Global Variome shared LOVD
NPHS2 (nephrosis 2, idiopathic, steroid-resistant ...)
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Curator:
Olivier Gribouval
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All variants in the NPHS2 gene
The variants shown are described using the NM_014625.2 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.
Exon
: number of exon/intron containing variant; 2 = exon 2, 12i = intron 12, 2i_7i = from intron 2 to intron 7, 8i_9 = intron 8/exon 9 boundary, _1 = 5' to exon 1, 18_ = 3' of exon 18, _1_18_ = encompassing the entire 18-exon gene
DNA change (cDNA)
: description of variant at DNA level, based on a coding DNA reference sequence (following HGVS recommendations); e.g. c.123C>T, c.123_145del, c.123_126dup. For deletions/duplications extending beyond the reference transcript resp. {0}/{2} is used to replace del/dup. Extent of the deletion/duplication should be specified using the genomic description (g.). "-" indicates the variant described on genomic level does not affect the coding DNA reference sequence.
RNA change
: description of variant at RNA level (following HGVS recommendations).
r.123c>u
r.? = unknown
r.(?) = RNA not analysed but probably transcribed copy of DNA variant
r.spl? = RNA not analysed but variant probably affects splicing
r.(spl?) = RNA not analysed but variant may affect splicing
r.0? = change expected to abolish transcription
Protein
: description of variant at protein level (following HGVS recommendations).
p.(Arg345Pro) = change predicted from DNA (RNA not analysed)
p.Arg345Pro = change derived from RNA analysis
p.? = unknown effect
p.0? = probably no protein produced
Allele
: On which allele is the variant located? Does not necessarily imply inheritance! 'Paternal' (confirmed or inferred), 'Maternal' (confirmed or inferred), 'Parent #1' or #2 for compound heterozygosity without having screened the parents, 'Unknown' for heterozygosity without having screened the parents, 'Both' for homozygozity.
Classification method
: The method used for the clinical classification of this variant.
All options:
ACMG
ACGS
EAHAD-CFDB
ENIGMA
IARC
InSiGHT
kConFab
other
Clinical classification
: Clinical classification of variant, preferably based on standardised criteria (e.g. ACMG), directed on the clinical consequences as published/submitted, indicated using an enriched system including inheritance: e.g. pathogenic, pathogenic (dominant), pathogenic (recessive), pathogenic (!), pathogenic (maternal), pathogenic (paternal). Standard inheritance is covered by dominant/recessive, imprinting by maternal/paternal. A '!' warns for exceptional circumstances to be explained in the 'Remarks' field (low penetrance, variants pathogenic in heterozygous state only, hypomorphic/hypermorphic variants, protective variants, etc.). Non-disease consequences (e.g. drug metabolism (pharmacogenetics), risk factor, blood group, tasting bitter) are indicated using additions to the benign classification; benign (dominant), benign (recessive), benign (!), etc. The value 'association' is used for variants associated with a phenotype and 'NA' for variants from in vitro/in silico records. NOTE: classification may differ from the opinion of the curator as given in a variant SUMMARY-record or the 'Functional effect concluded'). NOTE: pathogenic/likely pathogenic should go together with "variant (probably) affects function" In ClassFunctional.
All options:
pathogenic
pathogenic (dominant)
pathogenic (recessive)
pathogenic (!)
pathogenic (maternal)
pathogenic (paternal)
likely pathogenic
likely pathogenic (dominant)
likely pathogenic (recessive)
likely pathogenic (!)
likely pathogenic (maternal)
likely pathogenic (paternal)
VUS
VUS (!)
likely benign
likely benign (dominant)
likely benign (recessive)
likely benign (!)
likely benign (maternal)
likely benign (paternal)
benign
benign (dominant)
benign (recessive)
benign (!)
benign (maternal)
benign (paternal)
conflicting
association
NA
DNA change (genomic) (hg19)
: HGVS description of variant at DNA level, based on the genomic (chromosomal) DNA reference sequence; e.g. g.12345678C>T, g.12345679del, g.12345678_12345890dup
DNA change (hg38)
: HGVS description of variant at DNA level, based on the hg38 genomic (chromosomal) eference sequence; e.g. g.12345678C>T, g.12345679del, g.12345678_12345890dup
Published as
: listed only when different from "DNA change"; variant as reported originally (e.g. 521delT). Variants seen in animal models, tested in vitro, predicted from RNA analysis, etc. are described between brackets like c.(456C>G)
ISCN
: description of the variant according to ISCN nomenclature
DB-ID
: database ID of variant, grouping multiple observations of the same variant together, starting with the HGNC gene symbol, followed by an underscore (_) and a six digit number (e.g. DMD_012345). _000000 is used for variants where DNA was not analysed (change predicted from RNA analysis), variants seen in animal models or variants not seen in humans but functionally tested in vitro
Variant remarks
: remarks regarding variant described, e.g. germline mosaicism in mother, 345 kb deletion, muscle RNA analysed, not in 200 control chromosomes tested, on founder haplotype, etc.
Reference
: publication describing the variant submitted, incl. links to OMIM, PubMed or other source, e.g. "den Dunnen ASHG2003 P2346"
ClinVar ID
: ID of variant in ClinVar database
dbSNP ID
: the dbSNP ID
Origin
: Origin of variant/record: Germline = in all cells, De novo = in all cells, but not in either parent, Germline/De novo (untested) = in all cells, parents not tested (use only when De novo is likely, e.g. isolated/sporadic cases with dominant disease), Somatic = present in a subset of cells, but not in either parent, Uniparental disomy = from parental disomy (maternal or paternal), CLASSIFICATION record = submitter only sharing variant classification (note another report may share Individual data), SUMMARY record = master summary record from curator (may link to another database), In vitro (cloned) = data resulting from in vitro functional assays, animal model = data from animal model, Artefact = false positive variant call, DUPLICATE record = variant already described on another chromosome (e.g. unbalanced translocation, duplicating transposition, 2nd fusion transcript, etc.)
All options:
Germline
De novo
Germline/De novo (untested)
Somatic
Uniparental disomy
Uniparental disomy, maternal allele
Uniparental disomy, paternal allele
CLASSIFICATION record
SUMMARY record
In vitro (cloned)
In silico
animal model
Artefact
DUPLICATE record
Unknown
Not applicable
Segregation
: Indicates whether the variant segregates with the phenotype (yes), does not segregate with the phenotype (no) or segregation is unknown (?)
All options:
? = unknown
yes = segregates with phenotype
no = does not segregate with phenotype
- = not applicable
Frequency
: frequency in which the variant was found; e.g 5/760 chromosomes (in 5 of 760 chromosomes tested), 1/33 patients (in 1 of 33 patients analysed in study), 0.05 controls (in 5% of control cases tested)
Re-site
: restriction enzyme recognition site created (+) or destroyed (-); e.g. BglII+;BamHI-
VIP
: variant VIP-status was requested for matchmaking - need collaboration(s) to crack the case - please contact the submitter/curator. NOTE: to get VIP status ask the curator.
Methylation
: result of methylation test; GOM (gain of methylation), LOM (loss of methylation), 30% (30% methylated). NOTE: when several tests were done mention the method as well (e.g. MS-PCR 75%)
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Operator
Column type
Example
Matches
Text
Arg
all entries containing 'Arg'
space
Text
Arg Ser
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|
Text
Arg|Ser
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!
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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1086 entries on 11 pages. Showing entries 1 - 100.
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Effect
Exon
DNA change (cDNA)
RNA change
Protein
Classification method
Clinical classification
DNA change (genomic) (hg19)
DNA change (hg38)
Published as
ISCN
DB-ID
Variant remarks
Reference
ClinVar ID
dbSNP ID
Origin
Segregation
Frequency
Re-site
VIP
Methylation
Owner
-/.
-
c.-51G>T
r.(?)
p.(=)
-
benign
g.179545050C>A
g.179575915C>A
NPHS2(NM_014625.3):c.-51G>T
-
NPHS2_000177
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
-/.
-
c.-51G>T
r.(?)
p.(=)
-
benign
g.179545050C>A
g.179575915C>A
NPHS2(NM_014625.3):c.-51G>T
-
NPHS2_000177
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
?/.
1
c.-26G>T
r.(?)
p.(=)
-
VUS
g.179545025C>A
g.179575890C>A
-
-
NPHS2_000179
-
-
-
-
Germline
-
-
-
-
-
Judy Savige
?/?
1_8
c.?
r.?
p.?
-
VUS
g.?
-
-
-
NPHS2_000000
unknown variant 2nd chromosome
-
-
-
Germline
-
-
-
-
-
Johan den Dunnen
?/?
1_8
c.?
r.?
p.?
-
VUS
g.?
-
-
-
NPHS2_000000
unknown variant 2nd chromosome
-
-
-
Germline
-
-
-
-
-
Johan den Dunnen
?/?
1_8
c.?
r.?
p.?
-
VUS
g.?
-
-
-
NPHS2_000000
unknown variant 2nd chromosome
-
-
-
Germline
-
-
-
-
-
Johan den Dunnen
?/?
1_8
c.?
r.?
p.?
-
VUS
g.?
-
-
-
NPHS2_000000
unknown variant 2nd chromosome
-
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/?
3i
c.?
r.spl
p.?
-
pathogenic
g.179528895C>A
-
c.452+1G>T
-
NPHS2_000000
Variant Error [EREF/ESYNTAX]: This genomic variant does not match the reference sequence; the transcript variant also has an error. Please fix this entry and then remove this message.
Hinkes et al, 2008 (JASN)
-
-
Germline
-
-
-
-
-
Olivier Gribouval
?/?
1
c.17G>A
r.(?)
p.(Arg6Gln)
-
VUS
g.179544983C>T
g.179575848C>T
-
-
NPHS2_000165
-
Hinkes et al, 2008 (JASN)
-
-
Germline
-
-
-
-
-
Olivier Gribouval
+/?
1
c.28dup
r.(?)
p.(Arg10Lysfs*60)
-
pathogenic
g.179544972dup
g.179575837dup
-
-
NPHS2_000164
-
Ruf et al, 2004 (J Am Soc Nephrol)
-
-
Germline
-
-
-
-
-
Olivier Gribouval
+/?
1
c.28dup
r.(?)
p.(Arg10Lysfs*60)
-
pathogenic
g.179544972dup
g.179575837dup
-
-
NPHS2_000164
-
Hinkes et al, 2008 (JASN)
-
-
Germline
-
-
-
-
-
Olivier Gribouval
+/?
1
c.29G>C
r.(?)
p.(Arg10Thr)
-
pathogenic
g.179544971C>G
g.179575836C>G
-
-
NPHS2_000163
-
Tonna et al, 2008 (BMC Nephrology)
-
-
Germline
-
-
-
-
-
Olivier Gribouval
+/?
1
c.52A>T
r.(?)
p.(Arg18Trp)
-
pathogenic
g.179544948T>A
g.179575813T>A
-
-
NPHS2_000162
-
Berdeli et al, 2007 (Pediatr Nephrol)
-
-
Germline
-
-
-
-
-
Olivier Gribouval
+/?
1
c.59C>T
r.(?)
p.(P20L)
-
pathogenic
g.179544941G>A
g.179575806G>A
-
-
NPHS2_000004
not in 80 control chromosomes
{PMID10742096:Boute 2000},
OMIM:var0006
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/?
1
c.59C>T
r.(?)
p.(P20L)
-
pathogenic
g.179544941G>A
g.179575806G>A
-
-
NPHS2_000004
not in 80 control chromosomes
{PMID10742096:Boute 2000},
OMIM:var0006
-
-
Germline
-
-
-
-
-
Johan den Dunnen
?/?
1
c.59C>T
r.(?)
p.(P20L)
-
VUS
g.179544941G>A
g.179575806G>A
-
-
NPHS2_000004
-
-
-
rs74315344
Germline
-
-
-
-
-
Johan den Dunnen
-?/.
-
c.59C>T
r.(?)
p.(Pro20Leu)
-
likely benign
g.179544941G>A
g.179575806G>A
NPHS2(NM_014625.2):c.59C>T (p.(Pro20Leu)), NPHS2(NM_014625.3):c.59C>T (p.P20L)
-
NPHS2_000004
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
-?/.
-
c.59C>T
r.(?)
p.(Pro20Leu)
-
likely benign
g.179544941G>A
-
NPHS2(NM_014625.2):c.59C>T (p.(Pro20Leu)), NPHS2(NM_014625.3):c.59C>T (p.P20L)
-
NPHS2_000004
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
+/?
1
c.77G>T
r.(?)
p.(Arg26Met)
-
pathogenic
g.179544923C>A
g.179575788C>A
-
-
NPHS2_000161
-
Berdeli et al, 2007 (Pediatr Nephrol)
-
-
Germline
-
-
-
-
-
Olivier Gribouval
+/?
1
c.83A>T
r.(?)
p.(Lys28Met)
-
pathogenic
g.179544917T>A
g.179575782T>A
-
-
NPHS2_000160
-
Berdeli et al, 2007 (Pediatr Nephrol)
-
-
Germline
-
-
-
-
-
Olivier Gribouval
?/?
1
c.85G>A
r.(?)
p.(Ala29Thr)
-
VUS
g.179544915C>T
g.179575780C>T
-
-
NPHS2_000159
-
Weber et al, 2004 (Kid Int)
-
-
Germline
-
-
-
-
-
Olivier Gribouval
+/?
1
c.88G>A
r.(?)
p.(Glu30Lys)
-
pathogenic
g.179544912C>T
g.179575777C>T
-
-
NPHS2_000158
-
Berdeli et al, 2007 (Pediatr Nephrol)
-
-
Germline
-
-
-
-
-
Olivier Gribouval
?/.
-
c.88G>A
r.(?)
p.(Glu30Lys)
-
VUS
g.179544912C>T
-
NPHS2(NM_014625.4):c.88G>A (p.(Glu30Lys))
-
NPHS2_000158
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
?/?
1
c.88G>C
r.(?)
p.(Glu30Gln)
-
VUS
g.179544912C>G
g.179575777C>G
-
-
NPHS2_000157
-
Berdeli et al, 2007 (Pediatr Nephrol)
-
-
Germline
-
-
-
-
-
Olivier Gribouval
-/.
-
c.102A>G
r.(?)
p.(Gly34=)
-
benign
g.179544898T>C
g.179575763T>C
NPHS2(NM_014625.3):c.102A>G (p.G34=), NPHS2(NM_014625.4):c.102A>G (p.G34=)
-
NPHS2_000173
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
-/.
-
c.102A>G
r.(?)
p.(Gly34=)
-
benign
g.179544898T>C
g.179575763T>C
NPHS2(NM_014625.3):c.102A>G (p.G34=), NPHS2(NM_014625.4):c.102A>G (p.G34=)
-
NPHS2_000173
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
-/.
-
c.102A>G
r.(?)
p.(Gly34=)
-
benign
g.179544898T>C
g.179575763T>C
NPHS2(NM_014625.3):c.102A>G (p.G34=), NPHS2(NM_014625.4):c.102A>G (p.G34=)
-
NPHS2_000173
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/?
1
c.104dup
r.(?)
p.(Arg36ProfsTer34)
-
pathogenic
g.179544897dup
g.179575762dup
104/4insG
-
NPHS2_000001
not in 80 control chromosomes
{PMID10742096:Boute 2000},
OMIM:var0003
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/?
1
c.104dup
r.(?)
p.(Arg36Profs*34)
-
pathogenic
g.179544897dup
g.179575762dup
-
-
NPHS2_000001
-
Weber et al, 2004 (Kid Int)
-
-
Germline
-
-
-
-
-
Olivier Gribouval
+/?
1
c.104dup
r.(?)
p.(Arg36Profs*34)
-
pathogenic
g.179544897dup
g.179575762dup
-
-
NPHS2_000001
-
Machuca et al, 2010 (JASN)
-
-
Germline
-
-
-
-
-
Olivier Gribouval
+/?
1
c.115_116insT
r.(?)
p.(Gln39Leufs*31)
-
pathogenic
g.179544884_179544885insA
g.179575749_179575750insA
-
-
NPHS2_000155
-
Berdeli et al, 2007 (Pediatr Nephrol)
-
-
Germline
-
-
-
-
-
Olivier Gribouval
?/?
1
c.116A>T
r.(?)
p.(Gln39Leu)
-
VUS
g.179544884T>A
g.179575749T>A
-
-
NPHS2_000156
-
Berdeli et al, 2007 (Pediatr Nephrol)
-
-
Germline
-
-
-
-
-
Olivier Gribouval
?/?
1
c.124G>A
r.(?)
p.(Gly42Arg)
-
VUS
g.179544876C>T
g.179575741C>T
-
-
NPHS2_000154
-
He et al, 2007 (Clin J Am Soc Nephrol)
-
-
Germline
-
-
-
-
-
Olivier Gribouval
-?/.
-
c.124G>A
r.(?)
p.(Gly42Arg)
-
likely benign
g.179544876C>T
-
NPHS2(NM_014625.3):c.124G>A (p.G42R)
-
NPHS2_000154
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
+/?
1
c.132dup
r.(?)
p.(Pro45Alafs*25)
-
pathogenic
g.179544868dup
g.179575733dup
-
-
NPHS2_000153
-
Bakr et al, 2008 (Indian J Pediatr)
-
-
Germline
-
-
-
-
-
Olivier Gribouval
+/?
1
c.134del
r.(?)
p.(Pro45Argfs*54)
-
pathogenic
g.179544867del
g.179575732del
-
-
NPHS2_000152
-
Bouchireb et al, 2013 (Hum Mutat)
-
-
Germline
-
-
-
-
-
Olivier Gribouval
+/?
1
c.137C>A
r.(?)
p.(Ser46*)
-
pathogenic
g.179544863G>T
g.179575728G>T
-
-
NPHS2_000151
-
Bouchireb et al, 2013 (Hum Mutat)
-
-
Germline
-
-
-
-
-
Olivier Gribouval
-?/.
-
c.138G>A
r.(?)
p.(Ser46=)
-
likely benign
g.179544862C>T
g.179575727C>T
NPHS2(NM_014625.3):c.138G>A (p.S46=)
-
NPHS2_000172
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
+/?
1
c.138_142dup
r.(?)
p.(Ser48Trpfs*53)
-
pathogenic
g.179544858_179544862dup
g.179575723_179575727dup
-
-
NPHS2_000150
-
Bouchireb et al, 2013 (Hum Mutat)
-
-
Germline
-
-
-
-
-
Olivier Gribouval
+/?
1
c.167del
r.(?)
p.(Glu56Glyfs*43)
-
pathogenic
g.179544833del
g.179575698del
-
-
NPHS2_000149
-
Bouchireb et al, 2013 (Hum Mutat)
-
-
Germline
-
-
-
-
-
Olivier Gribouval
-/.
-
c.182C>T
r.(?)
p.(Ala61Val)
-
benign
g.179544818G>A
g.179575683G>A
NPHS2(NM_014625.3):c.182C>T (p.A61V)
-
NPHS2_000171
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
-?/.
-
c.182C>T
r.(?)
p.(Ala61Val)
-
likely benign
g.179544818G>A
g.179575683G>A
NPHS2(NM_014625.3):c.182C>T (p.A61V)
-
NPHS2_000171
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/?
1
c.211C>T
r.(?)
p.(Arg71*)
-
pathogenic
g.179544789G>A
g.179575654G>A
-
-
NPHS2_000148
-
Sun et al, 2009 (Pathology)
-
-
Germline
-
-
-
-
-
Olivier Gribouval
+/?
1
c.211C>T
r.(?)
p.(Arg71*)
-
pathogenic
g.179544789G>A
g.179575654G>A
-
-
NPHS2_000148
-
Vasudevan et al, 2012 (Indian Pediatrics)
-
-
Germline
-
-
-
-
-
Olivier Gribouval
-?/.
-
c.220G>A
r.(?)
p.(Gly74Ser)
-
likely benign
g.179544780C>T
-
NPHS2(NM_014625.3):c.220G>A (p.G74S)
-
NPHS2_000194
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
+/?
1
c.249del
r.(?)
p.(Leu84Trpfs*15)
-
pathogenic
g.179544751del
g.179575616del
-
-
NPHS2_000147
-
Bouchireb et al, 2013 (Hum Mutat)
-
-
Germline
-
-
-
-
-
Olivier Gribouval
?/?
1
c.259G>A
r.(?)
p.(Glu87Lys)
-
VUS
g.179544741C>T
g.179575606C>T
-
-
NPHS2_000146
-
Lahdenkari et al, 2005 (Pediatr Nephrol)
-
-
Germline
-
-
-
-
-
Olivier Gribouval
+/?
1
c.259G>T
r.(?)
p.(Glu87*)
-
pathogenic
g.179544741C>A
g.179575606C>A
-
-
NPHS2_000145
-
Tikhomirov et al, 2007 (Hum Genet)
-
-
Germline
-
-
-
-
-
Olivier Gribouval
+/?
1
c.259G>T
r.(?)
p.(Glu87*)
-
pathogenic
g.179544741C>A
g.179575606C>A
-
-
NPHS2_000145
-
Hinkes et al, 2008 (JASN)
-
-
Germline
-
-
-
-
-
Olivier Gribouval
+/?
1
c.264_265del
r.(?)
p.(Pro89Argfs*13)
-
pathogenic
g.179544735_179544736del
g.179575600_179575601del
-
-
NPHS2_000143
-
Hinkes et al, 2008 (JASN)
-
-
Germline
-
-
-
-
-
Olivier Gribouval
?/?
1
c.265C>A
r.(?)
p.(Pro89Thr)
-
VUS
g.179544735G>T
g.179575600G>T
-
-
NPHS2_000144
-
Berdeli et al, 2007 (Pediatr Nephrol)
-
-
Germline
-
-
-
-
-
Olivier Gribouval
+/?
1
c.274G>T
r.(spl?)
p.(?)
-
pathogenic
g.179544726C>A
g.179575591C>A
-
-
NPHS2_000002
not in 80 control chromosomes
{PMID10742096:Boute 2000},
OMIM:var0007
-
-
Germline
-
-
-
-
-
Johan den Dunnen
?/?
1
c.274G>T
r.(spl?)
p.(?)
-
VUS
g.179544726C>A
g.179575591C>A
-
-
NPHS2_000002
-
-
-
rs74315345
Germline
-
-
-
-
-
Johan den Dunnen
+/?
1
c.274G>T
r.(spl?)
p.(Gly92Cys)
-
pathogenic
g.179544726C>A
g.179575591C>A
-
-
NPHS2_000002
-
Weber et al, 2004 (Kid Int)
-
-
Germline
-
-
-
-
-
Olivier Gribouval
+/?
1
c.274G>T
r.(spl?)
p.(Gly92Cys)
-
pathogenic
g.179544726C>A
g.179575591C>A
-
-
NPHS2_000002
-
Santin et al, 2011 (Clin J Am Soc Nephrol)
-
-
Germline
-
-
-
-
-
Olivier Gribouval
+/?
1
c.274G>T
r.(spl?)
p.(Gly92Cys)
-
pathogenic
g.179544726C>A
g.179575591C>A
-
-
NPHS2_000002
-
Santin et al, 2011 (Clin J Am Soc Nephrol)
-
-
Germline
-
-
-
-
-
Olivier Gribouval
+/?
1
c.274G>T
r.(spl?)
p.(Gly92Cys)
-
pathogenic
g.179544726C>A
g.179575591C>A
-
-
NPHS2_000002
-
Santin et al, 2011 (Clin J Am Soc Nephrol)
-
-
Germline
-
-
-
-
-
Olivier Gribouval
+/?
1
c.274G>T
r.(spl?)
p.(Gly92Cys)
-
pathogenic
g.179544726C>A
g.179575591C>A
-
-
NPHS2_000002
-
Santin et al, 2011 (Clin J Am Soc Nephrol)
-
-
Germline
-
-
-
-
-
Olivier Gribouval
+/?
1
c.274G>T
r.(spl?)
p.(Gly92Cys)
-
pathogenic
g.179544726C>A
g.179575591C>A
-
-
NPHS2_000002
-
Santin et al, 2011 (Clin J Am Soc Nephrol)
-
-
Germline
-
-
-
-
-
Olivier Gribouval
+/?
1
c.274G>T
r.(spl?)
p.(Gly92Cys)
-
pathogenic
g.179544726C>A
g.179575591C>A
-
-
NPHS2_000002
-
Santin et al, 2011 (Clin J Am Soc Nephrol)
-
-
Germline
-
-
-
-
-
Olivier Gribouval
+?/.
1
c.274G>T
r.(?)
p.(Gly92Cys)
-
likely pathogenic
g.179544726G>T
g.179575591C>A
-
-
NPHS2_000002
Variant Error [EBUILDMISMATCH]: This variant seems to mismatch; the genomic variants on hg19 and hg38 seem to not belong together. Please fix this entry and then remove this message.
PubMed: SantĆn et al. 2011
-
-
Unknown
-
-
-
-
-
Elisabet Ars Criach
+/?
1i
c.274+2T>A
r.spl
p.?
-
pathogenic
g.179544724A>T
g.179575589A>T
-
-
NPHS2_000142
-
Hinkes et al, 2008 (JASN)
-
-
Germline
-
-
-
-
-
Olivier Gribouval
-/.
-
c.274+138G>A
r.(=)
p.(=)
-
benign
g.179544588C>T
g.179575453C>T
NPHS2(NM_014625.3):c.274+138G>A
-
NPHS2_000170
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
+/?
1i
c.275-1G>C
r.spl
p.?
-
pathogenic
g.179533929C>G
g.179564794C>G
-
-
NPHS2_000141
-
Bouchireb et al, 2013 (Hum Mutat)
-
-
Germline
-
-
-
-
-
Olivier Gribouval
+/?
2
c.275G>A
r.(spl?)
p.(Gly92Asp)
-
pathogenic
g.179533928C>T
g.179564793C>T
-
-
NPHS2_000140
-
Hinkes et al, 2007 (Pediatrics)
-
-
Germline
-
-
-
-
-
Olivier Gribouval
+/?
2
c.275G>A
r.(spl?)
p.(Gly92Asp)
-
pathogenic
g.179533928C>T
g.179564793C>T
-
-
NPHS2_000140
-
Hinkes et al, 2008 (JASN)
-
-
Germline
-
-
-
-
-
Olivier Gribouval
-/.
-
c.288C>T
r.(?)
p.(Ser96=)
-
benign
g.179533915G>A
g.179564780G>A
-
-
NPHS2_000176
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
?/?
2
c.289G>A
r.(?)
p.(Gly97Ser)
-
VUS
g.179533914C>T
g.179564779C>T
-
-
NPHS2_000139
-
Bouchireb et al, 2013 (Hum Mutat)
-
-
Germline
-
-
-
-
-
Olivier Gribouval
?/.
-
c.289G>A
r.(?)
p.(Gly97Ser)
-
VUS
g.179533914C>T
-
NPHS2(NM_014625.3):c.289G>A (p.G97S)
-
NPHS2_000139
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/.
-
c.293_294del
r.(?)
p.(Leu98Trpfs*4)
ACMG
pathogenic
g.179533909_179533910del
g.179564774_179564775del
-
-
NPHS2_000193
-
-
-
-
Germline
-
-
-
-
-
Kar-Hui Ng
+/?
2
c.304G>A
r.(?)
p.(Glu102Lys)
-
pathogenic
g.179533899C>T
g.179564764C>T
-
-
NPHS2_000138
-
Ruf et al, 2004 (J Am Soc Nephrol)
-
-
Germline
-
-
-
-
-
Olivier Gribouval
+/?
2
c.304G>A
r.(?)
p.(Glu102Lys)
-
pathogenic
g.179533899C>T
g.179564764C>T
-
-
NPHS2_000138
-
Hinkes et al, 2008 (JASN)
-
-
Germline
-
-
-
-
-
Olivier Gribouval
+/?
2
c.320T>C
r.(?)
p.(Leu107Pro)
-
pathogenic
g.179533883A>G
g.179564748A>G
-
-
NPHS2_000137
-
Bouchireb et al, 2013 (Hum Mutat)
-
-
Germline
-
-
-
-
-
Olivier Gribouval
+?/?
2
c.320T>C
r.(?)
p.(Leu107Pro)
-
likely pathogenic
g.179533883T>C
g.179564748A>G
-
-
NPHS2_000137
Variant Error [EBUILDMISMATCH]: This variant seems to mismatch; the genomic variants on hg19 and hg38 seem to not belong together. Please fix this entry and then remove this message.
PubMed: Bullich 2015
,
Journal: Bullich 2015
-
-
Germline
-
-
-
-
-
Elisabet Ars Criach
?/?
2
c.322A>G
r.(?)
p.(Ile108Val)
-
VUS
g.179533881T>C
g.179564746T>C
-
-
NPHS2_000136
-
Lipska et al, 2013 (J Appl Genetics)
-
-
Germline
-
-
-
-
-
Olivier Gribouval
+/?
2
c.344T>C
r.(?)
p.(Met115Thr)
-
pathogenic
g.179533859A>G
g.179564724A>G
-
-
NPHS2_000135
-
Berdeli et al, 2007 (Pediatr Nephrol)
-
-
Germline
-
-
-
-
-
Olivier Gribouval
+/?
2
c.344T>C
r.(?)
p.(Met115Thr)
-
pathogenic
g.179533859A>G
g.179564724A>G
-
-
NPHS2_000135
-
Berdeli et al, 2007 (Pediatr Nephrol)
-
-
Germline
-
-
-
-
-
Olivier Gribouval
+/?
2
c.346A>C
r.(?)
p.(Thr116Pro)
-
pathogenic
g.179533857T>G
g.179564722T>G
-
-
NPHS2_000134
-
Santin et al, 2011 (Clin J Am Soc Nephrol)
-
-
Germline
-
-
-
-
-
Olivier Gribouval
+/?
2
c.346A>C
r.(?)
p.(Thr116Pro)
-
pathogenic
g.179533857T>G
g.179564722T>G
-
-
NPHS2_000134
-
Santin et al, 2011 (Clin J Am Soc Nephrol)
-
-
Germline
-
-
-
-
-
Olivier Gribouval
+/?
2
c.346A>C
r.(?)
p.(Thr116Pro)
-
pathogenic
g.179533857T>G
g.179564722T>G
-
-
NPHS2_000134
-
Santin et al, 2011 (Clin J Am Soc Nephrol)
-
-
Germline
-
-
-
-
-
Olivier Gribouval
+/?
2
c.353C>T
r.(?)
p.(Pro118Leu)
-
pathogenic
g.179533850G>A
g.179564715G>A
-
-
NPHS2_000133
-
Weber et al, 2004 (Kid Int)
-
-
Germline
-
-
-
-
-
Olivier Gribouval
+/?
2
c.353C>T
r.(?)
p.(Pro118Leu)
-
pathogenic
g.179533850G>A
g.179564715G>A
-
-
NPHS2_000133
-
Weber et al, 2004 (Kid Int)
-
-
Germline
-
-
-
-
-
Olivier Gribouval
+/?
2
c.353C>T
r.(?)
p.(Pro118Leu)
-
pathogenic
g.179533850G>A
g.179564715G>A
-
-
NPHS2_000133
-
Ruf et al, 2004 (J Am Soc Nephrol)
-
-
Germline
-
-
-
-
-
Olivier Gribouval
+/?
2
c.353C>T
r.(?)
p.(Pro118Leu)
-
pathogenic
g.179533850G>A
g.179564715G>A
-
-
NPHS2_000133
-
Ruf et al, 2004 (J Am Soc Nephrol)
-
-
Germline
-
-
-
-
-
Olivier Gribouval
+/?
2
c.353C>T
r.(?)
p.(Pro118Leu)
-
pathogenic
g.179533850G>A
g.179564715G>A
-
-
NPHS2_000133
-
Ruf et al, 2004 (J Am Soc Nephrol)
-
-
Germline
-
-
-
-
-
Olivier Gribouval
+/?
2
c.353C>T
r.(?)
p.(Pro118Leu)
-
pathogenic
g.179533850G>A
g.179564715G>A
-
-
NPHS2_000133
-
Ekim et al, 2004 (Am J Kid Dis)
-
-
Germline
-
-
-
-
-
Olivier Gribouval
+/?
2
c.353C>T
r.(?)
p.(Pro118Leu)
-
pathogenic
g.179533850G>A
g.179564715G>A
-
-
NPHS2_000133
-
Ekim et al, 2004 (Am J Kid Dis)
-
-
Germline
-
-
-
-
-
Olivier Gribouval
+/?
2
c.353C>T
r.(?)
p.(Pro118Leu)
-
pathogenic
g.179533850G>A
g.179564715G>A
-
-
NPHS2_000133
-
Ekim et al, 2004 (Am J Kid Dis)
-
-
Germline
-
-
-
-
-
Olivier Gribouval
+/?
2
c.353C>T
r.(?)
p.(Pro118Leu)
-
pathogenic
g.179533850G>A
g.179564715G>A
-
-
NPHS2_000133
-
Ozcakar et al, 2006 (Pediatr Nephrol)
-
-
Germline
-
-
-
-
-
Olivier Gribouval
+/?
2
c.353C>T
r.(?)
p.(Pro118Leu)
-
pathogenic
g.179533850G>A
g.179564715G>A
-
-
NPHS2_000133
-
Ozcakar et al, 2006 (Pediatr Nephrol)
-
-
Germline
-
-
-
-
-
Olivier Gribouval
+/?
2
c.353C>T
r.(?)
p.(Pro118Leu)
-
pathogenic
g.179533850G>A
g.179564715G>A
-
-
NPHS2_000133
-
Hinkes et al, 2007 (Pediatrics)
-
-
Germline
-
-
-
-
-
Olivier Gribouval
+/?
2
c.353C>T
r.(?)
p.(Pro118Leu)
-
pathogenic
g.179533850G>A
g.179564715G>A
-
-
NPHS2_000133
-
Berdeli et al, 2007 (Pediatr Nephrol)
-
-
Germline
-
-
-
-
-
Olivier Gribouval
+/?
2
c.353C>T
r.(?)
p.(Pro118Leu)
-
pathogenic
g.179533850G>A
g.179564715G>A
-
-
NPHS2_000133
-
Berdeli et al, 2007 (Pediatr Nephrol)
-
-
Germline
-
-
-
-
-
Olivier Gribouval
+/?
2
c.353C>T
r.(?)
p.(Pro118Leu)
-
pathogenic
g.179533850G>A
g.179564715G>A
-
-
NPHS2_000133
-
Berdeli et al, 2007 (Pediatr Nephrol)
-
-
Germline
-
-
-
-
-
Olivier Gribouval
+/?
2
c.353C>T
r.(?)
p.(Pro118Leu)
-
pathogenic
g.179533850G>A
g.179564715G>A
-
-
NPHS2_000133
-
Berdeli et al, 2007 (Pediatr Nephrol)
-
-
Germline
-
-
-
-
-
Olivier Gribouval
+/?
2
c.353C>T
r.(?)
p.(Pro118Leu)
-
pathogenic
g.179533850G>A
g.179564715G>A
-
-
NPHS2_000133
-
Ozaltin et al, 2008 (Pediatr Nephrol)
-
-
Germline
-
-
-
-
-
Olivier Gribouval
+/?
2
c.353C>T
r.(?)
p.(Pro118Leu)
-
pathogenic
g.179533850G>A
g.179564715G>A
-
-
NPHS2_000133
-
Ozaltin et al, 2008 (Pediatr Nephrol)
-
-
Germline
-
-
-
-
-
Olivier Gribouval
+/?
2
c.353C>T
r.(?)
p.(Pro118Leu)
-
pathogenic
g.179533850G>A
g.179564715G>A
-
-
NPHS2_000133
-
Hinkes et al, 2008 (JASN)
-
-
Germline
-
-
-
-
-
Olivier Gribouval
+/?
2
c.353C>T
r.(?)
p.(Pro118Leu)
-
pathogenic
g.179533850G>A
g.179564715G>A
-
-
NPHS2_000133
-
Hinkes et al, 2008 (JASN)
-
-
Germline
-
-
-
-
-
Olivier Gribouval
+/?
2
c.353C>T
r.(?)
p.(Pro118Leu)
-
pathogenic
g.179533850G>A
g.179564715G>A
-
-
NPHS2_000133
-
Hinkes et al, 2008 (JASN)
-
-
Germline
-
-
-
-
-
Olivier Gribouval
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