Global Variome shared LOVD
COL4A4 (collagen, type IV, alpha 4)
LOVD v.3.0 Build 30b [
Current LOVD status
]
Register as submitter
|
Log in
Curator:
Judy Savige
View all genes
View COL4A4 gene homepage
View graphs about the COL4A4 gene database
Create a new gene entry
View all transcripts
View all transcripts of gene COL4A4
Create a new transcript information entry
View all variants
View all variants affecting transcripts
View unique variants in gene COL4A4
View all variants in gene COL4A4
Full data view for gene COL4A4
Create a new data submission
View active genomic custom columns
Enable more genomic custom columns
View all individuals
View all individuals with variants in gene COL4A4
Create a new data submission
View active custom columns
Enable more custom columns
View all diseases
View all diseases associated with gene COL4A4
Create a new disease information entry
View available phenotype columns
View all screenings
View all screenings for gene COL4A4
Create a new data submission
View active custom columns
Enable more custom columns
Submit new data
Unique variants in the COL4A4 gene
The variants shown are described using the NM_000092.4 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%)
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.
658 entries on 7 pages. Showing entries 1 - 100.
10 per page
25 per page
50 per page
100 per page
Legend
How to query
« First
Prev
1
2
3
4
5
6
7
Next
Last »
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
-
c.-894G>A
r.(?)
p.(=)
-
likely benign
g.228029515C>T
-
COL4A3(NM_000091.4):c.73C>T (p.P25S)
-
COL4A3_000180
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
-?/.
1
-
c.-892G>C
r.(?)
p.(=)
-
likely benign
g.228029513C>G
-
COL4A3(NM_000091.4):c.71C>G (p.A24G)
-
COL4A3_000179
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
?/.
1
-
c.-874_-851del
r.(?)
p.(=)
-
VUS
g.228029482_228029505del
g.227164766_227164789del
-
-
COL4A3_000003
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
-?/.
1
-
c.-866C>T
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
-?/.
1
-
c.-865_-854del
r.(?)
p.(=)
-
likely benign
g.228029485_228029496del
-
COL4A3(NM_000091.5):c.43_54del (p.(Pro15_Leu18del))
-
COL4A3_000460
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
-?/.
1
-
c.-854C>A
r.(?)
p.(=)
-
likely benign
g.228029475G>T
g.227164759G>T
-
-
COL4A3_000622
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
?/.
1
-
c.-838G>T
r.(?)
p.(=)
-
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
-
c.-812G>A
r.(?)
p.(=)
-
likely benign
g.228029433C>T
g.227164717C>T
-
-
COL4A3_000001
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
-?/., ?/.
3
-
c.-101-4A>G
r.spl?
p.?
-
likely benign, VUS
g.228012304T>C
g.227147588T>C
COL4A4(NM_000092.4):c.-101-4A>G (p.(=))
-
COL4A4_000392
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
,
VKGL-NL_Rotterdam
,
VKGL-NL_Utrecht
-/.
1
-
c.-79C>T
r.(?)
p.(=)
-
benign
g.228012278G>A
g.227147562G>A
COL4A4(NM_000092.4):c.-79C>T
-
COL4A4_000391
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
+?/.
1
2
c.-23T>G
r.(?)
p.(=)
-
likely pathogenic
g.228012222A>C
g.227147506A>C
-
-
COL4A4_000543
-
PubMed: Liu 2017
,
Journal: Liu 201
-
-
Germline
-
-
-
-
-
Judy Savige
-/.
1
-
c.-15T>C
r.(?)
p.(=)
-
benign
g.228012214A>G
g.227147498A>G
-
-
COL4A4_000628
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
-/-?, -?/., ./.
3
1, 2
c.-2C>T
r.(?)
p.(=)
-
benign, likely benign
g.228012201G>A
g.227147485G>A
-
-
COL4A4_000001
VKGL data sharing initiative Nederland
Matonagel,
PubMed: Badenas 2002
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Judy Savige
,
VKGL-NL_Nijmegen
+/.
2
_1_4i
c.(?_-1)_(192+1_193-1)del
r.0?
p.0?
-
pathogenic
g.(227985865_228004876)_(228012200_?)del, g.(227985865_228004876)_(228029530_228102683)del
-
del ex1 COL4A3, ex1-4 COL4A4, del ex1-4
-
COL4A4_000408
-
PubMed: Mencarelli 2015
,
Journal: Mencarelli 2015
,
PubMed: Moriniere 2014
,
Journal: Moriniere 2014
-
-
Germline
-
-
-
-
-
Judy Savige
./.
1
2
c.15C>T
r.(?)
p.(=)
-
-
g.228012185G>A
g.227147469G>A
-
-
COL4A4_000283
-
Matonagel
-
-
Germline
-
-
-
-
-
Judy Savige
./.
1
2
c.17T>A
r.(?)
p.(Ile6Lys)
-
-
g.228012183A>T
g.227147467A>T
-
-
COL4A4_000284
-
Matonagel
-
-
Germline
-
-
-
-
-
Judy Savige
-/-?, -/.
3
2
c.17T>C
r.(?)
p.(Ile6Thr)
-
benign
g.228012183A>G
g.227147467A>G
-
-
COL4A4_000002
VKGL data sharing initiative Nederland
PubMed: Badenas 2002
,
PubMed: Tazon Vega 2003
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Judy Savige
,
VKGL-NL_Nijmegen
+?/.
1
2
c.32_42del
r.(?)
p.(Cys11Phefs*46)
-
likely pathogenic
g.228012158_228012168del
g.227147442_227147452del
32delGCTCCTTCAGA
-
COL4A4_000003
Heterozygous. Missense.
PubMed: Badenas 2002
-
-
Germline
-
-
-
-
-
Judy Savige
+/+?, -?/., ?/.
3
2
c.50A>G
r.(?)
p.(Lys17Arg)
-
likely benign, pathogenic, VUS
g.228012150T>C
g.227147434T>C
COL4A4(NM_000092.4):c.50A>G (p.K17R)
-
COL4A4_000213
VKGL data sharing initiative Nederland
1000 genomes
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Judy Savige
,
VKGL-NL_Utrecht
+/., +?/.
2
-
c.71+1del
r.spl?
p.?
-
likely pathogenic, pathogenic
g.228012129del
g.227147413del
COL4A4(NM_000092.5):c.71+1del
-
COL4A4_000627
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
,
VKGL-NL_Nijmegen
+?/., ./.
2
2i
c.71+1G>A
r.spl, r.spl?
p.?
-
likely pathogenic
g.228012128C>T
g.227147412C>T
-
-
COL4A4_000285
VKGL data sharing initiative Nederland
Matonagel
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Judy Savige
,
VKGL-NL_Nijmegen
?/.
1
2i
c.71+5G>C
r.spl?
p.?
-
VUS
g.228012124C>G
g.227147408C>G
-
-
COL4A4_000542
-
-
-
-
Germline
-
-
-
-
-
Judy Savige
-/., -?/.
2
-
c.71+10C>T
r.(=)
p.(=)
-
benign, likely benign
g.228012119G>A
-
COL4A4(NM_000092.4):c.71+10C>T, COL4A4(NM_000092.5):c.71+10C>T
-
COL4A4_000683
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
,
VKGL-NL_Utrecht
+/.
1
2i
c.72-26_72-23del
r.72_r113del
p.Trp24*
ACMG
pathogenic (recessive)
g.228009300_228009303del
g.227144584_227144587del
c.72-26_72-23delTAAT
-
COL4A4_000670
ACMG PVS1 PM2 PP4; might affect branch point
PubMed: Wang 2021
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-?/.
1
-
c.72-18G>A
r.(=)
p.(=)
-
likely benign
g.228009292C>T
-
COL4A4(NM_000092.4):c.72-18G>A
-
COL4A4_000756
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
-?/.
1
-
c.72-12G>A
r.(=)
p.(=)
-
likely benign
g.228009286C>T
g.227144570C>T
-
-
COL4A4_000626
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
-?/.
1
-
c.72-3T>C
r.spl?
p.?
-
likely benign
g.228009277A>G
-
COL4A4(NM_000092.4):c.72-3T>C (p.?)
-
COL4A4_000751
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+/., +?/., ./.
11
3
c.81_86del
r.(?)
p.(Ile29_Leu30del)
-
likely pathogenic, pathogenic, VUS
g.228009262_228009267del
g.227144546_227144551del
79_84delATACTC, 81_86delACTCAT, COL4A4(NM_000092.4):c.81_86delACTCAT (p.I29_L30del)
-
COL4A4_000264
VKGL data sharing initiative Nederland
Matonagel,
PubMed: Moriniere 2014
,
Journal: Moriniere 2014
,
PubMed: Storey 2013
,
1 more item
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Helen Storey
,
Judy Savige
,
VKGL-NL_Utrecht
./.
1
3
c.82_85del
r.(?)
p.(Leu28Phefs*65)
-
-
g.228009262_228009265del
g.227144546_227144549del
81_84delACTC
-
COL4A4_000287
-
Matonagel
-
-
Germline
-
-
-
-
-
Judy Savige
?/.
1
3
c.86T>A
r.(?)
p.(Ile29Asn)
-
VUS
g.228009260A>T
g.227144544A>T
-
-
COL4A4_000541
-
-
-
-
Germline
-
-
-
-
-
Judy Savige
+/.
1
3
c.93_94del
r.(?)
p.(Ser32Cysfs*28)
-
pathogenic
g.228009254_228009255del
g.227144538_227144539del
93_94delTT
-
COL4A4_000540
-
-
-
-
Germline
-
-
-
-
-
Judy Savige
-/-?, -?/., ?/.
4
3
c.102A>G
r.(?)
p.(=), p.(Gln34=)
-
benign, likely benign, VUS
g.228009244T>C
g.227144528T>C
COL4A4(NM_000092.4):c.102A>G (p.Q34=)
-
COL4A4_000004
VKGL data sharing initiative Nederland
PubMed: Baek 2009
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Judy Savige
,
VKGL-NL_Rotterdam
,
VKGL-NL_Utrecht
,
VKGL-NL_Nijmegen
+/+?
1
3
c.104A>G
r.(?)
p.(Tyr35Cys)
-
pathogenic
g.228009242T>C
g.227144526T>C
-
-
COL4A4_000005
Compound heterozygous
PubMed: Longo 2006
-
-
Germline
-
-
-
-
-
Judy Savige
./.
1
3
c.114G>A
r.(?)
p.(=)
-
VUS
g.228009232C>T
g.227144516C>T
-
-
COL4A4_000539
-
PubMed: Moriniere 2014
,
Journal: Moriniere 2014
-
-
Germline
-
-
-
-
-
Judy Savige
+?/.
1
3i
c.114+1G>C
r.spl
p.?
-
likely pathogenic
g.228009231C>G
g.227144515C>G
IVS3+1G>C
-
COL4A4_000411
-
PubMed: Rosado 2014
-
-
Germline
-
-
-
-
-
Judy Savige
?/.
2
3i
c.114+2_114+4dup
r.spl?
p.?
-
VUS
g.228009228_228009230dup
g.227144512_227144514dup
114+2_114+4dupTAA
-
COL4A4_000538
-
-
-
-
Germline
-
-
-
-
-
Judy Savige
?/.
1
-
c.116G>A
r.(?)
p.(Ser39Asn)
-
VUS
g.228004953C>T
g.227140237C>T
COL4A4(NM_000092.4):c.116G>A (p.S39N)
-
COL4A4_000390
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
./.
1
4
c.138T>C
r.(?)
p.(=)
-
-
g.228004931A>G
g.227140215A>G
-
-
COL4A4_000262
-
1000 genomes
-
-
Germline
-
-
-
-
-
Judy Savige
?/.
1
-
c.190C>T
r.(?)
p.(Arg64Trp)
-
VUS
g.228004879G>A
-
COL4A4(NM_000092.4):c.190C>T (p.R64W)
-
COL4A4_000669
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/.
1
4i
c.192+2T>C
r.spl
p.?
-
pathogenic
g.228004875A>G
g.227140159A>G
-
-
COL4A4_000537
-
-
-
-
Germline
-
-
-
-
-
Judy Savige
+?/.
1
4i
c.193-2A>C
r.spl
p.?
-
pathogenic (recessive)
g.227985866T>G
g.227121150T>G
-
-
COL4A4_000545
-
-
-
-
Germline
yes
-
-
-
-
Tamara Nikuseva Martic
-/-?, -?/., ./.
4
5
c.195T>C
r.(?)
p.(=), p.(Gly65=)
-
benign, likely benign
g.227985862A>G
g.227121146A>G
COL4A4(NM_000092.4):c.195T>C (p.G65=), reported 2 times
-
COL4A4_000006
VKGL data sharing initiative Nederland
Matonagel,
PubMed: Tazon Vega 2003
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Judy Savige
,
VKGL-NL_Utrecht
,
VKGL-NL_Nijmegen
-?/., ./.
3
5
c.198A>G
r.(?)
p.(=), p.(Pro66=)
-
likely benign
g.227985859T>C
g.227121143T>C
COL4A4(NM_000092.4):c.198A>G (p.P66=)
-
COL4A4_000246
VKGL data sharing initiative Nederland
1000 genomes
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Judy Savige
,
VKGL-NL_Utrecht
,
VKGL-NL_Nijmegen
./.
1
5
c.217C>T
r.(?)
p.(Gln73*)
-
VUS
g.227985840G>A
g.227121124G>A
-
-
COL4A4_000536
-
PubMed: Fallerini 2014
, (DOI:Fallerini 2014:10.1111/cge.12258}
-
-
Germline
-
-
-
-
-
Judy Savige
?/.
1
-
c.218_233del
r.(?)
p.(Gln73Profs*16)
-
VUS
g.227985825_227985840del
-
COL4A4(NM_000092.4):c.218_233delAGGGTCCAATTGGACC (p.(Gln73fs))
-
COL4A4_000750
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
?/.
1
5
c.230G>A
r.(?)
p.(Gly77Glu)
-
VUS
g.227985827C>T
g.227121111C>T
-
-
COL4A4_000214
-
1000 genomes
-
-
Germline
-
-
-
-
-
Judy Savige
+?/.
1
-
c.293del
r.(?)
p.(Gly98AlafsTer23)
-
likely pathogenic
g.227985765del
g.227121049del
-
-
COL4A4_000625
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
-?/.
1
-
c.303A>T
r.(?)
p.(Gly101=)
-
likely benign
g.227985754T>A
-
COL4A4(NM_000092.4):c.303A>T (p.G101=)
-
COL4A4_000714
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
./.
1
5
c.313del
r.(?)
p.(Asp105Thrfs*16)
-
VUS
g.227985747del
g.227121031del
313delG
-
COL4A4_000535
-
PubMed: Moriniere 2014
,
Journal: Moriniere 2014
-
-
Germline
-
-
-
-
-
Judy Savige
-/-?
1
5i
c.327+86C>T
r.(?)
p.(=)
-
benign
g.227985644G>A
g.227120928G>A
-
-
COL4A4_000007
-
PubMed: Marcocci 2009
-
-
Germline
-
-
-
-
-
Judy Savige
+?/.
1
-
c.328-1G>A
r.spl?
p.?
-
likely pathogenic
g.227984656C>T
g.227119940C>T
-
-
COL4A4_000624
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
?/.
1
-
c.337G>C
r.(?)
p.(Gly113Arg)
-
VUS
g.227984646C>G
-
COL4A4(NM_000092.5):c.337G>C (p.(Gly113Arg))
-
COL4A4_000736
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+?/.
1
6
c.347G>A
r.(?)
p.(Gly116Glu)
-
likely pathogenic
g.227984636C>T
g.227119920C>T
-
-
COL4A4_000008
heterozygous; missense
PubMed: Buzza 2003
-
-
Germline
-
-
-
-
-
Judy Savige
+?/., ./.
2
7
c.410G>A
r.(?)
p.(Gly137Asp), p.Gly137Asp
-
likely pathogenic, VUS
g.227983440C>T
g.227118724C>T
-
-
COL4A4_000534
-
PubMed: Malone 2014
,
Journal: Malone 2014
-
-
Germline
-
-
-
-
-
Judy Savige
./.
1
7
c.436G>A
r.(?)
p.(Gly146Ser)
-
-
g.227983414C>T
g.227118698C>T
-
-
COL4A4_000289
-
Matonagel
-
-
Germline
-
-
-
-
-
Judy Savige
./.
2
7
c.446G>A
r.(?)
p.(Gly149Glu)
-
VUS
g.227983404C>T
g.227118688C>T
-
-
COL4A4_000533
-
PubMed: Weber 2016
,
Journal: Weber 2016
-
-
Germline
-
-
-
-
-
Judy Savige
./.
1
7
c.455G>A
r.(?)
p.(Gly152Glu)
-
VUS
g.227983395C>T
g.227118679C>T
-
-
COL4A4_000532
-
PubMed: Moriniere 2014
,
Journal: Moriniere 2014
-
-
Germline
-
-
-
-
-
Judy Savige
./.
1
7
c.480_482dup
r.(?)
p.(Gly161dup)
-
-
g.227983371_227983373dup
g.227118655_227118657dup
478_480dupGGA
-
COL4A4_000290
-
Matonagel
-
-
Germline
-
-
-
-
-
Judy Savige
+?/.
3
7
c.481G>C
r.(?)
p.(Gly161Arg)
-
likely pathogenic
g.227983369C>G
g.227118653C>G
COL4A4(NM_000092.4):c.481G>C (p.(Gly161Arg))
-
COL4A4_000531
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Judy Savige
,
VKGL-NL_Leiden
,
VKGL-NL_Nijmegen
?/.
1
-
c.482G>T
r.(?)
p.(Gly161Val)
-
VUS
g.227983368C>A
g.227118652C>A
-
-
COL4A4_000564
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+?/.
1
7i
c.489+6A>G
r.(?)
p.(=)
-
likely pathogenic
g.227983355T>C
g.227118639T>C
-
-
COL4A4_000009
-
PubMed: Baek 2009
-
-
Germline
-
-
-
-
-
Judy Savige
-/-?, -/.
2
7i
c.490-121T>G
r.(?)
p.(=), p.?
-
benign
g.227979533A>C
g.227114817A>C
-
-
COL4A4_000010
variant in COL4A4:c.1598G>A homozygote
PubMed: Baek 2009
,
PubMed: Plevova 2023
-
rs12465531
Germline
-
-
-
-
-
Johan den Dunnen
,
Judy Savige
-/.
1
-
c.490-72T>A
r.(=)
p.(=)
-
benign
g.227979484A>T
g.227114768A>T
COL4A4(NM_000092.4):c.490-72T>A
-
COL4A4_000389
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
-/.
1
-
c.490-40T>C
r.(=)
p.(=)
-
benign
g.227979452A>G
g.227114736A>G
-
-
COL4A4_000623
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
-?/.
1
-
c.490-36A>G
r.(=)
p.(=)
-
likely benign
g.227979448T>C
g.227114732T>C
-
-
COL4A4_000622
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+?/.
2
-
c.490-1G>C
r.spl?
p.?
-
likely pathogenic
g.227979413C>G
-
COL4A4(NM_000092.4):c.490-1G>C
-
COL4A4_000635
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
,
VKGL-NL_Nijmegen
+/.
1
8
c.498T>C
r.(?)
p.(=)
-
pathogenic
g.227979404A>G
g.227114688A>G
-
-
COL4A4_000530
-
-
-
-
Germline
-
-
-
-
-
Judy Savige
+/.
1
8
c.499G>C
r.(?)
p.(Gly167Arg)
-
pathogenic
g.227979405G>C
-
-
-
COL4A4_000632
1 more item
-
-
-
Germline
yes
-
-
-
-
Pavlina Plevova
+?/.
2
-
c.500G>A
r.(?)
p.(Gly167Glu)
-
likely pathogenic
g.227979402C>T
-
COL4A4(NM_000092.4):c.500G>A (p.G167E)
-
COL4A4_000735
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
,
VKGL-NL_Nijmegen
-?/.
1
-
c.507G>C
r.(?)
p.(Lys169Asn)
-
likely benign
g.227979395C>G
-
COL4A4(NM_000092.4):c.507G>C (p.K169N)
-
COL4A4_000698
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
./.
1
8
c.508G>A
r.(?)
p.(Gly170Arg)
-
VUS
g.227979394C>T
g.227114678C>T
-
-
COL4A4_000529
-
PubMed: Moriniere 2014
,
Journal: Moriniere 2014
-
-
Germline
-
-
-
-
-
Judy Savige
?/.
1
-
c.509G>A
r.(?)
p.(Gly170Glu)
-
VUS
g.227979393C>T
g.227114677C>T
-
-
COL4A4_000398
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/.
1
-
c.516dup
r.(?)
p.(Gly173Argfs*11)
-
pathogenic
g.227979390dup
-
COL4A4(NM_000092.4):c.516dupA (p.G173Rfs*11)
-
COL4A4_000728
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
+/.
1
-
c.551del
r.(?)
p.(Gly184Valfs*35)
-
pathogenic
g.227979352del
-
-
-
COL4A4_000697
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+?/.
1
-
c.558+4_558+7del
r.490_558del
p.164_186del
ACMG
likely pathogenic
g.227979340_227979343del
g.227114624_227114627del
-
-
COL4A4_000693
ACMG PM2, PM4, PP1, PP4
PubMed: He 2022
-
-
Germline/De novo (untested)
-
-
-
-
-
Johan den Dunnen
-?/.
1
-
c.558+118C>A
r.(=)
p.(=)
-
likely benign
g.227979226G>T
g.227114510G>T
-
-
COL4A4_000621
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/.
1
7i_25i
c.559-491_1370-667delinsT[?]
r.?
p.?
-
pathogenic
g.227955340_227976920delinsA[?]
-
559-491_1460-1808delinsPolyT
-
COL4A4_000417
-
PubMed: Oka 2014
,
Journal: Oka 2014
-
-
Germline
-
-
-
-
-
Judy Savige
-/.
1
-
c.559-155C>T
r.(=)
p.(=)
-
benign
g.227976584G>A
g.227111868G>A
COL4A4(NM_000092.4):c.559-155C>T
-
COL4A4_000388
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
-/-?
1
8i
c.559-83T>C
r.(?)
p.(=)
-
benign
g.227976512A>G
g.227111796A>G
-
-
COL4A4_000011
-
PubMed: Baek 2009
-
-
Germline
-
-
-
-
-
Judy Savige
-/.
2
-
c.559-21A>G
r.(=)
p.(=)
-
benign
g.227976450T>C
g.227111734T>C
COL4A4(NM_000092.4):c.559-21A>G
-
COL4A4_000387
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
,
VKGL-NL_Nijmegen
./.
1
9
c.560del
r.(?)
p.(Gly187Glufs*32)
-
VUS
g.227976430del
g.227111714del
560delG
-
COL4A4_000528
-
PubMed: Moriniere 2014
,
Journal: Moriniere 2014
-
-
Germline
-
-
-
-
-
Judy Savige
?/.
1
9
c.563A>G
r.(?)
p.(Asp188Gly)
-
VUS
g.227976425T>C
g.227111709T>C
203A>G (Asp188Gly)
-
COL4A4_000362
-
1000 genomes
-
-
Germline
-
-
-
-
-
Judy Savige
./.
1
9
c.586G>A
r.(?)
p.(Gly196Ser)
-
-
g.227976402C>T
g.227111686C>T
-
-
COL4A4_000291
-
Matonagel
-
-
Germline
-
-
-
-
-
Judy Savige
+?/.
1
-
c.594+1G>A
r.spl
p.?
ACMG
pathogenic (dominant)
g.227976393C>T
-
-
-
COL4A4_000684
ACMG: PVS1, PM2_SUP, PP1 mother and brother affected and carry the variant
-
-
-
Germline
yes
-
-
-
-
Andreas Laner
./.
1
9i
c.594+1G>C
r.spl
p.?
-
-
g.227976393C>G
g.227111677C>G
-
-
COL4A4_000292
-
Matonagel
-
-
Germline
-
-
-
-
-
Judy Savige
-/.
1
-
c.594+63T>C
r.(=)
p.(=)
-
benign
g.227976331A>G
g.227111615A>G
COL4A4(NM_000092.4):c.594+63T>C
-
COL4A4_000386
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
-/.
1
-
c.594+96C>T
r.(=)
p.(=)
-
benign
g.227976298G>A
g.227111582G>A
COL4A4(NM_000092.4):c.594+96C>T
-
COL4A4_000385
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
-/-?
1
9i
c.594+162G>T
r.(?)
p.(=)
-
benign
g.227976232C>A
g.227111516C>A
-
-
COL4A4_000013
-
PubMed: Baek 2009
-
-
Germline
-
-
-
-
-
Judy Savige
-/-?, -/.
3
9i
c.595-111T>C
r.(=), r.(?)
p.(=), p.?
-
benign
g.227974113A>G
g.227109397A>G
-
-
COL4A4_000012
variant in COL4A4:c.1598G>A homozygote, VKGL data sharing initiative Nederland
PubMed: Baek 2009
,
PubMed: Plevova 2023
-
rs6436654
CLASSIFICATION record, Germline
-
-
-
-
-
Johan den Dunnen
,
Judy Savige
,
VKGL-NL_Nijmegen
-?/.
1
-
c.595-67G>A
r.(=)
p.(=)
-
likely benign
g.227974069C>T
g.227109353C>T
-
-
COL4A4_000620
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+?/.
1
-
c.595-2A>G
r.spl?
p.?
-
likely pathogenic
g.227974004T>C
g.227109288T>C
-
-
COL4A4_000619
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/+?
1
10
c.595G>A
r.spl?
p.(Gly199Arg)
-
pathogenic
g.227974002C>T
g.227109286C>T
-
-
COL4A4_000014
-
PubMed: Baek 2009
-
-
Germline
-
-
-
-
-
Judy Savige
+?/.
1
-
c.596G>A
r.(?)
p.(Gly199Glu)
-
likely pathogenic
g.227974001C>T
-
COL4A4(NM_000092.4):c.596G>A (p.G199E)
-
COL4A4_000734
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
./.
1
10
c.621A>G
r.(?)
p.(=)
-
-
g.227973976T>C
g.227109260T>C
-
-
COL4A4_000293
-
Matonagel
-
-
Germline
-
-
-
-
-
Judy Savige
-/.
2
-
c.657+48A>G
r.(=), r.(?)
p.(=), p.?
-
benign
g.227973892T>C
g.227109176T>C
-
-
COL4A4_000652
variant in COL4A4:c.1598G>A homozygote, VKGL data sharing initiative Nederland
PubMed: Plevova 2023
-
rs73082223
CLASSIFICATION record, Germline
-
-
-
-
-
Johan den Dunnen
,
VKGL-NL_Nijmegen
-/.
1
-
c.657+62G>A
r.(?)
p.?
-
benign
g.227973878C>T
g.227109162C>T
-
-
COL4A4_000709
variant in COL4A4:c.1598G>A homozygote
PubMed: Plevova 2023
-
rs59938187
Germline
-
-
-
-
-
Johan den Dunnen
-/-?, -/.
3
10i
c.657+67A>G
r.(=), r.(?)
p.(=), p.?
-
benign
g.227973873T>C
g.227109157T>C
-
-
COL4A4_000015
variant in COL4A4:c.1598G>A homozygote, VKGL data sharing initiative Nederland
PubMed: Baek 2009
,
PubMed: Plevova 2023
-
rs58363082
CLASSIFICATION record, Germline
-
-
-
-
-
Johan den Dunnen
,
Judy Savige
,
VKGL-NL_Nijmegen
-/-?, -/.
3
10i
c.658-39T>C
r.(=), r.(?)
p.(=), p.?
-
benign
g.227973623A>G
g.227108907A>G
-
-
COL4A4_000016
variant in COL4A4:c.1598G>A homozygote, VKGL data sharing initiative Nederland
PubMed: Baek 2009
,
PubMed: Plevova 2023
-
rs12475686
CLASSIFICATION record, Germline
-
-
-
-
-
Johan den Dunnen
,
Judy Savige
,
VKGL-NL_Nijmegen
-/., -?/., ./.
4
11
c.666G>A
r.(?)
p.(=), p.(Pro222=)
-
benign, likely benign
g.227973576C>T
g.227108860C>T
COL4A4(NM_000092.4):c.666G>A (p.P222=)
-
COL4A4_000215
VKGL data sharing initiative Nederland
1000 genomes
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Judy Savige
,
VKGL-NL_Rotterdam
,
VKGL-NL_Utrecht
,
VKGL-NL_Nijmegen
?/.
3
-
c.680G>A
r.(?)
p.(Arg227His)
ACMG
VUS
g.227973562C>T
g.227108846C>T
COL4A4 c.680G>A, p.R227H, COL4A4(NM_000092.4):c.680G>A (p.R227H)
-
COL4A4_000384
heterozygous; unsolved, VKGL data sharing initiative Nederland
PubMed: Zacchia 2021
-
-
CLASSIFICATION record, Unknown
?
-
-
-
-
VKGL-NL_Rotterdam
,
VKGL-NL_Nijmegen
10 per page
25 per page
50 per page
100 per page
Legend
How to query
« First
Prev
1
2
3
4
5
6
7
Next
Last »
Screenscraping/webscraping (downloading large amounts of data using scripts) is strictly prohibited.
Use our
APIs
to retrieve data.
Powered by
LOVD v.3.0
Build 30b
LOVD software ©2004-2024
Leiden University Medical Center
Database contents © by their respective submitters and curators