
English/German mutation. Mutation leads to production of inactive OAT protein. |
PubMed: Brody 1992 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
Anne Polvi |
+/+? |
4 |
c.472_486del |
r.472_486del |
p.Tyr158_Gly162del |
- |
pathogenic |
g.126097149_126097163del |
g.124408580_124408594del |
15-bp deletion: del YTVKG |
- |
OAT_000044 |
- |
PubMed: Park 1992 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
Anne Polvi |
+?/. |
10 |
c.473A>C |
r.(?) |
p.(Tyr158Ser) |
- |
likely pathogenic |
g.126097158T>G |
g.124408589T>G |
OAT c.473A>C: p.Y158S |
- |
OAT_000097 |
homozygous |
PubMed: Cui 2018 |
- |
- |
Germline |
? |
- |
- |
- |
- |
LOVD |
+?/. |
5 |
c.505_506del |
r.(?) |
p.(Lys169Aspfs*11) |
- |
likely pathogenic |
g.126097126_126097127del |
g.124408557_124408558del |
OAT c.504_505delAA (exon 5) (p.K169DfsX10) |
- |
OAT_000096 |
heterozygous |
PubMed: Katagiri 2014 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
LOVD |
+?/. |
5 |
c.505_506del |
r.(?) |
p.(Lys169Aspfs*11) |
- |
likely pathogenic |
g.126097126_126097127del |
g.124408557_124408558del |
OAT c.504_505delAA (exon 5) (p.K169DfsX10) |
- |
OAT_000096 |
heterozygous |
PubMed: Katagiri 2014 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
LOVD |
+/+? |
4i_5i |
c.521-172_649-744del |
r.521_648del |
p.Gly175Cysfs*18 |
- |
pathogenic (recessive) |
g.126093239_126094310del |
g.124404670_124405741del |
1,072-bp deletion between 172 bp upstream and 772 bp downstream of exon 6: exon 6 skipping. |
- |
OAT_000046 |
very low RNA expression |
PubMed: Akaki 1992 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
Anne Polvi |
-/. |
- |
c.521-15_521-12del |
r.(=) |
p.(=) |
- |
benign |
g.126094149_126094152del |
g.124405580_124405583del |
OAT(NM_000274.4):c.521-15_521-12delGTTT |
- |
OAT_000075 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_AMC |
+/+? |
5 |
c.533G>A |
r.533g>a |
p.(Trp178*) |
- |
pathogenic |
g.126094120C>T |
g.124405551C>T |
Exon 6 bp +13G>A: W178X and exon 6 skipping |
- |
OAT_000048 |
Exon 8 skipping in less than 5% of transcripts. |
PubMed: Dietz 1993 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
Anne Polvi |
+/+? |
5 |
c.533_537del |
r.(?) |
p.(Trp178*) |
- |
pathogenic |
g.126094117_126094121del |
g.124405548_124405552del |
- |
- |
OAT_000047 |
- |
PubMed: Renner et al.2012 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
Anne Polvi |
+/. |
5 |
c.539G>C |
r.539g>c |
p.Arg180Thr |
- |
pathogenic |
g.126094114C>G |
g.124405545C>G |
- |
- |
OAT_000017 |
Mutant OAT protein was inactive. |
PubMed: Mitchell 1989 |
- |
- |
Germline |
yes |
0/19 FIN CON |
- |
- |
- |
Anne Polvi |
?/. |
5 |
c.539G>C |
r.539g>c |
p.Arg180Thr |
- |
VUS |
g.126094114C>G |
g.124405545C>G |
- |
- |
OAT_000017 |
Mutant OAT protein was inactive. |
PubMed: Mitchell 1989 |
- |
- |
Germline |
yes |
0/19 FIN CON |
- |
- |
- |
Anne Polvi |
+/+? |
5 |
c.539G>C |
r.(539g>c) |
p.(Arg180Thr) |
- |
pathogenic (recessive) |
g.126094114C>G |
g.124405545C>G |
Codon 180 AGG (Arg) > ACG (Thr) |
- |
OAT_000017 |
- |
PubMed: Mashima 1992 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
Anne Polvi |
+/+? |
5 |
c.539G>C |
r.(539g>c) |
p.(Arg180Thr) |
- |
pathogenic |
g.126094114C>G |
g.124405545C>G |
539G>C |
- |
OAT_000017 |
- |
PubMed: Brody 1992 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
Anne Polvi |
+/. |
- |
c.539G>C |
r.(?) |
p.(Arg180Thr) |
- |
pathogenic |
g.126094114C>G |
g.124405545C>G |
OAT R180T |
- |
OAT_000017 |
no nucleotide annotation, extrapolated from protein and databases; heterozygous |
PubMed: Peltola 2001 |
- |
- |
Unknown |
? |
- |
- |
- |
- |
LOVD |
+/. |
- |
c.539G>C |
r.(?) |
p.(Arg180Thr) |
- |
pathogenic |
g.126094114C>G |
g.124405545C>G |
OAT R180T |
- |
OAT_000017 |
no nucleotide annotation, extrapolated from protein and databases; heterozygous |
PubMed: Peltola 2001 |
- |
- |
Unknown |
? |
- |
- |
- |
- |
LOVD |
+/. |
- |
c.539G>C |
r.(?) |
p.(Arg180Thr) |
- |
pathogenic |
g.126094114C>G |
g.124405545C>G |
OAT R180T |
- |
OAT_000017 |
no nucleotide annotation, extrapolated from protein and databases; heterozygous |
PubMed: Peltola 2001 |
- |
- |
Unknown |
? |
- |
- |
- |
- |
LOVD |
+/+? |
5 |
c.542C>T |
r.(542C>u) |
p.(Thr181Met) |
- |
pathogenic |
g.126094111G>A |
g.124405542G>A |
Codon 181 ACG>ATG: Thr>Met |
- |
OAT_000049 |
- |
PubMed: Mashima 1996 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
Anne Polvi |
+?/. |
5 |
c.542C>T |
r.(?) |
p.(Thr181Met) |
- |
likely pathogenic (recessive) |
g.126094111G>A |
- |
c.542C>T |
- |
OAT_000049 |
- |
PubMed: Liu-2020 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
+/+? |
5 |
c.550G>A |
r.(550g>a) |
p.(Ala184Thr) |
- |
pathogenic |
g.126094103C>T |
g.124405534C>T |
- |
- |
OAT_000051 |
- |
PubMed: Michaud 1992 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
Anne Polvi |
+/+? |
5 |
c.550_552del |
r.(?) |
p.(Ala184del) |
- |
pathogenic |
g.126094103_126094105del |
g.124405534_124405536del |
550delGCT |
- |
OAT_000050 |
Portugese mutation. Mutation leads to production of inactive OAT protein. |
PubMed: Brody 1992 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
Anne Polvi |
?/. |
- |
c.578G>A |
r.(?) |
p.(Ser193Asn) |
- |
VUS |
g.126094075C>T |
- |
- |
- |
OAT_000102 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Nijmegen |
-?/. |
- |
c.582C>T |
r.(?) |
p.(Tyr194=) |
- |
likely benign |
g.126094071G>A |
g.124405502G>A |
OAT(NM_001322966.1):c.582C>T (p.Y194=), OAT(NM_001322966.2):c.582C>T (p.Y194=) |
- |
OAT_000083 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Rotterdam |
-?/. |
- |
c.582C>T |
r.(?) |
p.(Tyr194=) |
- |
likely benign |
g.126094071G>A |
- |
OAT(NM_001322966.1):c.582C>T (p.Y194=), OAT(NM_001322966.2):c.582C>T (p.Y194=) |
- |
OAT_000083 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_AMC |
+/+? |
5 |
c.583G>T |
r.(583g>u) |
p.(Asp195Tyr) |
- |
pathogenic |
g.126094070C>A |
g.124405501C>A |
Codon 195: GAT>TAT: Asp>Tyr |
- |
OAT_000052 |
- |
PubMed: Mashima 1996 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
Anne Polvi |
+/. |
5 |
c.596C>A |
r.596c>a |
p.Pro199Gln |
- |
pathogenic |
g.126094057G>T |
g.124405488G>T |
- |
- |
OAT_000008 |
- |
- |
- |
- |
Germline |
- |
- |
- |
- |
- |
Eva Trevisson |
+/+? |
5 |
c.596C>A |
r.(596c>a) |
p.(Pro199Gln) |
- |
pathogenic |
g.126094057G>T |
g.124405488G>T |
- |
- |
OAT_000008 |
- |
PubMed: Doimo 2012 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
Anne Polvi |
+/+? |
5 |
c.596C>A |
r.(596c>a) |
p.(Pro199Gln) |
- |
pathogenic |
g.126094057G>T |
g.124405488G>T |
- |
- |
OAT_000008 |
- |
PubMed: Sergouniotis 2012 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
Anne Polvi |
+/+? |
5 |
c.596C>A |
r.(596c>a) |
p.(Pro199Gln) |
- |
pathogenic |
g.126094057G>T |
g.124405488G>T |
- |
- |
OAT_000008 |
- |
PubMed: Sergouniotis 2012 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
Anne Polvi |
+/. |
- |
c.596C>A |
r.(?) |
p.(Pro199Gln) |
- |
pathogenic |
g.126094057G>T |
g.124405488G>T |
OAT c.596C>A, p.Pro199Gln |
- |
OAT_000008 |
compound heterozygous |
PubMed: Bell 2021 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
LOVD |
+/. |
- |
c.596C>A |
r.(?) |
p.(Pro199Gln) |
- |
pathogenic (recessive) |
g.126094057G>T |
g.124405488G>T |
- |
- |
OAT_000008 |
- |
PubMed: Mashima 1992 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/+? |
5 |
c.627T>A |
r.0 |
p.0 |
- |
pathogenic |
g.126094026A>T |
g.124405457A>T |
Codon 209 (exon 6) TAT (Try) > TAA (stop codon) |
- |
OAT_000040 |
- |
PubMed: Mashima 1992 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
Anne Polvi |
+?/. |
- |
c.627T>A |
r.(?) |
p.(Tyr209Ter) |
- |
likely pathogenic |
g.126094026A>T |
g.124405457A>T |
- |
- |
OAT_000040 |
- |
PubMed: Riera 2017 |
- |
- |
Germline |
yes |
- |
- |
- |
- |
LOVD |
+/. |
5 |
c.627T>A |
r.627u>a |
p.Tyr209* |
- |
pathogenic (recessive) |
g.126094026A>T |
- |
- |
- |
OAT_000040 |
- |
PubMed: Mashima 1992 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
-/. |
- |
c.648+14A>G |
r.(=) |
p.(=) |
- |
benign |
g.126093991T>C |
g.124405422T>C |
OAT(NM_000274.4):c.648+14A>G |
- |
OAT_000074 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Groningen |
-/. |
- |
c.648+14A>G |
r.(=) |
p.(=) |
- |
benign |
g.126093991T>C |
g.124405422T>C |
OAT(NM_000274.4):c.648+14A>G |
- |
OAT_000074 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Nijmegen |
+?/. |
6 |
c.661del |
r.(?) |
p.(Asp221Ilefs*9) |
- |
likely pathogenic (recessive) |
g.126092477del |
- |
c.661delG |
- |
OAT_000092 |
- |
PubMed: Liu-2020 |
- |
- |
Germline |
- |
- |
- |
- |
- |
LOVD |
+/. |
6 |
c.677C>T |
r.(?) |
p.(Ala226Val) |
- |
pathogenic |
g.126092461G>A |
g.124403892G>A |
- |
- |
OAT_000013 |
- |
PubMed: Brody 1992 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Eva Trevisson |