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_10_ |
c.-214_*368{0} |
r.0 |
p.0 |
- |
pathogenic |
g.(?_g.38211736)_(38280703_?)del |
g.(?_38352483)_(38421450_?)del |
delOTC gene >1.6 Mb |
- |
CYBB_000005 |
- |
PubMed: Climent 1999, PubMed: Martin-Hernandez 2014 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
_1_10_ |
c.-214_*368{0} |
r.0 |
p.0 |
- |
pathogenic |
g.(?_g.38211736)_(38280703_?)del |
g.(?_38352483)_(38421450_?)del |
delOTC gene >1.6 Mb |
- |
CYBB_000005 |
- |
PubMed: Martin-Hernandez 2014 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
_1_10_ |
c.-214_*368{0} |
r.0 |
p.0 |
- |
pathogenic |
g.(?_g.38211736)_(38280703_?)del |
g.(?_38352483)_(38421450_?)del |
del ex1-10 |
- |
CYBB_000005 |
- |
PubMed: Silvera-Ruiz 2019 |
- |
- |
Germline |
- |
5/24 case chromosomes |
- |
- |
- |
Johan den Dunnen |
+/. |
_1_10_ |
c.[-214_(1005+1_1006-1){2};(1005+1_1006-1)*368{0}] |
r.? |
p.? |
- |
pathogenic |
g.[(?_g.38211736)_(38271253_38280275)dup;(38271253_38280275)_(38280703_?)del] |
g.[(?_38352483)_(38412000_38421022)dup;(38412000_38421022)-(38421450_?)del] |
dup ex1-9/del ex10 |
- |
OTC_000442 |
- |
PubMed: Silvera-Ruiz 2019 |
- |
- |
Germline |
- |
1/24 case chromosomes |
- |
- |
- |
Johan den Dunnen |
+/. |
_1_10_ |
c.-2802714_*618720del |
r.0 |
p.0 |
- |
pathogenic (recessive) |
g.35409236_38899055del |
- |
hg17 35168893-38655271del |
- |
CYBB_000481 |
deletion incl. XK, CYBB, RPGR, OTC, TSPAN7 |
PubMed: Yamada 2010 |
- |
- |
De novo |
- |
- |
- |
- |
- |
Johan den Dunnen |
?/. |
1 |
c.-515_-512del |
r.(?) |
p.(?) |
- |
VUS |
g.38211435_38211438del |
g.38352182_38352185del |
-512insTCTT |
- |
OTC_000257 |
- |
PubMed: Azevedo 2003 |
- |
rs57752938 |
Germline |
- |
2/36 |
MboII |
- |
- |
Johan den Dunnen |
?/. |
1 |
c.-515_-512del |
r.(?) |
p.(?) |
- |
VUS |
g.38211435_38211438del |
g.38352182_38352185del |
-512insTCTT |
- |
OTC_000257 |
- |
PubMed: Azevedo 2003 |
- |
rs57752938 |
Germline |
- |
16/103 |
MboII |
- |
- |
Johan den Dunnen |
?/. |
1 |
c.-515_-512del |
r.(?) |
p.(?) |
- |
VUS |
g.38211435_38211438del |
g.38352182_38352185del |
-512insTCTT |
- |
OTC_000257 |
- |
PubMed: Azevedo 2003 |
- |
rs57752938 |
Germline |
- |
1/57 |
MboII |
- |
- |
Johan den Dunnen |
?/. |
1 |
c.-515_-512del |
r.(?) |
p.(?) |
- |
VUS |
g.38211435_38211438del |
g.38352182_38352185del |
-512insTCTT |
- |
OTC_000257 |
- |
PubMed: Azevedo 2003 |
- |
rs57752938 |
Germline |
- |
13/57 |
MboII |
- |
- |
Johan den Dunnen |
?/. |
1 |
c.-515_-512del |
r.(?) |
p.(?) |
- |
VUS |
g.38211435_38211438del |
g.38352182_38352185del |
-512insTCTT |
- |
OTC_000257 |
- |
PubMed: Azevedo 2003 |
- |
rs57752938 |
Germline |
- |
7/57 |
MboII |
- |
- |
Johan den Dunnen |
-/. |
1 |
c.-515_-512del |
r.(?) |
p.(?) |
- |
benign |
g.38211435_38211438del |
g.38352182_38352185del |
-512insTCTT |
- |
OTC_000257 |
- |
PubMed: Azevedo 2003 |
- |
rs57752938 |
Germline |
- |
13/103 |
MboII |
- |
- |
Johan den Dunnen |
-/. |
1 |
c.-515_-512del |
r.(?) |
p.(?) |
- |
benign |
g.38211435_38211438del |
g.38352182_38352185del |
-512insTCTT |
- |
OTC_000257 |
- |
PubMed: Azevedo 2003 |
- |
rs57752938 |
Germline |
- |
18/36 |
MboII |
- |
- |
Johan den Dunnen |
-/. |
1 |
c.-515_-512del |
r.(?) |
p.(?) |
- |
benign |
g.38211435_38211438del |
g.38352182_38352185del |
-512insTCTT |
- |
OTC_000257 |
- |
PubMed: Azevedo 2003 |
- |
rs57752938 |
Germline |
- |
21/57 |
MboII |
- |
- |
Johan den Dunnen |
-/. |
1 |
c.-515_-512del |
r.(?) |
p.(?) |
- |
benign |
g.38211435_38211438del |
g.38352182_38352185del |
-512insTCTT |
- |
OTC_000257 |
- |
PubMed: Azevedo 2003 |
- |
rs57752938 |
Germline |
- |
38/103 |
MboII |
- |
- |
Johan den Dunnen |
-/. |
1 |
c.-515_-512del |
r.(?) |
p.(?) |
- |
benign |
g.38211435_38211438del |
g.38352182_38352185del |
-512insTCTT |
- |
OTC_000257 |
- |
PubMed: Azevedo 2003 |
- |
rs57752938 |
Germline |
- |
7/36 |
MboII |
- |
- |
Johan den Dunnen |
-/. |
1 |
c.-515_-512del |
r.(?) |
p.(?) |
- |
benign |
g.38211435_38211438del |
g.38352182_38352185del |
-512insTCTT |
- |
OTC_000257 |
- |
PubMed: Azevedo 2003 |
- |
rs57752938 |
Germline |
- |
3/57 |
MboII |
- |
- |
Johan den Dunnen |
-/. |
1 |
c.-515_-512del |
r.(?) |
p.(?) |
- |
benign |
g.38211435_38211438del |
g.38352182_38352185del |
-512insTCTT |
- |
OTC_000257 |
- |
PubMed: Azevedo 2003 |
- |
rs5917572 |
Germline |
- |
3/103 |
MboII |
- |
- |
Johan den Dunnen |
?/. |
1 |
c.-441A>G |
r.(?) |
p.(?) |
- |
VUS |
g.38211509A>G |
g.38352256A>G |
(-441G>A) |
- |
OTC_000256 |
- |
PubMed: Azevedo 2003 |
- |
rs5917572 |
Germline |
- |
2/36 |
- |
- |
- |
Johan den Dunnen |
?/. |
1 |
c.-441A>G |
r.(?) |
p.(?) |
- |
VUS |
g.38211509A>G |
g.38352256A>G |
(-441G>A) |
- |
OTC_000256 |
- |
PubMed: Azevedo 2003 |
- |
rs5917572 |
Germline |
- |
16/103 |
- |
- |
- |
Johan den Dunnen |
?/. |
1 |
c.-441A>G |
r.(?) |
p.(?) |
- |
VUS |
g.38211509A>G |
g.38352256A>G |
(-441G>A) |
- |
OTC_000256 |
- |
PubMed: Azevedo 2003 |
- |
rs5917572 |
Germline |
- |
1/57 |
- |
- |
- |
Johan den Dunnen |
?/. |
1 |
c.-441A>G |
r.(?) |
p.(?) |
- |
VUS |
g.38211509A>G |
g.38352256A>G |
(-441G>A) |
- |
OTC_000256 |
- |
PubMed: Azevedo 2003 |
- |
rs5917572 |
Germline |
- |
13/57 |
- |
- |
- |
Johan den Dunnen |
?/. |
1 |
c.-441A>G |
r.(?) |
p.(?) |
- |
VUS |
g.38211509A>G |
g.38352256A>G |
(-441G>A) |
- |
OTC_000256 |
- |
PubMed: Azevedo 2003 |
- |
rs5917572 |
Germline |
- |
7/57 |
- |
- |
- |
Johan den Dunnen |
-/. |
1 |
c.-441A>G |
r.(?) |
p.(?) |
- |
benign |
g.38211509A>G |
g.38352256A>G |
(-441G>A) |
- |
OTC_000256 |
- |
PubMed: Azevedo 2003 |
- |
rs5917572 |
Germline |
- |
13/103 |
- |
- |
- |
Johan den Dunnen |
-/. |
1 |
c.-441A>G |
r.(?) |
p.(?) |
- |
benign |
g.38211509A>G |
g.38352256A>G |
(-441G>A) |
- |
OTC_000256 |
- |
PubMed: Azevedo 2003 |
- |
rs5917572 |
Germline |
- |
18/36 |
- |
- |
- |
Johan den Dunnen |
-/. |
1 |
c.-441A>G |
r.(?) |
p.(?) |
- |
benign |
g.38211509A>G |
g.38352256A>G |
(-441G>A) |
- |
OTC_000256 |
- |
PubMed: Azevedo 2003 |
- |
rs5917572 |
Germline |
- |
21/57 |
- |
- |
- |
Johan den Dunnen |
-/. |
1 |
c.-441A>G |
r.(?) |
p.(?) |
- |
benign |
g.38211509A>G |
g.38352256A>G |
(-441G>A) |
- |
OTC_000256 |
- |
PubMed: Azevedo 2003 |
- |
rs5917572 |
Germline |
- |
38/103 |
- |
- |
- |
Johan den Dunnen |
-/. |
1 |
c.-441A>G |
r.(?) |
p.(?) |
- |
benign |
g.38211509A>G |
g.38352256A>G |
(-441G>A) |
- |
OTC_000256 |
- |
PubMed: Azevedo 2003 |
- |
rs5917572 |
Germline |
- |
7/36 |
- |
- |
- |
Johan den Dunnen |
-/. |
1 |
c.-441A>G |
r.(?) |
p.(?) |
- |
benign |
g.38211509A>G |
g.38352256A>G |
(-441G>A) |
- |
OTC_000256 |
- |
PubMed: Azevedo 2003 |
- |
rs5917572 |
Germline |
- |
3/57 |
- |
- |
- |
Johan den Dunnen |
-/. |
1 |
c.-441A>G |
r.(?) |
p.(?) |
- |
benign |
g.38211509A>G |
g.38352256A>G |
(-441G>A) |
- |
OTC_000256 |
- |
PubMed: Azevedo 2003 |
- |
rs5917572 |
Germline |
- |
3/103 |
- |
- |
- |
Johan den Dunnen |
-/. |
1 |
c.-441A>G |
r.(?) |
p.(?) |
- |
benign |
g.38211509A>G |
g.38352256A>G |
(-441G>A) |
- |
OTC_000256 |
- |
PubMed: Azevedo 2003 |
- |
rs5917572 |
Germline |
- |
1/36 |
- |
- |
- |
Johan den Dunnen |
-/. |
1 |
c.-441A>G |
r.(?) |
p.(?) |
- |
benign |
g.38211509A>G |
g.38352256A>G |
(-441G>A) |
- |
OTC_000256 |
- |
PubMed: Azevedo 2003 |
- |
rs5917572 |
Germline |
- |
1/57 |
- |
- |
- |
Johan den Dunnen |
+/. |
1 |
c.-366A>G |
r.(=) |
p.(=) |
- |
pathogenic |
g.38211584A>G |
g.38352331A>G |
- |
- |
OTC_000400 |
not in 300 control chromosomes |
- |
- |
- |
Germline |
- |
- |
- |
- |
- |
Lenka Dvořáková |
?/. |
1 |
c.-365G>A |
r.(?) |
p.(?) |
- |
VUS |
g.38211585G>A |
g.38352332G>A |
- |
- |
OTC_000255 |
- |
PubMed: Azevedo 2003 |
- |
rs5963030 |
Germline |
- |
2/36 |
TseI;Fnu4HI;BbvI |
- |
- |
Johan den Dunnen |
?/. |
1 |
c.-365G>A |
r.(?) |
p.(?) |
- |
VUS |
g.38211585G>A |
g.38352332G>A |
- |
- |
OTC_000255 |
- |
PubMed: Azevedo 2003 |
- |
rs5963030 |
Germline |
- |
16/103 |
TseI;Fnu4HI;BbvI |
- |
- |
Johan den Dunnen |
?/. |
1 |
c.-365G>A |
r.(?) |
p.(?) |
- |
VUS |
g.38211585G>A |
g.38352332G>A |
- |
- |
OTC_000255 |
- |
PubMed: Azevedo 2003 |
- |
rs5963030 |
Germline |
- |
1/57 |
TseI;Fnu4HI;BbvI |
- |
- |
Johan den Dunnen |
?/. |
1 |
c.-365G>A |
r.(?) |
p.(?) |
- |
VUS |
g.38211585G>A |
g.38352332G>A |
- |
- |
OTC_000255 |
- |
PubMed: Azevedo 2003 |
- |
rs5963030 |
Germline |
- |
7/57 |
TseI;Fnu4HI;BbvI |
- |
- |
Johan den Dunnen |
-/. |
1 |
c.-359A>G |
r.(?) |
p.(?) |
- |
benign |
g.38211591A>G |
g.38352338A>G |
- |
- |
OTC_000254 |
Variant Error [EREF/ERANGE]: This genomic variant does not match the reference sequence; the transcript variant also has an error. Please fix this entry and then remove this message. |
PubMed: Azevedo 2003 |
- |
rs5917573 |
Germline |
- |
24/103 |
- |
- |
- |
Johan den Dunnen |
-/. |
1 |
c.-359A>G |
r.(?) |
p.(?) |
- |
benign |
g.38211591A>G |
g.38352338A>G |
- |
- |
OTC_000254 |
Variant Error [EREF/ERANGE]: This genomic variant does not match the reference sequence; the transcript variant also has an error. Please fix this entry and then remove this message. |
PubMed: Azevedo 2003 |
- |
rs5917573 |
Germline |
- |
2/36 |
- |
- |
- |
Johan den Dunnen |
-/. |
1 |
c.-359A>G |
r.(?) |
p.(?) |
- |
benign |
g.38211591A>G |
g.38352338A>G |
- |
- |
OTC_000254 |
Variant Error [EREF/ERANGE]: This genomic variant does not match the reference sequence; the transcript variant also has an error. Please fix this entry and then remove this message. |
PubMed: Azevedo 2003 |
- |
rs5917573 |
Germline |
- |
6/57 |
- |
- |
- |
Johan den Dunnen |
-/. |
1 |
c.-359A>G |
r.(?) |
p.(?) |
- |
benign |
g.38211591A>G |
g.38352338A>G |
- |
- |
OTC_000254 |
Variant Error [EREF/ERANGE]: This genomic variant does not match the reference sequence; the transcript variant also has an error. Please fix this entry and then remove this message. |
PubMed: Azevedo 2003 |
- |
rs5917573 |
Germline |
- |
1/36 |
- |
- |
- |
Johan den Dunnen |
-/. |
1 |
c.-359A>G |
r.(?) |
p.(?) |
- |
benign |
g.38211591A>G |
g.38352338A>G |
- |
- |
OTC_000254 |
Variant Error [EREF/ERANGE]: This genomic variant does not match the reference sequence; the transcript variant also has an error. Please fix this entry and then remove this message. |
PubMed: Azevedo 2003 |
- |
rs5917573 |
Germline |
- |
1/57 |
- |
- |
- |
Johan den Dunnen |
-/. |
1 |
c.-359A>G |
r.(?) |
p.(?) |
- |
benign |
g.38211591A>G |
g.38352338A>G |
- |
- |
OTC_000254 |
Variant Error [EREF/ERANGE]: This genomic variant does not match the reference sequence; the transcript variant also has an error. Please fix this entry and then remove this message. |
PubMed: Azevedo 2003 |
- |
rs5917573 |
Germline |
- |
3/57 |
- |
- |
- |
Johan den Dunnen |
-/. |
1 |
c.-359A>G |
r.(?) |
p.(?) |
- |
benign |
g.38211591A>G |
g.38352338A>G |
- |
- |
OTC_000254 |
Variant Error [EREF/ERANGE]: This genomic variant does not match the reference sequence; the transcript variant also has an error. Please fix this entry and then remove this message. |
PubMed: Azevedo 2003 |
- |
rs5917573 |
Germline |
- |
4/103 |
- |
- |
- |
Johan den Dunnen |
-/. |
1 |
c.-359A>G |
r.(?) |
p.(?) |
- |
benign |
g.38211591A>G |
g.38352338A>G |
- |
- |
OTC_000254 |
Variant Error [EREF/ERANGE]: This genomic variant does not match the reference sequence; the transcript variant also has an error. Please fix this entry and then remove this message. |
PubMed: Azevedo 2003 |
- |
rs5917573 |
Germline |
- |
6/36 |
- |
- |
- |
Johan den Dunnen |
-/. |
1 |
c.-359A>G |
r.(?) |
p.(?) |
- |
benign |
g.38211591A>G |
g.38352338A>G |
- |
- |
OTC_000254 |
Variant Error [EREF/ERANGE]: This genomic variant does not match the reference sequence; the transcript variant also has an error. Please fix this entry and then remove this message. |
PubMed: Azevedo 2003 |
- |
rs5917573 |
Germline |
- |
2/57 |
- |
- |
- |
Johan den Dunnen |
-/. |
1 |
c.-359A>G |
r.(?) |
p.(?) |
- |
benign |
g.38211591A>G |
g.38352338A>G |
- |
- |
OTC_000254 |
Variant Error [EREF/ERANGE]: This genomic variant does not match the reference sequence; the transcript variant also has an error. Please fix this entry and then remove this message. |
PubMed: Azevedo 2003 |
- |
rs5917573 |
Germline |
- |
4/103 |
- |
- |
- |
Johan den Dunnen |
+/. |
_1_3i |
c.(?_-1)_(298+1_299-1)del |
r.0? |
p.0? |
- |
pathogenic |
g.(?_38211949)_(38229131_38240594)del |
- |
- |
- |
OTC_000098 |
deletion exons 1-3 |
PubMed: Tuchman 1997 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Bert Bakker |
+/. |
_1_8i |
c.(?_-1)_(867+1_868-1)del |
r.0 |
p.0 |
- |
pathogenic |
g.(?_38211949)_(38268279_38271114)del |
- |
- |
- |
OTC_000357 |
- |
PubMed: Yamaguchi 2006 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Bert Bakker |
+/. |
_1_9i |
c.(?_-1)_(1005+1_1006-1)del |
r.0 |
p.0 |
- |
pathogenic |
g.(?_38211949)_(38271253_38280275)del |
- |
- |
- |
OTC_000355 |
- |
PubMed: Yamaguchi 2006 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Bert Bakker |
?/. |
_1_10_ |
c.(?_-1)_(*1_?)del |
r.0 |
p.0 |
- |
VUS |
g.(?_38211949)_(38280336_?)del |
- |
- |
- |
OTC_000210 |
deletion entire OTC gene |
PubMed: Suess 1992 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Bert Bakker |
?/. |
_1_10_ |
c.(?_-1)_(*1_?)del |
r.0 |
p.0 |
- |
VUS |
g.(?_38211949)_(38280336_?)del |
- |
- |
- |
OTC_000210 |
- |
PubMed: Yamaguchi 2006 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Bert Bakker |
?/. |
_1_10_ |
c.(?_-1)_(*1_?)del |
r.0 |
p.0 |
- |
VUS |
g.(?_38211949)_(38280336_?)del |
- |
- |
- |
OTC_000210 |
deletion entire OTC gene |
PubMed: Tuchman 2002 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Bert Bakker |
?/. |
1 |
c.1A>G |
r.(?) |
p.0? |
- |
VUS |
g.38211950A>G |
g.38352697A>G |
- |
- |
OTC_000099 |
- |
PubMed: Oppliger Leibundgut 1997 |
- |
- |
Germline |
- |
- |
MboII;SfaNI |
- |
- |
Bert Bakker |
?/. |
1 |
c.1A>T |
r.(?) |
p.(Met1Leu) |
- |
VUS |
g.38211950A>T |
g.38352697A>T |
- |
- |
OTC_000361 |
- |
PubMed: Yamaguchi 2006 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Bert Bakker |
?/. |
1 |
c.2T>C |
r.(?) |
p.(Met1Thr) |
- |
VUS |
g.38211951T>C |
g.38352698T>C |
- |
- |
OTC_000365 |
- |
PubMed: Yamaguchi 2006 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Bert Bakker |
?/. |
1 |
c.3G>A |
r.(?) |
p.0? |
- |
VUS |
g.38211952G>A |
g.38352699G>A |
- |
- |
OTC_000211 |
- |
PubMed: Climent and Rubio 2002 |
- |
- |
Germline |
- |
- |
SfaNI;HpyCH4III |
- |
- |
Bert Bakker |
?/. |
1 |
c.26T>G |
r.(?) |
p.(Leu9X) |
- |
VUS |
g.38211975T>G |
g.38352722T>G |
c.25T>G |
- |
OTC_000264 |
not in 100 control chromosomes; OTC activity 0, Serum ammonia 430, Urine orotic acid 532, Serum citrulline 33.5, |
PubMed: Kim 2006 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Bert Bakker |
?/. |
1 |
c.29dup |
r.(?) |
p.(Asn10LysfsTer6) |
- |
VUS |
g.38211978dup |
g.38352725dup |
- |
- |
OTC_000375 |
- |
PubMed: Yamaguchi 2006 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Bert Bakker |
?/. |
1 |
c.29_32del |
r.(?) |
p.(Asn10MetfsTer27) |
- |
VUS |
g.38211978_38211981del |
g.38352725_38352728del |
- |
- |
OTC_000370 |
- |
PubMed: Yamaguchi 2006 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Bert Bakker |
?/. |
1 |
c.34G>A |
r.(?) |
p.(Ala12Thr) |
- |
VUS |
g.38211983G>A |
g.38352730G>A |
- |
- |
OTC_000260 |
- |
Tanaka 2005 |
- |
- |
Germline |
- |
- |
TseI;CviRI;Fnu4HI;BbvI |
- |
- |
Bert Bakker |
?/. |
1 |
c.42del |
r.(?) |
p.(Phe14LeufsTer24) |
- |
VUS |
g.38211991del |
g.38352738del |
- |
- |
OTC_000379 |
Leader sequence; OTC activity 23% |
Hwu 2003 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Bert Bakker |
-/. |
1 |
c.46A>G |
r.(?) |
p.(Asn16Asp ) |
- |
benign |
g.38211995A>G |
g.38352742A>G |
- |
- |
OTC_000390 |
100 individuals tested |
PubMed: Mitchell 2009 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
-/. |
1 |
c.46A>G |
r.(?) |
p.(Asn16Asp) |
- |
benign |
g.38211995A>G |
g.38352742A>G |
- |
- |
OTC_000390 |
- |
PubMed: Mitchell 2009 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
?/. |
1 |
c.53del |
r.(?) |
p.(His18ProfsTer20) |
- |
VUS |
g.38212002del |
g.38352749del |
- |
- |
OTC_000212 |
- |
PubMed: Tuchman 2002 |
- |
- |
Germline |
- |
- |
AvaII |
- |
- |
Bert Bakker |
?/. |
1 |
c.67C>T |
r.(?) |
p.(Arg23X) |
- |
VUS |
g.38212016C>T |
g.38352763C>T |
- |
- |
OTC_000011 |
Leader sequence |
Grompe 1991 |
- |
- |
Germline |
- |
- |
TaqI;AsuII |
- |
- |
Bert Bakker |
+/. |
- |
c.67C>T |
r.(?) |
p.(Arg23Ter) |
- |
pathogenic |
g.38212016C>T |
g.38352763C>T |
- |
- |
OTC_000011 |
- |
PubMed: Martin-Hernandez 2014 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
-?/. |
- |
c.76C>T |
r.(?) |
p.(Arg26Trp) |
- |
likely benign |
g.38212025C>T |
g.38352772C>T |
OTC(NM_000531.5):c.76C>T (p.R26W, p.(Arg26Trp)) |
- |
OTC_000413 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Leiden |
-?/. |
- |
c.76C>T |
r.(?) |
p.(Arg26Trp) |
- |
likely benign |
g.38212025C>T |
- |
OTC(NM_000531.5):c.76C>T (p.R26W, p.(Arg26Trp)) |
- |
OTC_000413 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Rotterdam |
?/. |
1 |
c.77G>A |
r.(spl?) |
p.(Arg26Gln) |
- |
VUS |
g.38212026G>A |
g.38352773G>A |
- |
- |
OTC_000003 |
OTC activity 0% |
Grompe 1989 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Bert Bakker |
?/. |
1 |
c.77G>A |
r.(spl?) |
p.(Arg26Gln) |
- |
VUS |
g.38212026G>A |
g.38352773G>A |
- |
- |
OTC_000003 |
OTC activity ?, Serum ammonia 1260, Urine orotic acid 3685, Serum citrulline 0, |
PubMed: Kim 2006 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Bert Bakker |
+/. |
- |
c.77G>A |
r.(?) |
p.(Arg26Gln) |
- |
pathogenic |
g.38212026G>A |
g.38352773G>A |
- |
- |
OTC_000003 |
1 heterozygous, no homozygous; Clinindb (India) |
PubMed: Narang 2020, Journal: Narang 2020 |
- |
rs68031618 |
Germline |
- |
1/2795 individuals |
- |
- |
- |
Mohammed Faruq |
?/. |
1 |
c.77G>C |
r.(spl?) |
p.(Arg26Pro) |
- |
VUS |
g.38212026G>C |
g.38352773G>C |
- |
- |
OTC_000270 |
Leader sequence, CpG dinucleotide, possible donor splice site error |
PubMed: Yamaguchi 2006 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Bert Bakker |
?/. |
1 |
c.77G>C |
r.(spl?) |
p.(Arg26Pro) |
- |
VUS |
g.38212026G>C |
g.38352773G>C |
- |
- |
OTC_000270 |
not in 100 control chromosomes; OTC activity 0, Serum ammonia 280, Urine orotic acid 783, Serum citrulline 11.2 |
PubMed: Kim 2006 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Bert Bakker |
?/. |
1i |
c.77+1G>A |
r.spl? |
p.(?) |
- |
VUS |
g.38212027G>A |
g.38352774G>A |
- |
- |
OTC_000298 |
- |
PubMed: Yamaguchi 2006 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Bert Bakker |
?/. |
1i |
c.77+1G>T |
r.spl |
p.(?) |
- |
VUS |
g.38212027G>T |
g.38352774G>T |
c.IVS1+1G>T |
- |
OTC_000100 |
- |
PubMed: Tuchman 1997 |
- |
- |
Germline |
- |
- |
MseI |
- |
- |
Bert Bakker |
?/. |
1i |
c.77+2dup |
r.spl? |
p.? |
- |
VUS |
g.38212028dup |
g.38352775dup |
- |
- |
OTC_000302 |
- |
PubMed: Yamaguchi 2006 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Bert Bakker |
+?/. |
1i |
c.77+3_77+6del |
r.spl |
p.? |
- |
likely pathogenic |
g.38212029_38212032del |
g.38352776_38352779del |
c.IVS1+3_6delAAGT |
- |
OTC_000213 |
- |
PubMed: Tuchman 2002 |
- |
- |
Germline |
- |
- |
HpHI |
- |
- |
Bert Bakker |
?/. |
1i |
c.77+4A>C |
r.spl |
p.(?) |
- |
VUS |
g.38212030A>C |
g.38352777A>C |
c.IVS1+4A>C |
- |
OTC_000078 |
OTC activity 0% |
Hoshide 1996 |
- |
- |
Germline |
- |
- |
TspRI |
- |
- |
Bert Bakker |
?/. |
1i |
c.77+5G>A |
r.spl |
p.(?) |
- |
VUS |
g.38212031G>A |
g.38352778G>A |
c.IVS1+5G>A |
- |
OTC_000101 |
- |
PubMed: Tuchman 1997 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Bert Bakker |
?/. |
- |
c.77+437C>A |
r.(=) |
p.(=) |
- |
VUS |
g.38212463C>A |
g.38353210C>A |
- |
- |
OTC_000401 |
- |
- |
- |
- |
Germline |
- |
- |
- |
- |
- |
Yu Sun |
+/. |
1i |
c.77+6024_216+474del |
r.[78_298del, 78_386del] |
p.del |
- |
pathogenic |
g.38218050_38227156del |
g.38358797_38367903del |
- |
- |
OTC_000394 |
9095 bpdeletion (incl. exon 2) |
PubMed: Engel 2008 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
?/. |
1i |
c.78-3C>G |
r.77_78insag |
p.fsX |
- |
VUS |
g.38226541C>G |
g.38367288C>G |
- |
- |
OTC_000312 |
OTC activity below 5% |
Bisanzi 2002 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Bert Bakker |
?/. |
1i |
c.78-1G>C |
r.spl? |
p.(?) |
- |
VUS |
g.38226543G>C |
g.38367290G>C |
- |
- |
OTC_000314 |
- |
PubMed: Yamaguchi 2006 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Bert Bakker |
+/. |
1i_4i |
c.(77+1_78-1)_(386+1_387-1)del |
r.? |
p.? |
- |
pathogenic |
g.(38212027_38226543)_(38240683_38260527)del |
- |
- |
- |
OTC_000358 |
- |
PubMed: Yamaguchi 2006 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Bert Bakker |
-?/. |
- |
c.83G>A |
r.(?) |
p.(Gly28Glu) |
- |
likely benign |
g.38226549G>A |
g.38367296G>A |
OTC(NM_000531.5):c.83G>A (p.G28E) |
- |
OTC_000404 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Rotterdam |
?/. |
2 |
c.94C>T |
r.(?) |
p.(Gln32X) |
- |
VUS |
g.38226560C>T |
g.38367307C>T |
- |
- |
OTC_000108 |
- |
PubMed: Oppliger Leibundgut 1997 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Bert Bakker |
?/. |
2 |
c.106C>T |
r.(?) |
p.(Gln36X) |
- |
VUS |
g.38226572C>T |
g.38367319C>T |
- |
- |
OTC_000181 |
- |
PubMed: Genet 2000 |
- |
- |
Germline |
- |
- |
Fnu4HI;CviRI |
- |
- |
Bert Bakker |
?/. |
2 |
c.115G>T |
r.(?) |
p.(Gly39Cys) |
- |
VUS |
g.38226581G>T |
g.38367328G>T |
- |
- |
OTC_000140 |
- |
Calvas 1998 |
- |
- |
Germline |
- |
- |
HaeIII;Sau96I |
- |
- |
Bert Bakker |
?/. |
2 |
c.118C>T |
r.(?) |
p.(Arg40Cys) |
- |
VUS |
g.38226584C>T |
g.38367331C>T |
- |
- |
OTC_000060 |
- |
Oppliger Leibundgut 1995 |
- |
- |
Germline |
- |
- |
HaeIII |
- |
- |
Bert Bakker |
?/. |
2 |
c.119G>A |
r.(?) |
p.(Arg40His) |
- |
VUS |
g.38226585G>A |
g.38367332G>A |
- |
- |
OTC_000046 |
OTC activity 6% |
PubMed: Tuchman 1994b |
- |
- |
Germline |
- |
- |
MaeIII;Tsp45I |
- |
- |
Bert Bakker |
+?/. |
- |
c.119G>A |
r.(?) |
p.(Arg40His) |
- |
likely pathogenic |
g.38226585G>A |
g.38367332G>A |
- |
- |
OTC_000046 |
- |
- |
- |
- |
Germline |
- |
- |
- |
- |
- |
Sha Hong |
+/. |
- |
c.119G>A |
r.(?) |
p.(Arg40His) |
- |
pathogenic |
g.38226585G>A |
g.38367332G>A |
- |
- |
OTC_000046 |
- |
PubMed: Martin-Hernandez 2014 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
- |
c.119G>A |
r.(?) |
p.(Arg40His) |
- |
pathogenic |
g.38226585G>A |
g.38367332G>A |
- |
- |
OTC_000046 |
- |
PubMed: Martin-Hernandez 2014 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
- |
c.119G>A |
r.(?) |
p.(Arg40His) |
- |
pathogenic |
g.38226585G>A |
g.38367332G>A |
- |
- |
OTC_000046 |
- |
PubMed: Martin-Hernandez 2014 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
+/. |
- |
c.119G>A |
r.(?) |
p.(Arg40His) |
- |
pathogenic |
g.38226585G>A |
g.38367332G>A |
- |
- |
OTC_000046 |
- |
PubMed: Martin-Hernandez 2014 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Johan den Dunnen |
?/. |
2 |
c.122A>G |
r.(?) |
p.(Asp41Gly) |
- |
VUS |
g.38226588A>G |
g.38367335A>G |
- |
- |
OTC_000334 |
- |
PubMed: Yamaguchi 2006 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Bert Bakker |
?/. |
2 |
c.127C>T |
r.(?) |
p.(Leu43Phe) |
- |
VUS |
g.38226593C>T |
g.38367340C>T |
- |
- |
OTC_000102 |
- |
PubMed: Oppliger Leibundgut 1997 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Bert Bakker |
?/. |
- |
c.130A>G |
r.(?) |
p.(Thr44Ala) |
- |
VUS |
g.38226596A>G |
g.38367343A>G |
OTC(NM_000531.5):c.130A>G (p.T44A) |
- |
OTC_000411 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Rotterdam |
?/. |
2 |
c.131C>T |
r.(?) |
p.(Thr44IIe) |
- |
VUS |
g.38226597C>T |
g.38367344C>T |
- |
- |
OTC_000079 |
OTC activity 0, Serum Ammonia 389, Urine orotic acid 238, Serum citrulline 28.0 |
PubMed: Yoo 1996; PubMed: Kim 2006 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Bert Bakker |
?/. |
2 |
c.133C>G |
r.(?) |
p.(Leu45Val) |
- |
VUS |
g.38226599C>G |
g.38367346C>G |
- |
- |
OTC_000141 |
- |
PubMed: Tuchman 1998 |
- |
- |
Germline |
- |
- |
HpyCH4III;TspRI |
- |
- |
Bert Bakker |