Global Variome shared LOVD
COL4A3 (collagen, type IV, alpha 3 (Goodpasture an...))
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Curator:
Judy Savige
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Unique variants in the COL4A3 gene
The variants shown are described using the NM_000091.4 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%)
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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'
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645 entries on 7 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.-26G>T
r.(?)
p.(=)
-
VUS
g.228029417G>T
g.227164701G>T
-
-
COL4A3_000456
-
-
-
-
Germline
-
-
-
-
-
Judy Savige
-?/.
2
1
c.-10C>T
r.(?)
p.(=)
-
likely benign
g.228029433C>T
g.227164717C>T
1-10C>T 3'UTR
-
COL4A3_000001
VKGL data sharing initiative Nederland
PubMed: Tazon Vega 2003
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Judy Savige
,
VKGL-NL_Nijmegen
+/.
2
_1_1i
c.(?_-1)_(87+1_88-1)del
r.0?
p.0?
-
pathogenic, VUS
g.(227985865_228004876)_(228029530_228102683)del, g.(?_228029442)_(228029530_228102683)del
-
del ex1, del ex1 COL4A3, ex1-4 COL4A4
-
COL4A3_000446, COL4A4_000408
-
PubMed: Mencarelli 2015
,
Journal: Mencarelli 2015
,
PubMed: Moriniere 2014
,
Journal: Moriniere 2014
-
-
Germline
-
-
-
-
-
Judy Savige
+/.
1
14
c.?
r.805_828del
p.Glu269_Ser276del
-
pathogenic
g.?
-
805_828delGAGCCTGGACCTCCTGGACCCTCA
-
COL4A3_000000
-
PubMed: Zhang 2011
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/+?
1
1
c.1A>T
r.(?)
p.0?
-
pathogenic
g.228029443A>T
g.227164727A>T
M1L
-
COL4A3_000002
nonsense; Compound heterozygous;
PubMed: Longo 2002
-
-
Germline
-
-
-
-
-
Judy Savige
+/., ./.
2
1
c.2T>A
r.(?)
p.(Met1?), p.0?
ACMG
pathogenic, VUS
g.228029444T>A
g.227164728T>A
Met1Lys, p.Met1Lys
-
COL4A3_000457
-
PubMed: Weber 2016
,
Journal: Weber 2016
-
-
Germline
yes
-
-
-
-
Judy Savige
,
Jasmina Comic
./.
2
1
c.2T>C
r.(?)
p.0?
-
VUS
g.228029444T>C
g.227164728T>C
Met1Thr, p.Met1Thr
-
COL4A3_000250
-
PubMed: Uzak 2013
-
-
Germline
-
-
-
-
-
Judy Savige
./.
1
1
c.2T>G
r.(?)
p.0?
-
VUS
g.228029444T>G
g.227164728T>G
p.Met1Arg
-
COL4A3_000458
-
PubMed: Moriniere 2014
,
Journal: Moriniere 2014
-
-
Germline
-
-
-
-
-
Judy Savige
./.
1
1
c.3G>A
r.(?)
p.0?
-
-
g.228029445G>A
g.227164729G>A
Met1Ile
-
COL4A3_000251
-
-
-
-
Germline
-
-
-
-
-
Judy Savige
?/.
1
-
c.17C>A
r.(?)
p.(Ala6Asp)
-
VUS
g.228029459C>A
-
COL4A3(NM_000091.5):c.17C>A (p.(Ala6Asp))
-
COL4A3_000783
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
./.
1
1
c.19C>A
r.(?)
p.(Pro7Thr)
-
-
g.228029461C>A
g.227164745C>A
-
-
COL4A3_000252
-
-
-
-
Germline
-
-
-
-
-
Judy Savige
./.
1
1
c.21C>A
r.(?)
p.(Pro7=)
-
-
g.228029463C>A
g.227164747C>A
-
-
COL4A3_000178
-
1000 genome; NCBI
-
-
Germline
-
-
-
-
-
Judy Savige
?/.
1
1
c.26C>T
r.(?)
p.(Pro9Leu)
-
VUS
g.228029468C>T
g.227164752C>T
-
-
COL4A3_000459
-
-
-
-
Germline
-
-
-
-
-
Judy Savige
-?/.
1
-
c.33G>T
r.(?)
p.(Val11=)
-
likely benign
g.228029475G>T
g.227164759G>T
-
-
COL4A3_000622
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/+?, +/., ./., ?/.
11
1
c.40_63del
r.(?)
p.(Leu14_Leu21del)
-
pathogenic, pathogenic (recessive), VUS
g.228029482_228029505del
g.227164766_227164789del
30_53del, 40del24, 40_63delCTGCCGCTCCTGCTGGTGCTCCTG, 40_63delCTGCCGCTCCTGCTGGTGCTCCTG (del LPLLLVLL)
-
COL4A3_000003
Compound heterozygous, VKGL data sharing initiative Nederland
PubMed: Chatterjee 2013
,
Journal: Chatterjee 2013
,
PubMed: Longo 2002
,
PubMed: Tazon Vega 2003
,
1 more item
-
rs876657397
CLASSIFICATION record, Germline
yes
-
-
-
-
Judy Savige
,
VKGL-NL_Nijmegen
,
Pavlina Plevova
./.
1
1
c.40_66del
r.(?)
p.(Leu14_Ala22del)
-
-
g.228029482_228029508del
g.227164766_227164792del
-
-
COL4A3_000254
-
-
-
-
Germline
-
-
-
-
-
Judy Savige
-?/., ?/.
2
1
c.43_54del
r.(?)
p.(Pro15_Leu18del)
-
likely benign, VUS
g.228029485_228029496del
g.227164769_227164780del
COL4A3(NM_000091.5):c.43_54del (p.(Pro15_Leu18del))
-
COL4A3_000460
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Judy Savige
,
VKGL-NL_Leiden
-?/.
1
-
c.45G>A
r.(?)
p.(=)
-
likely benign
g.228029487G>A
-
COL4A3(NM_000091.4):c.45G>A (p.P15=)
-
COL4A3_000781
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
+?/., -?/.
2
1
c.71C>G
r.(?)
p.(Ala24Gly)
-
likely benign, likely pathogenic
g.228029513C>G
g.227164797C>G
COL4A3(NM_000091.4):c.71C>G (p.A24G)
-
COL4A3_000179
VKGL data sharing initiative Nederland
1000 genome; NCBI
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Judy Savige
,
VKGL-NL_Utrecht
+?/., -?/.
2
1
c.73C>T
r.(?)
p.(Pro25Ser)
-
likely benign, likely pathogenic
g.228029515C>T
g.227164799C>T
COL4A3(NM_000091.4):c.73C>T (p.P25S)
-
COL4A3_000180
VKGL data sharing initiative Nederland
1000 genome; NCBI
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Judy Savige
,
VKGL-NL_Utrecht
-/.
2
-
c.88-4C>T
r.spl?
p.?
-
benign
g.228102680C>T
g.227237964C>T
COL4A3(NM_000091.4):c.88-4C>T
-
COL4A3_000402
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
,
VKGL-NL_Nijmegen
./.
1
1i_50i
c.88-?_4755+?dup
r.?
p.?
-
VUS
g.228102684_228174034dup
-
88-?_4755+?dup
-
COL4A3_000461
1 more item
PubMed: Moriniere 2014
,
Journal: Moriniere 2014
-
-
Germline
-
-
-
-
-
Judy Savige
./.
1
2
c.96dup
r.(?)
p.(Cys33Leufs*2)
-
-
g.228102692dup
g.227237976dup
c.95_96insC
-
COL4A3_000255
-
-
-
-
Germline
-
-
-
-
-
Judy Savige
./.
3
2
c.127G>A
r.(?)
p.(Gly43Arg)
-
-
g.228102723G>A
g.227238007G>A
-
-
COL4A3_000004
Missense, Polymorphism
PubMed: Badenas 2002
,
PubMed: Slajpah 2007
,
PubMed: Wang 2004
-
-
Germline
-
-
-
-
-
Judy Savige
-/-?, -/., ./.
7
2
c.127G>C
r.(?)
p.(Gly43Arg)
-
benign
g.228102723G>C
g.227238007G>C
COL4A3(NM_000091.4):c.127G>C (p.G43R)
-
COL4A3_000005
Polymorphism, VKGL data sharing initiative Nederland
PubMed: Heidet 2001
,
PubMed: Kovacs 2016
,
Journal: Kovacs 2016
,
PubMed: Longo 2002
,
1 more item
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Judy Savige
,
VKGL-NL_Utrecht
,
VKGL-NL_Nijmegen
-?/., ?/.
2
-
c.136G>A
r.(?)
p.(Gly46Arg)
-
likely benign, VUS
g.228102732G>A
g.227238016G>A
COL4A3(NM_000091.4):c.136G>A (p.G46R)
-
COL4A3_000689
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
,
VKGL-NL_Nijmegen
-/., ./.
6
2i
c.144+12C>A
r.(=), r.(?)
p.(=)
-
benign
g.228102752C>A
g.227238036C>A
COL4A3(NM_000091.5):c.144+12C>A, MFF-DT(NR_102371.1):n.1681+50G>T
-
COL4A3_000006
VKGL data sharing initiative Nederland
PubMed: Badenas 2002
,
PubMed: Kovacs 2016
,
Journal: Kovacs 2016
,
PubMed: Tazon Vega 2003
,
1 more item
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Judy Savige
,
VKGL-NL_Groningen
,
VKGL-NL_Nijmegen
-?/.
1
-
c.144+18T>G
r.(=)
p.(=)
-
likely benign
g.228102758T>G
-
COL4A3(NM_000091.4):c.144+18T>G
-
COL4A3_000723
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
-?/.
1
-
c.144+39A>T
r.(=)
p.(=)
-
likely benign
g.228102779A>T
g.227238063A>T
-
-
COL4A3_000623
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
-/.
2
-
c.145-99C>T
r.(=), r.(?)
p.(=), p.?
-
benign
g.228104760C>T
g.227240044C>T
45-99C>T
-
COL4A3_000700
variant in COL4A4:c.1598G>A homozygote, VKGL data sharing initiative Nederland
PubMed: Plevova 2023
-
rs7579991
CLASSIFICATION record, Germline
-
-
-
-
-
Johan den Dunnen
,
VKGL-NL_Nijmegen
./.
1
3
c.145G>C
r.(?)
p.(Gly49Arg)
-
VUS
g.228104859G>C
g.227240143G>C
-
-
COL4A3_000462
-
PubMed: Moriniere 2014
,
Journal: Moriniere 2014
-
-
Germline
-
-
-
-
-
Judy Savige
./.
1
2i
c.162-2A>G
exon 3 (90 bp) skip
p.?
-
VUS
g.228104874A>G
-
-
-
COL4A3_000463
1 more item
PubMed: Oka 2014
,
Journal: Oka 2014
-
-
Germline
-
-
-
-
-
Judy Savige
+/., ./.
4
3
c.162dup
r.(?)
p.(Gly55Trpfs*15)
-
pathogenic
g.228104876dup
g.227240160dup
162_163insT
-
COL4A3_000246
-
PubMed: Storey 2013
-
-
Germline
-
-
-
-
-
Helen Storey
,
Judy Savige
+/., ?/.
2
3
c.172G>A
r.(?)
p.(Gly58Ser)
-
pathogenic, VUS
g.228104886G>A
g.227240170G>A
COL4A3(NM_000091.4):c.172G>A (p.(Gly58Ser))
-
COL4A3_000181
VKGL data sharing initiative Nederland
1000 genome; NCBI
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Judy Savige
,
VKGL-NL_Leiden
./.
1
3
c.172G>C
r.(?)
p.(Gly58Arg)
-
VUS
g.228104886G>C
g.227240170G>C
-
-
COL4A3_000464
-
PubMed: Moriniere 2014
,
Journal: Moriniere 2014
-
-
Germline
-
-
-
-
-
Judy Savige
+/.
1
-
c.184dup
r.(?)
p.(Gln62ProfsTer8)
-
pathogenic
g.228104898dup
g.227240182dup
-
-
COL4A3_000624
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
./.
1
3
c.222G>A
r.(?)
p.(Pro74=)
-
-
g.228104936G>A
g.227240220G>A
-
-
COL4A3_000182
-
1000 genome; NCBI
-
-
Germline
-
-
-
-
-
Judy Savige
-/., -?/., ./.
5
3
c.222G>T
r.(?)
p.(=), p.(Pro74=), p.(Pro74Pro)
-
benign, likely benign
g.228104936G>T
g.227240220G>T
COL4A3(NM_000091.4):c.222G>T (p.P74=)
-
COL4A3_000007
VKGL data sharing initiative Nederland
PubMed: Tazon Vega 2003
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Judy Savige
,
VKGL-NL_Rotterdam
,
VKGL-NL_Utrecht
,
VKGL-NL_Nijmegen
+?/.
1
3
c.233A>G
r.(?)
p.(Lys78Arg)
-
likely pathogenic
g.228104947A>G
g.227240231A>G
-
-
COL4A3_000183
-
1000 genome; NCBI
-
-
Germline
-
-
-
-
-
Judy Savige
+?/.
1
-
c.235-1G>A
r.spl?
p.?
-
likely pathogenic
g.228109035G>A
-
COL4A3(NM_000091.4):c.235-1G>A
-
COL4A3_000763
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
./.
1
4
c.261G>A
r.(?)
p.(=)
-
-
g.228109062G>A
g.227244346G>A
-
-
COL4A3_000259
-
-
-
-
Germline
-
-
-
-
-
Judy Savige
+/., ./.
2
4
c.272G>T
r.(?)
p.(Gly91Val)
ACMG
likely pathogenic, VUS
g.228109073G>T
g.227244357G>T
-
-
COL4A3_000465
-
PubMed: Weber 2016
,
Journal: Weber 2016
-
-
Germline
yes
-
-
-
-
Judy Savige
,
Jasmina Comic
-/.
1
-
c.280-40_280-39insG
r.(?)
p.?
-
benign
g.228109627_228109628insG
g.227244911_227244912insG
280-39_40insG
-
COL4A3_000742
variant in COL4A4:c.1598G>A homozygote
PubMed: Plevova 2023
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+?/.
1
4i_8i
c.(279+1_280-1)_(468+1_469-1)dup
r.?
p.?
-
VUS
g.(228109081_228109666)_(228112301_228113158)dup
-
ex5-8 dup
-
COL4A3_000450
-
-
-
-
Germline
-
-
-
-
-
Judy Savige
-?/.
1
-
c.324+20C>T
r.(=)
p.(=)
-
likely benign
g.228109731C>T
-
COL4A3(NM_000091.4):c.324+20C>T
-
COL4A3_000752
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
-/., -?/.
3
5i
c.324+73C>T
r.(=), r.(?)
p.(=), p.?
-
benign, likely benign
g.228109784C>T
g.227245068C>T
-
-
COL4A3_000008
Intronic, variant in COL4A4:c.1598G>A homozygote, VKGL data sharing initiative Nederland
PubMed: Plevova 2023
,
PubMed: Voskarides 2007
-
rs6750210
CLASSIFICATION record, Germline
-
-
-
-
-
Johan den Dunnen
,
Judy Savige
,
VKGL-NL_Nijmegen
-/.
2
-
c.325-27C>T
r.(=)
p.(=)
-
benign
g.228110643C>T
g.227245927C>T
COL4A3(NM_000091.4):c.325-27C>T
-
COL4A3_000403
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
,
VKGL-NL_Nijmegen
?/.
1
6
c.329C>T
r.(?)
p.(Thr110Ile)
-
VUS
g.228110674C>T
g.227245958C>T
-
-
COL4A3_000466
-
-
-
-
Germline
-
-
-
-
-
Judy Savige
-?/.
1
-
c.341C>T
r.(?)
p.(Thr114Ile)
-
likely benign
g.228110686C>T
g.227245970C>T
-
-
COL4A3_000625
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+?/.
1
-
c.344G>C
r.(?)
p.(Gly115Ala)
-
likely pathogenic
g.228110689G>C
g.227245973G>C
-
-
COL4A3_000626
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
./.
2
6
c.345del
r.(?)
p.(Pro116Leufs*37), p.Pro116Leufs*37
-
VUS
g.228110690del
g.227245974del
345delG, c.345delG
-
COL4A3_000467
-
PubMed: Moriniere 2014
,
Journal: Moriniere 2014
,
PubMed: Rosado 2015
,
Journal: Rosado 2015
-
-
Germline
-
-
-
-
-
Judy Savige
-?/., ./., ?/.
7
6
c.346C>A
r.(?)
p.(Pro116Thr)
-
likely benign, VUS
g.228110691C>A
g.227245975C>A
COL4A3(NM_000091.5):c.346C>A (p.P116T)
-
COL4A3_000009
3 heterozygous, no homozygous;
Clinindb (India)
, Polymorphism, VKGL data sharing initiative Nederland
PubMed: Moriniere 2014
,
Journal: Moriniere 2014
,
PubMed: Narang 2020
,
Journal: Narang 2020
,
1 more item
-
rs115324397
CLASSIFICATION record, Germline
-
3/2795 individuals
-
-
-
Judy Savige
,
VKGL-NL_Groningen
,
VKGL-NL_Nijmegen
,
Mohammed Faruq
+/+, +/+?
2
6
c.351C>A
r.(?)
p.(Tyr117*)
-
pathogenic
g.228110696C>A
g.227245980C>A
-
-
COL4A3_000010
Nonsense
PubMed: Nagel 2005
-
-
Germline
-
-
-
-
-
Judy Savige
?/.
1
-
c.389G>T
r.(?)
p.(Gly130Val)
-
VUS
g.228111402G>T
-
-
-
COL4A3_000694
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/+?
1
7
c.390dup
r.(?)
p.(Glu131*)
-
pathogenic
g.228111403dup
g.227246687dup
c.389_390insT
-
COL4A3_000011
Insertion; Heterozygous.
PubMed: Heidet 2001
-
-
Germline
-
-
-
-
-
Judy Savige
+/.
2
7
c.391G>T
r.(?)
p.(Glu131*)
-
pathogenic
g.228111404G>T
g.227246688G>T
-
-
COL4A3_000248
-
PubMed: Storey 2013
-
-
Germline
-
-
-
-
-
Helen Storey
,
Judy Savige
./.
1
7
c.393del
r.(?)
p.(Glu131Aspfs*22)
-
VUS
g.228111406del
g.227246690del
del393G
-
COL4A3_000468
-
PubMed: Malone 2014
,
Journal: Malone 2014
-
-
Germline
-
-
-
-
-
Judy Savige
./.
1
7
c.394C>T
r.(?)
p.(Gln132*)
-
-
g.228111407C>T
g.227246691C>T
-
-
COL4A3_000262
-
-
-
-
Germline
-
-
-
-
-
Judy Savige
-/., ./.
4
7
c.399G>A
r.(?)
p.(=), p.(Gly133=)
-
benign
g.228111412G>A
g.227246696G>A
COL4A3(NM_000091.4):c.399G>A (p.G133=)
-
COL4A3_000184
VKGL data sharing initiative Nederland
1000 genome; NCBI
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Judy Savige
,
VKGL-NL_Utrecht
,
VKGL-NL_Nijmegen
./.
1
7
c.402del
r.(?)
p.(Pro135Glnfs*18)
-
-
g.228111415del
g.227246699del
c.400delT
-
COL4A3_000264
-
-
-
-
Germline
-
-
-
-
-
Judy Savige
+?/.
1
-
c.407G>T
r.(?)
p.(Gly136Val)
-
likely pathogenic
g.228111420G>T
-
COL4A3(NM_000091.4):c.407G>T (p.G136V)
-
COL4A3_000739
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
+/.
4
7
c.415G>C
r.(?)
p.(Gly139Arg)
-
pathogenic, pathogenic (dominant), pathogenic (recessive)
g.228111428G>C
g.227246712G>C
-
-
COL4A3_000441
possible digenic inheritance
PubMed: Plevova 2023
-
-
Germline
?
-
-
-
-
Johan den Dunnen
,
Pavlina Plevova
./.
1
7
c.422C>T
r.(?)
p.(Pro141Leu)
-
-
g.228111435C>T
g.227246719C>T
-
-
COL4A3_000265
1 more item
-
-
-
Germline
-
-
-
-
-
Judy Savige
-/-?, -/., ./.
6
7
c.422T>C
r.(?)
p.(Leu141Pro)
-
benign, VUS
g.228111435T>C
g.227246719T>C
COL4A3(NM_000091.5):c.422T>C (p.L141P), MFF-DT(NR_102371.1):n.1593-8545A>G
-
COL4A3_000012
missense. Homozygous, Polymorphism, variant in COL4A4:c.1598G>A homozygote,
1 more item
PubMed: Hoefele 2010
,
PubMed: Kovacs 2016
,
Journal: Kovacs 2016
,
PubMed: Plevova 2023
,
1 more item
-
rs10178458
CLASSIFICATION record, Germline
-
-
-
-
-
Johan den Dunnen
,
Judy Savige
,
VKGL-NL_Groningen
,
VKGL-NL_Nijmegen
./.
1
7
c.432_440delinsGATTA
r.(?)
p.(Gly145Ilefs*7)
-
VUS
g.228111445_228111453delinsGATTA
g.227246729_227246737delinsGATTA
-
-
COL4A3_000469
-
PubMed: Moriniere 2014
,
Journal: Moriniere 2014
-
-
Germline
-
-
-
-
-
Judy Savige
+?/.
1
7
c.434G>A
r.(?)
p.(Gly145Glu)
-
likely pathogenic
g.228111447G>A
g.227246731G>A
-
-
COL4A3_000013
Missense
PubMed: Nagel 2005
-
-
Germline
-
-
-
-
-
Judy Savige
./.
1
7
c.441G>A
r.(?)
p.(=)
-
-
g.228111454G>A
g.227246738G>A
-
-
COL4A3_000266
-
-
-
-
Germline
-
-
-
-
-
Judy Savige
+/., +?/.
2
7i
c.441+2T>C
r.spl, r.spl?
p.?
-
likely pathogenic, pathogenic
g.228111456T>C
g.227246740T>C
COL4A3(NM_000091.5):c.441+2T>C
-
COL4A3_000470
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Judy Savige
,
VKGL-NL_Leiden
?/.
1
-
c.441+5G>T
r.spl?
p.?
-
VUS
g.228111459G>T
g.227246743G>T
-
-
COL4A3_000627
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
-/.
1
-
c.441+146G>T
r.(?)
p.?
-
benign
g.228111600G>T
g.227246884G>T
-
-
COL4A3_000743
variant in COL4A4:c.1598G>A homozygote
PubMed: Plevova 2023
-
rs10168566
Germline
-
-
-
-
-
Johan den Dunnen
-/.
1
-
c.441+150G>T
r.(?)
p.?
-
benign
g.228111604G>T
g.227246888G>T
-
-
COL4A3_000744
variant in COL4A4:c.1598G>A homozygote
PubMed: Plevova 2023
-
rs10168567
Germline
-
-
-
-
-
Johan den Dunnen
-/.
1
-
c.442-88A>G
r.(?)
p.?
-
benign
g.228112186A>G
g.227247470A>G
-
-
COL4A3_000745
variant in COL4A4:c.1598G>A homozygote
PubMed: Plevova 2023
-
rs4321358
Germline
-
-
-
-
-
Johan den Dunnen
?/.
1
-
c.442-28A>G
r.(=)
p.(=)
-
VUS
g.228112246A>G
g.227247530A>G
-
-
COL4A3_000628
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/., +?/., ./.
7
8
c.443G>T
r.(?)
p.(Gly148Val), p.Gly148Val
ACMG
likely pathogenic, pathogenic, pathogenic (dominant), VUS
g.228112275G>T
g.227247559G>T
COL4A3(NM_000091.5):c.443G>T (p.G148V)
-
COL4A3_000267
VKGL data sharing initiative Nederland
PubMed: Boucher 2020
,
PubMed: Malone 2014
,
Journal: Malone 2014
-
-
CLASSIFICATION record, Germline, Germline/De novo (untested)
?
-
-
-
-
Johan den Dunnen
,
Judy Savige
,
VKGL-NL_Groningen
,
VKGL-NL_Nijmegen
,
Jasmina Comic
-?/.
1
-
c.456G>T
r.(?)
p.(Leu152Phe)
-
likely benign
g.228112288G>T
-
COL4A3(NM_000091.4):c.456G>T (p.(Leu152Phe))
-
COL4A3_000769
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+?/.
1
8
c.461G>C
r.(?)
p.(Gly154Ala)
-
likely pathogenic
g.228112293G>C
g.227247577G>C
-
-
COL4A3_000225
-
PubMed: Storey 2013
-
-
Germline
-
-
-
-
-
Helen Storey
+/.
1
8_8i
c.462_468+1del
r.spl
p.?
-
pathogenic
g.228112294_228112301del
g.227247578_227247585del
462_468+1delACAAAAGG
-
COL4A3_000471
-
-
-
-
Germline
-
-
-
-
-
Judy Savige
-/.
1
-
c.468+139C>T
r.(?)
p.?
-
benign
g.228112439C>T
g.227247723C>T
-
-
COL4A3_000746
variant in COL4A4:c.1598G>A homozygote
PubMed: Plevova 2023
-
rs12612699
Germline
-
-
-
-
-
Johan den Dunnen
-/.
1
-
c.469-53G>T
r.(=)
p.(=)
-
benign
g.228113106G>T
g.227248390G>T
COL4A3(NM_000091.4):c.469-53G>T
-
COL4A3_000404
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
./.
2
9
c.473C>A
r.(?)
p.(Ala158Asp)
-
-
g.228113163C>A
g.227248447C>A
-
-
COL4A3_000014
Missense, Polymorphism
PubMed: Badenas 2002
,
PubMed: Wang 2004
-
-
Germline
-
-
-
-
-
Judy Savige
-/-?, -/., -?/., ./.
12
9
c.485A>G
r.(?)
p.(Glu162Gly)
-
benign, likely benign
g.228113175A>G
g.227248459A>G
COL4A3(NM_000091.5):c.485A>G (p.E162G), MFF-DT(NR_102371.1):n.1593-10285T>C
-
COL4A3_000015
Missense, missense. Homozygous, Polymorphism, variant in COL4A4:c.1598G>A homozygote,
1 more item
PubMed: Badenas 2002
,
PubMed: Heidet 2001
,
PubMed: Hoefele 2010
,
PubMed: Longo 2002
,
PubMed: Wang 2004
,
5 more items
-
rs6436669
CLASSIFICATION record, Germline
-
-
-
-
-
Johan den Dunnen
,
Judy Savige
,
VKGL-NL_Groningen
,
VKGL-NL_Nijmegen
./.
1
9
c.485G>A
r.(?)
p.(Gly162Glu)
-
-
g.228113175G>A
g.227248459G>A
-
-
COL4A3_000268
1 more item
-
-
-
Germline
-
-
-
-
-
Judy Savige
+?/.
1
9
c.494T>C
r.(?)
p.(Ile165Thr)
-
likely pathogenic
g.228113184T>C
g.227248468T>C
-
-
COL4A3_000185
-
1000 genome; NCBI
-
-
Germline
-
-
-
-
-
Judy Savige
./.
1
9
c.510A>T
r.(?)
p.(Lys170Asn)
-
-
g.228113200A>T
g.227248484A>T
-
-
COL4A3_000269
-
-
-
-
Germline
-
-
-
-
-
Judy Savige
-?/.
1
-
c.513C>T
r.(?)
p.(Gly171=)
-
likely benign
g.228113203C>T
-
COL4A3(NM_000091.4):c.513C>T (p.G171=)
-
COL4A3_000724
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
+?/.
1
9
c.520G>A
r.(?)
p.(Gly174Arg)
-
likely pathogenic
g.228113210G>A
g.227248494G>A
-
-
COL4A3_000472
-
-
-
-
Germline
-
-
-
-
-
Judy Savige
+/., +?/.
2
-
c.520G>T
r.(?)
p.(Gly174Trp)
-
likely pathogenic, pathogenic
g.228113210G>T
g.227248494G>T
COL4A3(NM_000091.4):c.520G>T (p.G174W)
-
COL4A3_000629
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
,
VKGL-NL_Nijmegen
./.
1
9
c.522dup
r.(?)
p.(Leu175Valfs*38)
-
-
g.228113212dup
g.227248496dup
c.520insG
-
COL4A3_000270
-
-
-
-
Germline
-
-
-
-
-
Judy Savige
+?/.
1
9
c.532G>A
r.(?)
p.(Ala178Thr)
-
likely pathogenic
g.228113222G>A
g.227248506G>A
-
-
COL4A3_000186
-
1000 genome; NCBI
-
-
Germline
-
-
-
-
-
Judy Savige
+?/., ./.
2
9
c.546G>T
r.(?)
p.(Gln182His)
-
likely pathogenic
g.228113236G>T
g.227248520G>T
-
-
COL4A3_000249
-
PubMed: Storey 2013
-
-
Germline
-
-
-
-
-
Helen Storey
,
Judy Savige
./.
1
9i
c.546+1G>T
r.spl
p.?
-
VUS
g.228113237G>T
g.227248521G>T
-
-
COL4A3_000473
-
PubMed: Moriniere 2014
,
Journal: Moriniere 2014
-
-
Germline
-
-
-
-
-
Judy Savige
?/.
1
9i
c.546+12C>T
r.(?)
p.(=)
-
VUS
g.228113248C>T
g.227248532C>T
-
-
COL4A3_000474
-
-
-
-
Germline
-
-
-
-
-
Judy Savige
-/., -?/.
4
9i
c.547-9A>C
r.(=), r.(?)
p.(=)
-
benign, likely benign
g.228115847A>C
g.227251131A>C
COL4A3(NM_000091.4):c.547-9A>C, COL4A3(NM_000091.5):c.547-9A>C
-
COL4A3_000405
VKGL data sharing initiative Nederland
PubMed: Kovacs 2016
,
Journal: Kovacs 2016
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Judy Savige
,
VKGL-NL_Leiden
,
VKGL-NL_Utrecht
,
VKGL-NL_Nijmegen
+?/.
1
-
c.547G>T
r.(?)
p.(Gly183Cys)
-
likely pathogenic
g.228115856G>T
g.227251140G>T
-
-
COL4A3_000630
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
-?/.
1
-
c.573T>C
r.(?)
p.(Pro191=)
-
likely benign
g.228115882T>C
-
COL4A3(NM_000091.4):c.573T>C (p.P191=)
-
COL4A3_000749
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
+?/.
1
-
c.593dup
r.(?)
p.(Pro199SerfsTer14)
-
likely pathogenic
g.228115902dup
g.227251186dup
-
-
COL4A3_000631
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
-?/.
1
-
c.599C>T
r.(?)
p.(Pro200Leu)
-
likely benign
g.228115908C>T
g.227251192C>T
COL4A3(NM_000091.4):c.599C>T (p.(Pro200Leu))
-
COL4A3_000605
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
./.
1
10
c.604T>C
r.(?)
p.(Phe202Leu)
-
-
g.228115913T>C
g.227251197T>C
-
-
COL4A3_000272
-
-
-
-
Germline
-
-
-
-
-
Judy Savige
-?/.
1
-
c.610-36T>A
r.(=)
p.(=)
-
likely benign
g.228116016T>A
g.227251300T>A
-
-
COL4A3_000632
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/.
2
10i_11i
c.610-?_645+?del
r.(?)
p.?
-
pathogenic
g.228116052_228116087del
-
ex 11del
-
COL4A3_000475
1 more item
-
-
-
Germline
-
-
-
-
-
Judy Savige
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