Global Variome shared LOVD
AIRE (autoimmune regulator)
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Curator:
Roberto Perniola
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Unique variants in the AIRE gene
The variants shown are described using the NM_000383.3 transcript reference sequence.
Legend
Please note that a short description of a certain column can be displayed when you move your mouse cursor over the column's header and hold it still. Below, a more detailed description is shown per column.
Effect
: The variant's effect on the function of the gene/protein, displayed in the format 'R/C'. R is the value reported by the source (publication, submitter) and this classification may vary between records. C is the value concluded by the curator. Note that in some database the curator uses Summary records to give details on the classification of the variant.Values used: '+' indicating the variant affects function, '+?' probably affects function, '-' does not affect function, '-?' probably does not affect function, '?' effect unknown, '.' effect was not classified.
Reported
: The number of times this variant has been reported in the database.
Exon
: number of exon/intron containing variant; 2 = exon 2, 12i = intron 12, 2i_7i = from intron 2 to intron 7, 8i_9 = intron 8/exon 9 boundary, _1 = 5' to exon 1, 18_ = 3' of exon 18, _1_18_ = encompassing the entire 18-exon gene
DNA change (cDNA)
: description of variant at DNA level, based on a coding DNA reference sequence (following HGVS recommendations); e.g. c.123C>T, c.123_145del, c.123_126dup. For deletions/duplications extending beyond the reference transcript resp. {0}/{2} is used to replace del/dup. Extent of the deletion/duplication should be specified using the genomic description (g.). "-" indicates the variant described on genomic level does not affect the coding DNA reference sequence.
RNA change
: description of variant at RNA level (following HGVS recommendations).
r.123c>u
r.? = unknown
r.(?) = RNA not analysed but probably transcribed copy of DNA variant
r.spl? = RNA not analysed but variant probably affects splicing
r.(spl?) = RNA not analysed but variant may affect splicing
r.0? = change expected to abolish transcription
Protein
: description of variant at protein level (following HGVS recommendations).
p.(Arg345Pro) = change predicted from DNA (RNA not analysed)
p.Arg345Pro = change derived from RNA analysis
p.? = unknown effect
p.0? = probably no protein produced
Classification method
: The method used for the clinical classification of this variant.
All options:
ACMG
ACGS
EAHAD-CFDB
ENIGMA
IARC
InSiGHT
kConFab
other
Clinical classification
: Clinical classification of variant, preferably based on standardised criteria (e.g. ACMG), directed on the clinical consequences as published/submitted, indicated using an enriched system including inheritance: e.g. pathogenic, pathogenic (dominant), pathogenic (recessive), pathogenic (!), pathogenic (maternal), pathogenic (paternal). Standard inheritance is covered by dominant/recessive, imprinting by maternal/paternal. A '!' warns for exceptional circumstances to be explained in the 'Remarks' field (low penetrance, variants pathogenic in heterozygous state only, hypomorphic/hypermorphic variants, protective variants, etc.). Non-disease consequences (e.g. drug metabolism (pharmacogenetics), risk factor, blood group, tasting bitter) are indicated using additions to the benign classification; benign (dominant), benign (recessive), benign (!), etc. The value 'association' is used for variants associated with a phenotype and 'NA' for variants from in vitro/in silico records. NOTE: classification may differ from the opinion of the curator as given in a variant SUMMARY-record or the 'Functional effect concluded'). NOTE: pathogenic/likely pathogenic should go together with "variant (probably) affects function" In ClassFunctional.
All options:
pathogenic
pathogenic (dominant)
pathogenic (recessive)
pathogenic (!)
pathogenic (maternal)
pathogenic (paternal)
likely pathogenic
likely pathogenic (dominant)
likely pathogenic (recessive)
likely pathogenic (!)
likely pathogenic (maternal)
likely pathogenic (paternal)
VUS
VUS (!)
likely benign
likely benign (dominant)
likely benign (recessive)
likely benign (!)
likely benign (maternal)
likely benign (paternal)
benign
benign (dominant)
benign (recessive)
benign (!)
benign (maternal)
benign (paternal)
conflicting
association
NA
DNA change (genomic) (hg19)
: HGVS description of variant at DNA level, based on the genomic (chromosomal) DNA reference sequence; e.g. g.12345678C>T, g.12345679del, g.12345678_12345890dup
DNA change (hg38)
: HGVS description of variant at DNA level, based on the hg38 genomic (chromosomal) eference sequence; e.g. g.12345678C>T, g.12345679del, g.12345678_12345890dup
Published as
: listed only when different from "DNA change"; variant as reported originally (e.g. 521delT). Variants seen in animal models, tested in vitro, predicted from RNA analysis, etc. are described between brackets like c.(456C>G)
ISCN
: description of the variant according to ISCN nomenclature
DB-ID
: database ID of variant, grouping multiple observations of the same variant together, starting with the HGNC gene symbol, followed by an underscore (_) and a six digit number (e.g. DMD_012345). _000000 is used for variants where DNA was not analysed (change predicted from RNA analysis), variants seen in animal models or variants not seen in humans but functionally tested in vitro
Variant remarks
: remarks regarding variant described, e.g. germline mosaicism in mother, 345 kb deletion, muscle RNA analysed, not in 200 control chromosomes tested, on founder haplotype, etc.
Reference
: publication describing the variant submitted, incl. links to OMIM, PubMed or other source, e.g. "den Dunnen ASHG2003 P2346"
ClinVar ID
: ID of variant in ClinVar database
dbSNP ID
: the dbSNP ID
Origin
: Origin of variant/record: Germline = in all cells, De novo = in all cells, but not in either parent, Germline/De novo (untested) = in all cells, parents not tested (use only when De novo is likely, e.g. isolated/sporadic cases with dominant disease), Somatic = present in a subset of cells, but not in either parent, Uniparental disomy = from parental disomy (maternal or paternal), CLASSIFICATION record = submitter only sharing variant classification (note another report may share Individual data), SUMMARY record = master summary record from curator (may link to another database), In vitro (cloned) = data resulting from in vitro functional assays, animal model = data from animal model, Artefact = false positive variant call, DUPLICATE record = variant already described on another chromosome (e.g. unbalanced translocation, duplicating transposition, 2nd fusion transcript, etc.)
All options:
Germline
De novo
Germline/De novo (untested)
Somatic
Uniparental disomy
Uniparental disomy, maternal allele
Uniparental disomy, paternal allele
CLASSIFICATION record
SUMMARY record
In vitro (cloned)
In silico
animal model
Artefact
DUPLICATE record
Unknown
Not applicable
Segregation
: Indicates whether the variant segregates with the phenotype (yes), does not segregate with the phenotype (no) or segregation is unknown (?)
All options:
? = unknown
yes = segregates with phenotype
no = does not segregate with phenotype
- = not applicable
Frequency
: frequency in which the variant was found; e.g 5/760 chromosomes (in 5 of 760 chromosomes tested), 1/33 patients (in 1 of 33 patients analysed in study), 0.05 controls (in 5% of control cases tested)
Re-site
: restriction enzyme recognition site created (+) or destroyed (-); e.g. BglII+;BamHI-
VIP
: variant VIP-status was requested for matchmaking - need collaboration(s) to crack the case - please contact the submitter/curator. NOTE: to get VIP status ask the curator.
Methylation
: result of methylation test; GOM (gain of methylation), LOM (loss of methylation), 30% (30% methylated). NOTE: when several tests were done mention the method as well (e.g. MS-PCR 75%)
How to query this table
All list views have search fields which can be used to search data. You can search for a complete word or you can search for a part of a search term. If you enclose two or more words in double quotes, LOVD will search for the combination of those words only exactly in the order you specify. Note that search terms are case-insensitive and that wildcards such as * are treated as normal text! For all options, like "and", "or", and "not" searches, or searching for prefixes or suffixes, see the table below.
Operator
Column type
Example
Matches
Text
Arg
all entries containing 'Arg'
space
Text
Arg Ser
all entries containing 'Arg' and 'Ser'
|
Text
Arg|Ser
all entries containing 'Arg' or 'Ser'
!
Text
!fs
all entries not containing 'fs'
^
Text
^p.(Arg
all entries beginning with 'p.(Arg'
$
Text
Ser)$
all entries ending with 'Ser)'
=""
Text
=""
all entries with this field empty
=""
Text
="p.0"
all entries exactly matching 'p.0'
!=""
Text
!=""
all entries with this field not empty
!=""
Text
!="p.0"
all entries not exactly matching 'p.0?'
combination
Text
*|Ter !fs
all entries containing '*' or 'Ter' but not containing 'fs'
Date
2020
all entries matching the year 2020
|
Date
2020-03|2020-04
all entries matching March or April, 2020
!
Date
!2020-03
all entries not matching March, 2020
<
Date
<2020
all entries before the year 2020
<=
Date
<=2020-06
all entries in or before June, 2020
>
Date
>2020-06
all entries after June, 2020
>=
Date
>=2020-06-15
all entries on or after June 15th, 2020
combination
Date
2019|2020 <2020-03
all entries in 2019 or 2020, and before March, 2020
Numeric
23
all entries exactly matching 23
|
Numeric
23|24
all entries exactly matching 23 or 24
!
Numeric
!23
all entries not exactly matching 23
<
Numeric
<23
all entries lower than 23
<=
Numeric
<=23
all entries lower than, or equal to, 23
>
Numeric
>23
all entries higher than 23
>=
Numeric
>=23
all entries higher than, or equal to, 23
combination
Numeric
>=20 <30 !23
all entries with values from 20 to 29, but not equal to 23
Some more advanced examples:
Example
Matches
Asian
all entries containing 'Asian', 'asian', including 'Caucasian', 'caucasian', etc.
Asian !Caucasian
all entries containing 'Asian' but not containing 'Caucasian'
Asian|African !Caucasian
all entries containing 'Asian' or 'African', but not containing 'Caucasian'
"South Asian"
all entries containing 'South Asian', but not containing 'South East Asian'
To sort on a certain column, click on the column header or on the arrows. If that column is already selected to sort on, the sort order will be swapped. The column currently sorted on has a darker blue background color than the other columns. The up and down arrows next to the column name indicate the current sorting direction. When sorting on any field other than the default, LOVD will sort secondarily on the default sort column.
231 entries on 3 pages. Showing entries 1 - 100.
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Effect
Reported
Exon
DNA change (cDNA)
RNA change
Protein
Classification method
Clinical classification
DNA change (genomic) (hg19)
DNA change (hg38)
Published as
ISCN
DB-ID
Variant remarks
Reference
ClinVar ID
dbSNP ID
Origin
Segregation
Frequency
Re-site
VIP
Methylation
Owner
-?/?
1
1
c.-528G>A
r.?
p.=
-
likely benign
g.45705362G>A
g.44285479G>A
-
-
AIRE_000199
-
Lovewell et al. [2006]
-
-
Unknown
-
-
-
-
-
Roberto Perniola
-?/?
1
1
c.-103C>T
r.(?)
p.=
-
likely benign
g.45705787C>T
g.44285904C>T
-
-
AIRE_000211
-
Lovewell et al. [2006]
-
-
Unknown
-
-
-
-
-
Roberto Perniola
+/?
1
1
c.1A>G
r.(?)
p.0?
-
pathogenic
g.45705890A>G
g.44286007A>G
-
-
AIRE_000002
-
PubMed: Faiyaz-Ul-Haque 2009
-
-
Unknown
-
-
-
-
-
Roberto Perniola
+/?
1
1
c.1A>T
r.(?)
p.0?
-
pathogenic
g.45705890A>T
g.44286007A>T
-
-
AIRE_000001
ATG>TTG
PubMed: Stolarski 2006
-
-
Unknown
-
-
-
-
-
Roberto Perniola
+/?
1
1
c.2T>C
r.(?)
p.0?
-
pathogenic
g.45705891T>C
g.44286008T>C
-
-
AIRE_000003
ATG>ACG
PubMed: Orlova 2010
-
-
Unknown
-
-
-
-
-
Roberto Perniola
-?/.
1
-
c.10G>A
r.(?)
p.(Asp4Asn)
-
likely benign
g.45705899G>A
-
AIRE(NM_000383.3):c.10G>A (p.(Asp4Asn))
-
AIRE_000584
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+/?
4
1
c.21_43dup
r.(?)
p.(Arg15Hisfs*5)
-
pathogenic, pathogenic (recessive)
g.45705910_45705932dup
g.44286027_44286049dup
-, 21_43dup23
-
AIRE_000004
CGC>CAC
PubMed: Cihakova 2001
,
PubMed: Podkrajsek 2005
,
PubMed: Podkrajsek 2008
,
PubMed: Toth 2010
-
-
Germline, Unknown
-
-
-
-
-
Roberto Perniola
+/?
1
1
c.22C>T
r.(?)
p.(Arg8Cys)
-
pathogenic
g.45705911C>T
g.44286028C>T
-
-
AIRE_000005
CGC>TGC
PubMed: Wolff 2007
-
-
Unknown
-
-
-
-
-
Roberto Perniola
+/?
1
1
c.43C>T
r.(?)
p.(Arg15Cys)
-
pathogenic
g.45705932C>T
g.44286049C>T
-
-
AIRE_000006
CGC>TGC
PubMed: Sato 2002
-
-
Unknown
-
-
-
-
-
Roberto Perniola
+/?
1
1
c.44G>T
r.(?)
p.(Arg15Leu)
-
pathogenic
g.45705933G>T
g.44286050G>T
-
-
AIRE_000007
CGC>CTC
PubMed: Pearce 1998
-
-
Unknown
-
-
-
-
-
Roberto Perniola
+?/.
1
-
c.47C>G
r.(?)
p.(Thr16Arg)
-
likely pathogenic
g.45705936C>G
g.44286053C>G
-
-
AIRE_000522
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/?
6
1
c.47C>T
r.(?)
p.(Thr16Met)
-
pathogenic
g.45705936C>T
g.44286053C>T
-
-
AIRE_000008
ACG>ATG
Capalbo 2009 Horm Res, Valenzise 2010 Horm Res Paediatr,
PubMed: Cihakova 2001
,
PubMed: Mazza 2011
,
2 more items
-
-
Unknown
-
-
-
-
-
Roberto Perniola
+/?
1
1
c.55G>A
r.(?)
p.(Ala19Thr)
-
pathogenic
g.45705944G>A
g.44286061G>A
-
-
AIRE_000009
GCG>ACG
PubMed: Liu 2010
-
-
Unknown
-
-
-
-
-
Roberto Perniola
+/., +/?
4
1
c.62C>T
r.(?)
p.(Ala21Val)
-
pathogenic, pathogenic (recessive)
g.45705951C>T
g.44286068C>T
-
-
AIRE_000010
GCC>GTC
Capalbo 2009 Horm Res,
PubMed: Halonen 2002
,
PubMed: Mazza 2011
,
PubMed: Podkrajsek 2008
-
-
Germline, Unknown
-
-
-
-
-
Johan den Dunnen
,
Roberto Perniola
-?/., -?/?
3
1
c.63C>T
r.(?)
p.(Ala21=), p.Ala21 (silent)
-
likely benign
g.45705952C>T
g.44286069C>T
-, AIRE(NM_000383.2):c.63C>T (p.A21=)
-
AIRE_000135
VKGL data sharing initiative Nederland
Scott et al. [1998]
-
-
CLASSIFICATION record, Unknown
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_Utrecht
,
Roberto Perniola
+/?
1
1
c.64_69del
r.(?)
p.(Val22_Asp23del)
-
pathogenic
g.45705953_45705958del
g.44286070_44286075del
-
-
AIRE_000011
GCC GTG GAC AGC > GCC AGC
PubMed: Meloni 2005
-
-
Unknown
-
-
-
-
-
Roberto Perniola
-?/.
1
-
c.66G>T
r.(?)
p.(Val22=)
-
likely benign
g.45705955G>T
-
AIRE(NM_000383.2):c.66G>T (p.V22=)
-
AIRE_000571
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
+/?
2
1
c.83T>C
r.(?)
p.(Leu28Pro)
-
pathogenic
g.45705972T>C
g.44286089T>C
-
-
AIRE_000013
CTG>CCG
PubMed: Heino 1999
,
PubMed: Pearce 1998
-
-
Unknown
-
-
-
-
-
Roberto Perniola
+/?
1
1
c.86T>C
r.(?)
p.(Leu29Pro)
-
pathogenic
g.45705975T>C
g.44286092T>C
-
-
AIRE_000014
CTG>CCG
Kogawa 2002 Clin Immunol
-
-
Unknown
-
-
-
-
-
Roberto Perniola
+/?
1
1
c.93_94insT
r.(?)
p.(Leu32Serfs*3)
-
pathogenic
g.45705982_45705983insT
g.44286099_44286100insT
-
-
AIRE_000015
CTG>TCT
PubMed: Faiyaz-Ul-Haque 2009
-
-
Unknown
-
-
-
-
-
Roberto Perniola
-/.
3
-
c.99T>C
r.(?)
p.(Ala33=)
-
benign
g.45705988T>C
g.44286105T>C
AIRE(NM_000383.2):c.99T>C (p.A33=), AIRE(NM_000383.4):c.99T>C (p.A33=)
-
AIRE_000501
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
,
VKGL-NL_Utrecht
,
VKGL-NL_Nijmegen
?/.
1
-
c.122A>G
r.(?)
p.(Asp41Gly)
-
VUS
g.45706011A>G
-
AIRE(NM_000383.4):c.122A>G (p.(Asp41Gly))
-
AIRE_000580
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+/.
1
_1i_13i_
c.(?_132-57)_(1567-67_?)del
r.0?
p.0?
-
pathogenic (recessive)
g.(45578081_45705964)_(45717472_?)del
g.(?_44286081)_(44297589_5103720)del
c.(?_68)_(1567-14_?)del
-
AIRE_000170
deletion does not include D21S1912
PubMed: Podkrajsek 2008
-
-
Germline
-
-
-
-
-
Roberto Perniola
+/?
1
1_4
c.132_538del
r.?
p.?
-
pathogenic
g.(?_45706021)_(45707474_?)del
-
-
-
AIRE_000171
-
PubMed: Cihakova 2001
-
-
Unknown
-
-
-
-
-
Roberto Perniola
+/?, ?/.
2
1i
c.132+1G>C
r.spl, r.spl?
p.?
-
pathogenic, VUS
g.45706022G>C
g.44286139G>C
-, AIRE(NM_000383.4):c.132+1G>C
-
AIRE_000123
VKGL data sharing initiative Nederland
Freisinger 2005 Horm Res
-
-
CLASSIFICATION record, Unknown
-
-
-
-
-
VKGL-NL_Leiden
,
Roberto Perniola
+/?
3
1i
c.[132+1G>C;132+5del]
r.spl?
p.?
-
pathogenic
g.[45706022G>C;45706026del]
-
-
-
AIRE_000124
-
Capalbo 2009 Horm Res,
PubMed: Mazza 2011
,
PubMed: Stolarski 2006
-
-
Unknown
-
-
-
-
-
Roberto Perniola
?/.
1
-
c.132+5del
r.spl?
p.?
-
VUS
g.45706026del
-
AIRE(NM_000383.4):c.132+5del
-
AIRE_000588
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
-?/.
1
-
c.132+16del
r.(=)
p.(=)
-
likely benign
g.45706037del
-
AIRE(NM_000383.2):c.132+16delG
-
AIRE_000579
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
?/.
1
-
c.169C>G
r.(?)
p.(Gln57Glu)
-
VUS
g.45706476C>G
-
AIRE(NM_000383.2):c.169C>G (p.Q57E)
-
AIRE_000558
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/?
1
2
c.191_226del
r.(?)
p.(Ser64_Asp76delinsTyr)
-
pathogenic
g.45706498_45706533del
g.44286615_44286650del
-
-
AIRE_000016
TCC Â… GAC > TAC
PubMed: Heino 1999
-
-
Unknown
-
-
-
-
-
Roberto Perniola
+/?
2
2
c.205_208dup
r.(?)
p.(Asp70Alafs*148)
-
pathogenic
g.45706512_45706515dup
g.44286629_44286632dup
-
-
AIRE_000017
GAC>GCA
PubMed: Faiyaz-Ul-Haque 2009
,
PubMed: Heino 1999
-
-
Unknown
-
-
-
-
-
Roberto Perniola
-?/., ?/?
2
2
c.226G>A
r.(?)
p.(Asp76Asn)
-
likely benign, VUS
g.45706533G>A
g.44286650G>A
-, AIRE(NM_000383.2):c.226G>A (p.D76N)
-
AIRE_000018
GAC>AAC, VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record, Unknown
-
-
-
-
-
VKGL-NL_Rotterdam
,
Roberto Perniola
+/?
1
2
c.230T>C
r.(?)
p.(Phe77Ser)
-
pathogenic
g.45706537T>C
g.44286654T>C
-
-
AIRE_000019
TTC>TCC
PubMed: Meloni 2005
-
-
Unknown
-
-
-
-
-
Roberto Perniola
+/?
1
2
c.232T>A
r.(?)
p.(Trp78Arg)
-
pathogenic
g.45706539T>A
g.44286656T>A
-
-
AIRE_000021
-
-
-
-
Unknown
-
-
-
-
-
Roberto Perniola
+/?
7
2
c.232T>C
r.(?)
p.(Trp78Arg)
-
pathogenic
g.45706539T>C
g.44286656T>C
-
-
AIRE_000020
TGG>CGG
Capalbo 2009 Horm Res,
PubMed: Cervato 2009
,
PubMed: Cihakova 2001
,
PubMed: Mazza 2011
,
3 more items
-
-
Unknown
-
-
-
-
-
Roberto Perniola
+/?
1
2
c.238G>T
r.(?)
p.(Val80Leu)
-
pathogenic
g.45706545G>T
g.44286662G>T
-
-
AIRE_000022
GTG>TTG
PubMed: Bjorses 2000
-
-
Unknown
-
-
-
-
-
Roberto Perniola
+/?
1
2
c.239T>G
r.(?)
p.(Val80Gly)
-
pathogenic
g.45706546T>G
g.44286663T>G
-
-
AIRE_000023
GTG>GGG
PubMed: Zaidi 2009
-
-
Unknown
-
-
-
-
-
Roberto Perniola
+/?
1
2
c.247A>G
r.(?)
p.(Lys83Glu)
-
pathogenic
g.45706554A>G
g.44286671A>G
-
-
AIRE_000024
AAG>GAG
PubMed: Nagamine 1997
,
OMIM:var0002
-
-
Unknown
-
-
-
-
-
Roberto Perniola
+/?
2
2
c.254A>G
r.(?)
p.(Tyr85Cys)
-
pathogenic
g.45706561A>G
g.44286678A>G
-
-
AIRE_000025
TAC>TGC
PubMed: Bjorses 2000
,
PubMed: Halonen 2002
-
-
Unknown
-
-
-
-
-
Roberto Perniola
+/.
1
-
c.260del
r.(?)
p.(Leu87Argfs*60)
-
pathogenic
g.45706567del
-
AIRE(NM_000383.2):c.260delT (p.L87Rfs*60)
-
AIRE_000567
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/?
2
2
c.260T>C
r.(?)
p.(Leu87Pro)
-
pathogenic
g.45706567T>C
g.44286684T>C
-
-
AIRE_000026
CTG>CCG
Montin 2008 Clin Exp Immunol,
PubMed: Mazza 2011
-
-
Unknown
-
-
-
-
-
Roberto Perniola
+/?
1
2
c.262G>T
r.(?)
p.(Glu88*)
-
pathogenic
g.45706569G>T
g.44286686G>T
-
-
AIRE_000027
GAG>TAG
Cepedano Dans 2002 An Esp Pediatr
-
-
Unknown
-
-
-
-
-
Roberto Perniola
+/?
1
2
c.269A>G
r.(?)
p.(Tyr90Cys)
-
pathogenic
g.45706576A>G
g.44286693A>G
-
-
AIRE_000028
TAT>TGT
PubMed: Pearce 1998
-
-
Unknown
-
-
-
-
-
Roberto Perniola
+/?
1
2
c.274C>T
r.(?)
p.(Arg92Trp)
-
pathogenic
g.45706581C>T
g.44286698C>T
-
-
AIRE_000029
CGG>TGG
PubMed: F Magitta 2008
-
-
Unknown
-
-
-
-
-
Roberto Perniola
+/?
1
2
c.278T>G
r.(?)
p.(Leu93Arg)
-
pathogenic
g.45706585T>G
g.44286702T>G
-
-
AIRE_000030
CTG>CGG
Ward 1999 JCEM
-
-
Unknown
-
-
-
-
-
Roberto Perniola
+/?
2
2
c.290T>C
r.(?)
p.(Leu97Pro)
-
pathogenic
g.45706597T>C
g.44286714T>C
-
-
AIRE_000031
CTG>CCG
PubMed: Dominguez 2006
,
PubMed: Wolff 2007
-
-
Unknown
-
-
-
-
-
Roberto Perniola
+/?
1
2
c.306del
r.(?)
p.(Asp103Metfs*44)
-
pathogenic
g.45706613del
g.44286730del
-
-
AIRE_000032
AAA GAT > AAG ATG
Shin and Lee 2011 Horm Res Paediatr
-
-
Unknown
-
-
-
-
-
Roberto Perniola
-?/?
1
2i
c.307+61C>T
r.(=)
p.=
-
likely benign
g.45706675C>T
g.44286792C>T
-
-
AIRE_000219
-
Scott et al. [1998]
-
-
Unknown
-
-
-
-
-
Roberto Perniola
?/?
1
3
c.342G>T
r.(?)
p.(Lys114Asn)
-
VUS
g.45706895G>T
g.44287012G>T
-
-
AIRE_000033
AAG>AAT
-
-
-
Unknown
-
-
-
-
-
Roberto Perniola
?/., ?/?
2
3
c.347C>T
r.(?)
p.(Pro116Leu)
-
VUS
g.45706900C>T
g.44287017C>T
-, AIRE(NM_000383.3):c.347C>T (p.(Pro116Leu))
-
AIRE_000034
CCG>CTG, VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record, Unknown
-
-
-
-
-
VKGL-NL_Leiden
,
Roberto Perniola
-?/.
1
-
c.348G>A
r.(?)
p.(Pro116=)
-
likely benign
g.45706901G>A
g.44287018G>A
AIRE(NM_000383.2):c.348G>A (p.P116=)
-
AIRE_000529
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/?
1
3
c.352del
r.(?)
p.(Val118Serfs*29)
-
pathogenic
g.45706905del
g.44287022del
-
-
AIRE_000035
GTC>TCC
Jaimes Parada 2008 An Pediatr Barc
-
-
Unknown
-
-
-
-
-
Roberto Perniola
?/.
1
-
c.352G>A
r.(?)
p.(Val118Ile)
-
VUS
g.45706905G>A
-
AIRE(NM_000383.3):c.352G>A (p.(Val118Ile))
-
AIRE_000585
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
-?/.
2
-
c.354C>G
r.(?)
p.(Val118=)
-
likely benign
g.45706907C>G
g.44287024C>G
AIRE(NM_000383.2):c.354C>G (p.V118=)
-
AIRE_000530
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_Utrecht
?/.
1
-
c.371C>T
r.(?)
p.(Pro124Leu)
-
VUS
g.45706924C>T
g.44287041C>T
-
-
AIRE_000550
conflicting interpretations of pathogenicity; 1 heterozygous, no homozygous;
Clinindb (India)
PubMed: Narang 2020
,
Journal: Narang 2020
-
rs193922417
Germline
-
1/2795 individuals
-
-
-
Mohammed Faruq
?/.
1
-
c.386C>A
r.(?)
p.(Pro129His)
-
VUS
g.45706939C>A
-
AIRE(NM_000383.4):c.386C>A (p.(Pro129His))
-
AIRE_000581
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
-?/?
1
3
c.387C>T
r.(?)
p.=
-
likely benign
g.45706940C>T
g.44287057C>T
-
-
AIRE_000141
-
Tazi-Ahnini et al. [2002]
-
-
Unknown
-
-
-
-
-
Roberto Perniola
+/?
1
3
c.402del
r.(?)
p.(Ser135Glnfs*12)
-
pathogenic
g.45706955del
g.44287072del
-
-
AIRE_000036
GCC TCA > GCT CAG
PubMed: Wolff 2007
-
-
Unknown
-
-
-
-
-
Roberto Perniola
?/?
1
3
c.415C>G
r.(?)
p.(Arg139Gly)
-
VUS
g.45706968C>G
g.44287085C>G
-
-
AIRE_000037
CGA>GGA
-
-
-
Unknown
-
-
-
-
-
Roberto Perniola
+/., +/?
8
3
c.415C>T
r.(?)
p.(Arg139*)
-
pathogenic
g.45706968C>T
g.44287085C>T
-, AIRE(NM_000383.4):c.415C>T (p.R139*)
-
AIRE_000038
CGA>TGA, VKGL data sharing initiative Nederland
Valenzise 2010 Horm Res Paediatr,
PubMed: Cervato 2009
,
PubMed: Cihakova 2001
,
PubMed: Mazza 2011
,
3 more items
-
-
CLASSIFICATION record, Unknown
-
-
-
-
-
VKGL-NL_Groningen
,
Roberto Perniola
?/?
1
3
c.428C>T
r.(?)
p.(Pro143Leu)
-
VUS
g.45706981C>T
g.44287098C>T
-
-
AIRE_000039
CCA>CTA
-
-
-
Unknown
-
-
-
-
-
Roberto Perniola
-?/.
1
-
c.454G>A
r.(?)
p.(Ala152Thr)
-
likely benign
g.45707007G>A
g.44287124G>A
AIRE(NM_000383.2):c.454G>A (p.A152T)
-
AIRE_000551
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-?/.
1
-
c.457A>G
r.(?)
p.(Ser153Gly)
-
likely benign
g.45707010A>G
-
AIRE(NM_000383.2):c.457A>G (p.S153G)
-
AIRE_000559
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+?/?
1
3
c.462A>T
r.spl?
p.(?)
-
likely pathogenic (recessive)
g.45707015A>T
g.44287132A>T
-
-
AIRE_000142
-
PubMed: Podkrajsek 2008
-
-
De novo
-
-
-
-
-
Roberto Perniola
+/?, +?/.
2
3
c.463G>A
r.(?)
p.(Gly155Ser)
-
likely pathogenic, pathogenic
g.45707016G>A
g.44287133G>A
-
-
AIRE_000040
1 heterozygous, no homozygous;
Clinindb (India)
, GGC>AGC
PubMed: Narang 2020
,
Journal: Narang 2020
,
PubMed: von Schnurbein 2008
-
rs193922418
Germline, Unknown
-
1/2793 individuals
-
-
-
Roberto Perniola
,
Mohammed Faruq
+/?
2
3i
c.463+2T>C
r.(spl?)
p.?
-
pathogenic
g.45707018T>C
g.44287135T>C
-
-
AIRE_000126
-
PubMed: Dominguez 2006
,
PubMed: Wang 1998
-
-
Unknown
-
-
-
-
-
Roberto Perniola
-?/.
1
-
c.464-16del
r.(=)
p.(=)
-
likely benign
g.45707384del
-
AIRE(NM_000383.2):c.464-16delC
-
AIRE_000562
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
?/.
1
-
c.479C>T
r.(?)
p.(Ala160Val)
-
VUS
g.45707415C>T
g.44287532C>T
AIRE(NM_000383.2):c.479C>T (p.A160V)
-
AIRE_000552
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
?/., ?/?
2
4
c.485C>T
r.(?)
p.(Pro162Leu)
-
VUS
g.45707421C>T
g.44287538C>T
-, AIRE(NM_000383.2):c.485C>T (p.P162L)
-
AIRE_000041
CCC>CTC, VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record, Unknown
-
-
-
-
-
VKGL-NL_Rotterdam
,
Roberto Perniola
+/.
1
-
c.489del
r.(?)
p.(Lys164ArgfsTer214)
-
pathogenic
g.45707425del
g.44287542del
AIRE(NM_000383.4):c.489delC (p.K164Rfs*214)
-
AIRE_000544
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
?/?
1
4
c.497C>T
r.(?)
p.(Pro166Leu)
-
VUS
g.45707433C>T
g.44287550C>T
-
-
AIRE_000042
CCG>CTG
-
-
-
Unknown
-
-
-
-
-
Roberto Perniola
+/?
1
4
c.508_509ins13
r.?
p.(Ala170...fs*51)
-
pathogenic
g.45707444_45707445ins13
-
-
-
AIRE_000043
-
PubMed: Bjorses 2000
-
-
Unknown
-
-
-
-
-
Roberto Perniola
+/?
2
4
c.517C>T
r.(?)
p.(Gln173*)
-
pathogenic
g.45707453C>T
g.44287570C>T
-
-
AIRE_000044
CAG>TAG
PubMed: Bjorses 2000
,
PubMed: Heino 1999
-
-
Unknown
-
-
-
-
-
Roberto Perniola
-?/., ?/?
2
4
c.521G>A
r.(?)
p.(Arg174His)
-
likely benign, VUS
g.45707457G>A
g.44287574G>A
-, AIRE(NM_000383.2):c.521G>A (p.R174H)
-
AIRE_000045
CGC>CAC, VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record, Unknown
-
-
-
-
-
VKGL-NL_Utrecht
,
Roberto Perniola
-?/.
2
-
c.531C>T
r.(?)
p.(Leu177=)
-
likely benign
g.45707467C>T
g.44287584C>T
AIRE(NM_000383.2):c.531C>T (p.L177=)
-
AIRE_000503
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_Utrecht
-?/.
1
-
c.538+7G>A
r.(=)
p.(=)
-
likely benign
g.45707481G>A
-
AIRE(NM_000383.4):c.538+7G>A
-
AIRE_000589
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
-?/?
1
4i
c.538+42A>G
r.(=)
p.=
-
likely benign
g.45707516A>G
g.44287633A>G
-
-
AIRE_000228
-
Tazi-Ahnini et al. [2002]
-
-
Unknown
-
-
-
-
-
Roberto Perniola
-?/?
1
4i
c.538+49A>G
r.(=)
p.=
-
likely benign
g.45707523A>G
g.44287640A>G
-
-
AIRE_000230
-
Tazi-Ahnini et al. [2002]
-
-
Unknown
-
-
-
-
-
Roberto Perniola
-?/?
1
4i
c.539-46A>G
r.(=)
p.=
-
likely benign
g.45708182A>G
g.44288299A>G
-
-
AIRE_000248
-
Tazi-Ahnini et al. [2002]
-
-
Unknown
-
-
-
-
-
Roberto Perniola
+/?
1
5
c.540del
r.(?)
p.(Ile181Phefs*197)
-
pathogenic
g.45708229del
g.44288346del
-
-
AIRE_000046
GGG ATT > GGA TTC
PubMed: Podkrajsek 2005
-
-
Unknown
-
-
-
-
-
Roberto Perniola
-/., -?/., ?/?
4
5
c.548C>A
r.(?)
p.(Thr183Asn)
-
benign, likely benign, VUS
g.45708237C>A
g.44288354C>A
-, AIRE(NM_000383.2):c.548C>A (p.T183N), AIRE(NM_000383.3):c.548C>A (p.(Thr183Asn))
-
AIRE_000047
ACC>AAC, VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record, Unknown
-
-
-
-
-
VKGL-NL_Leiden
,
VKGL-NL_Rotterdam
,
VKGL-NL_Utrecht
,
Roberto Perniola
?/?
1
5
c.560C>T
r.(?)
p.(Ser187Leu)
-
VUS
g.45708249C>T
g.44288366C>T
-
-
AIRE_000048
TCA>TTA
-
-
-
Unknown
-
-
-
-
-
Roberto Perniola
-/.
4
-
c.588C>T
r.(=), r.(?)
p.(=), p.(Ser196=)
-
benign, likely benign
g.45708277C>T
g.44288394C>T
AIRE(NM_000383.2):c.588C>T (p.S196=), AIRE(NM_000383.4):c.588C>T (p.S196=)
-
AIRE_000505
VKGL data sharing initiative Nederland
PubMed: Podkrajsek 2008
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Johan den Dunnen
,
VKGL-NL_Groningen
,
VKGL-NL_Utrecht
,
VKGL-NL_Nijmegen
-?/., ?/?
2
5
c.589G>A
r.(?)
p.(Gly197Arg)
-
likely benign, VUS
g.45708278G>A
g.44288395G>A
-, AIRE(NM_000383.3):c.589G>A (p.(Gly197Arg))
-
AIRE_000049
GGG>AGG, VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record, Unknown
-
-
-
-
-
VKGL-NL_Leiden
,
Roberto Perniola
-?/., ?/?
2
5
c.595G>A
r.(?)
p.(Val199Ile)
-
likely benign, VUS
g.45708284G>A
g.44288401G>A
-, AIRE(NM_000383.2):c.595G>A (p.V199I)
-
AIRE_000050
GTC>ATC, VKGL data sharing initiative Nederland
Nejentsev 2009 Science
-
-
CLASSIFICATION record, Unknown
-
-
-
-
-
VKGL-NL_Utrecht
,
Roberto Perniola
?/?
1
5
c.599C>T
r.(?)
p.(Pro200Leu)
-
VUS
g.45708288C>T
g.44288405C>T
-
-
AIRE_000051
CCG>CTG
-
-
-
Unknown
-
-
-
-
-
Roberto Perniola
-?/.
1
-
c.600G>A
r.(?)
p.(Pro200=)
-
likely benign
g.45708289G>A
g.44288406G>A
AIRE(NM_000383.2):c.600G>A (p.P200=)
-
AIRE_000533
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
+/?
7
5
c.607C>T
r.(?)
p.(Arg203*)
-
pathogenic
g.45708296C>T
g.44288413C>T
-
-
AIRE_000052
CGA>TGA
Valenzise 2010 Horm Res Paediatr,
PubMed: Bjorses 2000
,
PubMed: Cervato 2009
,
PubMed: Cervato 2010
,
3 more items
-
-
Unknown
-
-
-
-
-
Roberto Perniola
-?/?
1
5
c.615C>T
r.(?)
p.=
-
likely benign
g.45708304C>T
g.44288421C>T
-
-
AIRE_000146
-
-
-
-
Unknown
-
-
-
-
-
Roberto Perniola
-?/.
1
-
c.616G>C
r.(?)
p.(Val206Leu)
-
likely benign
g.45708305G>C
-
AIRE(NM_000383.3):c.616G>C (p.(Val206Leu))
-
AIRE_000586
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
-?/.
2
-
c.652+14C>T
r.(=)
p.(=)
-
likely benign
g.45708355C>T
g.44288472C>T
AIRE(NM_000383.2):c.652+14C>T
-
AIRE_000506
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
,
VKGL-NL_Nijmegen
-?/.
1
-
c.652+15G>A
r.(=)
p.(=)
-
likely benign
g.45708356G>A
-
AIRE(NM_000383.2):c.652+15G>A
-
AIRE_000572
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
-?/?
1
5i
c.652+44G>A
r.(=)
p.=
-
likely benign
g.45708385G>A
g.44288502G>A
-
-
AIRE_000252
-
Tazi-Ahnini et al. [2002]
-
-
Unknown
-
-
-
-
-
Roberto Perniola
+/?
1
5i
c.653-6_653-4del
r.(spl?)
p.?
-
pathogenic
g.45709534_45709536del
g.44289651_44289653del
c.653-7_653-5del
-
AIRE_000127
-
PubMed: Podkrajsek 2005
-
-
Unknown
-
-
-
-
-
Roberto Perniola
-?/.
1
-
c.654C>T
r.(?)
p.(Gly218=)
-
likely benign
g.45709541C>T
g.44289658C>T
AIRE(NM_000383.2):c.654C>T (p.G218=)
-
AIRE_000507
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-?/., ?/?
2
6
c.655G>A
r.(?)
p.(Gly219Ser)
-
likely benign, VUS
g.45709542G>A
g.44289659G>A
-, AIRE(NM_000383.2):c.655G>A (p.G219S)
-
AIRE_000053
GGC>AGC, VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record, Unknown
-
-
-
-
-
VKGL-NL_Utrecht
,
Roberto Perniola
-/.
3
-
c.681C>T
r.(?)
p.(Gly227=)
-
benign
g.45709568C>T
g.44289685C>T
AIRE(NM_000383.2):c.681C>T (p.G227=), AIRE(NM_000383.4):c.681C>T (p.G227=)
-
AIRE_000508
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
,
VKGL-NL_Utrecht
,
VKGL-NL_Nijmegen
+/., +/?
2
6
c.682G>T
r.(?)
p.(Gly228Trp)
-
pathogenic
g.45709569G>T
g.44289686G>T
-
-
AIRE_000054
1 heterozygous, no homozygous;
Clinindb (India)
, GGG>TGG
PubMed: Cetani 2001
,
PubMed: Narang 2020
,
Journal: Narang 2020
-
rs121434257
Germline, Unknown
-
1/2795 individuals
-
-
-
Roberto Perniola
,
Mohammed Faruq
-?/.
2
-
c.705G>A
r.(?)
p.(Lys235=)
-
likely benign
g.45709592G>A
g.44289709G>A
AIRE(NM_000383.2):c.705G>A (p.K235=)
-
AIRE_000509
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_Utrecht
?/?
1
6
c.718G>A
r.(?)
p.(Gly240Ser)
-
VUS
g.45709605G>A
g.44289722G>A
-
-
AIRE_000055
GGC>AGC
-
-
-
Unknown
-
-
-
-
-
Roberto Perniola
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