Global Variome shared LOVD
MLC1 (megalencephalic leukoencephalopathy with subc...)
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Unique variants in the MLC1 gene
The variants shown are described using the NM_015166.3 transcript reference sequence.
Legend
Please note that a short description of a certain column can be displayed when you move your mouse cursor over the column's header and hold it still. Below, a more detailed description is shown per column.
Effect
: The variant's effect on the function of the gene/protein, displayed in the format 'R/C'. R is the value reported by the source (publication, submitter) and this classification may vary between records. C is the value concluded by the curator. Note that in some database the curator uses Summary records to give details on the classification of the variant.Values used: '+' indicating the variant affects function, '+?' probably affects function, '-' does not affect function, '-?' probably does not affect function, '?' effect unknown, '.' effect was not classified.
Reported
: The number of times this variant has been reported in the database.
Exon
: number of exon/intron containing variant; 2 = exon 2, 12i = intron 12, 2i_7i = from intron 2 to intron 7, 8i_9 = intron 8/exon 9 boundary, _1 = 5' to exon 1, 18_ = 3' of exon 18, _1_18_ = encompassing the entire 18-exon gene
DNA change (cDNA)
: description of variant at DNA level, based on a coding DNA reference sequence (following HGVS recommendations); e.g. c.123C>T, c.123_145del, c.123_126dup. For deletions/duplications extending beyond the reference transcript resp. {0}/{2} is used to replace del/dup. Extent of the deletion/duplication should be specified using the genomic description (g.). "-" indicates the variant described on genomic level does not affect the coding DNA reference sequence.
RNA change
: description of variant at RNA level (following HGVS recommendations).
r.123c>u
r.? = unknown
r.(?) = RNA not analysed but probably transcribed copy of DNA variant
r.spl? = RNA not analysed but variant probably affects splicing
r.(spl?) = RNA not analysed but variant may affect splicing
r.0? = change expected to abolish transcription
Protein
: description of variant at protein level (following HGVS recommendations).
p.(Arg345Pro) = change predicted from DNA (RNA not analysed)
p.Arg345Pro = change derived from RNA analysis
p.? = unknown effect
p.0? = probably no protein produced
Classification method
: The method used for the clinical classification of this variant.
All options:
ACMG
ACGS
EAHAD-CFDB
ENIGMA
IARC
InSiGHT
kConFab
other
Clinical classification
: Clinical classification of variant, preferably based on standardised criteria (e.g. ACMG), directed on the clinical consequences as published/submitted, indicated using an enriched system including inheritance: e.g. pathogenic, pathogenic (dominant), pathogenic (recessive), pathogenic (!), pathogenic (maternal), pathogenic (paternal). Standard inheritance is covered by dominant/recessive, imprinting by maternal/paternal. A '!' warns for exceptional circumstances to be explained in the 'Remarks' field (low penetrance, variants pathogenic in heterozygous state only, hypomorphic/hypermorphic variants, protective variants, etc.). Non-disease consequences (e.g. drug metabolism (pharmacogenetics), risk factor, blood group, tasting bitter) are indicated using additions to the benign classification; benign (dominant), benign (recessive), benign (!), etc. The value 'association' is used for variants associated with a phenotype and 'NA' for variants from in vitro/in silico records. NOTE: classification may differ from the opinion of the curator as given in a variant SUMMARY-record or the 'Functional effect concluded'). NOTE: pathogenic/likely pathogenic should go together with "variant (probably) affects function" In ClassFunctional.
All options:
pathogenic
pathogenic (dominant)
pathogenic (recessive)
pathogenic (!)
pathogenic (maternal)
pathogenic (paternal)
likely pathogenic
likely pathogenic (dominant)
likely pathogenic (recessive)
likely pathogenic (!)
likely pathogenic (maternal)
likely pathogenic (paternal)
VUS
VUS (!)
likely benign
likely benign (dominant)
likely benign (recessive)
likely benign (!)
likely benign (maternal)
likely benign (paternal)
benign
benign (dominant)
benign (recessive)
benign (!)
benign (maternal)
benign (paternal)
conflicting
association
NA
DNA change (genomic) (hg19)
: HGVS description of variant at DNA level, based on the genomic (chromosomal) DNA reference sequence; e.g. g.12345678C>T, g.12345679del, g.12345678_12345890dup
DNA change (hg38)
: HGVS description of variant at DNA level, based on the hg38 genomic (chromosomal) eference sequence; e.g. g.12345678C>T, g.12345679del, g.12345678_12345890dup
Published as
: listed only when different from "DNA change"; variant as reported originally (e.g. 521delT). Variants seen in animal models, tested in vitro, predicted from RNA analysis, etc. are described between brackets like c.(456C>G)
ISCN
: description of the variant according to ISCN nomenclature
DB-ID
: database ID of variant, grouping multiple observations of the same variant together, starting with the HGNC gene symbol, followed by an underscore (_) and a six digit number (e.g. DMD_012345). _000000 is used for variants where DNA was not analysed (change predicted from RNA analysis), variants seen in animal models or variants not seen in humans but functionally tested in vitro
Variant remarks
: remarks regarding variant described, e.g. germline mosaicism in mother, 345 kb deletion, muscle RNA analysed, not in 200 control chromosomes tested, on founder haplotype, etc.
Reference
: publication describing the variant submitted, incl. links to OMIM, PubMed or other source, e.g. "den Dunnen ASHG2003 P2346"
ClinVar ID
: ID of variant in ClinVar database
dbSNP ID
: the dbSNP ID
Origin
: Origin of variant/record: Germline = in all cells, De novo = in all cells, but not in either parent, Germline/De novo (untested) = in all cells, parents not tested (use only when De novo is likely, e.g. isolated/sporadic cases with dominant disease), Somatic = present in a subset of cells, but not in either parent, Uniparental disomy = from parental disomy (maternal or paternal), CLASSIFICATION record = submitter only sharing variant classification (note another report may share Individual data), SUMMARY record = master summary record from curator (may link to another database), In vitro (cloned) = data resulting from in vitro functional assays, animal model = data from animal model, Artefact = false positive variant call, DUPLICATE record = variant already described on another chromosome (e.g. unbalanced translocation, duplicating transposition, 2nd fusion transcript, etc.)
All options:
Germline
De novo
Germline/De novo (untested)
Somatic
Uniparental disomy
Uniparental disomy, maternal allele
Uniparental disomy, paternal allele
CLASSIFICATION record
SUMMARY record
In vitro (cloned)
In silico
animal model
Artefact
DUPLICATE record
Unknown
Not applicable
Segregation
: Indicates whether the variant segregates with the phenotype (yes), does not segregate with the phenotype (no) or segregation is unknown (?)
All options:
? = unknown
yes = segregates with phenotype
no = does not segregate with phenotype
- = not applicable
Frequency
: frequency in which the variant was found; e.g 5/760 chromosomes (in 5 of 760 chromosomes tested), 1/33 patients (in 1 of 33 patients analysed in study), 0.05 controls (in 5% of control cases tested)
Re-site
: restriction enzyme recognition site created (+) or destroyed (-); e.g. BglII+;BamHI-
VIP
: variant VIP-status was requested for matchmaking - need collaboration(s) to crack the case - please contact the submitter/curator. NOTE: to get VIP status ask the curator.
Methylation
: result of methylation test; GOM (gain of methylation), LOM (loss of methylation), 30% (30% methylated). NOTE: when several tests were done mention the method as well (e.g. MS-PCR 75%)
How to query this table
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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'
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.
203 entries on 3 pages. Showing entries 1 - 100.
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Effect
Reported
Exon
DNA change (cDNA)
RNA change
Protein
Classification method
Clinical classification
DNA change (genomic) (hg19)
DNA change (hg38)
Published as
ISCN
DB-ID
Variant remarks
Reference
ClinVar ID
dbSNP ID
Origin
Segregation
Frequency
Re-site
VIP
Methylation
Owner
+/.
3
10i_12_
c.(894+1_895-1)_*2192{0}
r.?
p.?
-
pathogenic, pathogenic (recessive)
g.(?_50497820)_(50502628_50506861)del
g.(?_50059391)_(50064199_50068432)del
del ex11-12
-
MLC1_000075
-
-
-
-
Germline, SUMMARY record
-
-
-
-
-
Rogier Min
+/.
1
-
c.-1143062_*3317068del
r.0?
p.0?
-
pathogenic
g.47182944_51666786del
-
-
-
ALG12_000022
mosaicism, hemizygous in 0.56 cells
PubMed: DDDS 2015
,
Journal: DDDS 2015
-
-
Somatic
-
-
-
-
-
Johan den Dunnen
-?/.
1
-
c.-4806C>A
r.(?)
p.(=)
-
likely benign
g.50528530G>T
g.50090101G>T
MOV10L1(NM_018995.3):c.13G>T (p.A5S)
-
MLC1_000039
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+?/.
3
1
c.-195T>C
r.195u>c, r.?
p.(=), p.?
-
likely pathogenic, likely pathogenic (recessive)
g.50523919A>G
g.50085490A>G
-
-
MLC1_000201
-
-
-
-
Germline, SUMMARY record
-
-
-
-
-
Rogier Min
+?/.
2
1
c.-190A>G
r.190a>g, r.?
p.(=), p.?
-
likely pathogenic, likely pathogenic (recessive)
g.50523914T>C
g.50085485T>C
-
-
MLC1_000200
-
-
-
-
Germline, SUMMARY record
-
-
-
-
-
Rogier Min
-/.
1
-
c.-156C>T
r.(?)
p.(=)
-
benign
g.50523880G>A
g.50085451G>A
-549C>T
-
MLC1_000199
-
PubMed: Rubie 2003
-
-
Germline
-
2/100 control individuals
-
-
-
Johan den Dunnen
+?/.
5
2
c.-42C>T
r.(?), r.?
p.(=), p.?
-
likely pathogenic, likely pathogenic (recessive)
g.50523373G>A
g.50084944G>A
-
-
MLC1_000198
variant suggested to create upstream translation initiation site
PubMed: Kariminejad 2015
-
-
Germline, SUMMARY record
-
-
-
-
-
Johan den Dunnen
,
Rogier Min
-/.
1
12
c.*733_*885rep[3>1]
r.(=)
p.(=)
-
benign
g.50499129_50499281rep[3>1]
g.50060700_50060852rep[3>1]
-
-
MLC1_000052
3 unit 51bp repeat 3'UTR reduced to 1 unit
PubMed: Boor 2006
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-/.
1
12
c.*733_*885rep[3>2]
r.(=)
p.(=)
-
benign
g.50499129_50499281rep[3>2]
g.50060700_50060852rep[3>2]
-
-
MLC1_000053
3 unit 51bp repeat 3'UTR reduced to 2 units
PubMed: Boor 2006
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
11i_12_
c.1059+568_*2192{0}
r.?
p.?
-
pathogenic (recessive)
g.(?_50497820)_50501896del
g.(?_50059391)_50063467del
g.240706_251959del
-
MLC1_000061
deletion breakpoint can not be determined from data provided
PubMed: Boor 2006
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
-
c.?
r.(?)
p.(Gly206Ter)
-
pathogenic (recessive)
g.?
-
c.597_598delA+33 (Gly206X)
-
MLC1_000000
description variant makes no sense
PubMed: Boor 2006
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
-
c.3G>A
r.(?)
p.(Met1?)
-
pathogenic
g.50523329C>T
g.50084900C>T
-
-
MLC1_000031
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
?/.
1
-
c.40C>G
r.(?)
p.(Arg14Gly)
-
VUS
g.50523292G>C
-
MLC1(NM_015166.4):c.40C>G (p.(Arg14Gly))
-
MLC1_000202
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+/., +?/.
5
2
c.65G>A
r.(?)
p.(Arg22Gln)
-
likely pathogenic, likely pathogenic (recessive), pathogenic (recessive)
g.50523267C>T
g.50084838C>T
-
-
MLC1_000073
-
PubMed: Wang 2011
-
-
Germline, SUMMARY record
-
-
-
-
-
Johan den Dunnen
,
Rogier Min
+/.
2
2
c.67C>T
r.(?)
p.(Gln23Ter)
-
pathogenic, pathogenic (recessive)
g.50523265G>A
g.50084836G>A
-
-
MLC1_000197
-
-
-
-
Germline, SUMMARY record
-
-
-
-
-
Rogier Min
-?/., ?/.
2
-
c.95C>T
r.(?)
p.(Ala32Val)
-
likely benign, VUS
g.50523237G>A
g.50084808G>A
MLC1(NM_015166.3):c.95C>T (p.A32V)
-
MLC1_000037
VKGL data sharing initiative Nederland
PubMed: Wang 2011
,
PubMed: Cao 2016
-
-
CLASSIFICATION record, Germline
-
-
-
-
-
Johan den Dunnen
,
VKGL-NL_Rotterdam
+?/.
2
2
c.109del
r.(?)
p.(Leu37CysfsTer21)
-
likely pathogenic, likely pathogenic (recessive)
g.50523224del
g.50084795del
109delC
-
MLC1_000196
-
-
-
-
Germline, SUMMARY record
-
-
-
-
-
Rogier Min
+/.
4
2
c.111_112dup
r.(?)
p.(Gln38Argfs*21), p.(Gln38ArgfsTer21)
-
pathogenic, pathogenic (recessive)
g.50523220_50523221dup
g.50084791_50084792dup
110_111insGC
-
MLC1_000059
-
PubMed: Boor 2006
-
-
Germline, SUMMARY record
-
-
-
-
-
Johan den Dunnen
,
Rogier Min
+/.
6
2
c.135del
r.(?), r.135del
p.(Cys46AlafsTer12), p.Cys46AlafsTer12
-
pathogenic, pathogenic (recessive)
g.50523202del
g.50084773del
135delC
-
MLC1_000195
-
PubMed: Kariminejad 2015
-
-
Germline, SUMMARY record
-
-
-
-
-
Johan den Dunnen
,
Rogier Min
+/.
111
2
c.135dup
r.(?)
p.(Cys46Leufs*34), p.(Cys46LeufsTer34)
-
pathogenic, pathogenic (recessive)
g.50523202dup
g.50084773dup
134_136insC, 135insC, 320insC
-
MLC1_000046
-
PubMed: Ben-Zeev 2002
,
PubMed: Ganapathy 2019
,
PubMed: Gorospe 2004
,
PubMed: Leegwater 2002
-
-
Germline, SUMMARY record
-
-
-
-
-
Johan den Dunnen
,
Rogier Min
+/.
12
2
c.136del
r.(?)
p.(Cys46AlafsTer12)
-
pathogenic, pathogenic (recessive)
g.50523196del
g.50084767del
136delT
-
MLC1_000194
-
PubMed: Kariminejad 2015
-
-
Germline, SUMMARY record
-
-
-
-
-
Johan den Dunnen
,
Rogier Min
-/.
1
-
c.153G>A
r.(?)
p.(Thr51=)
-
benign
g.50523179C>T
g.50084750C>T
268G>A (T52)
-
MLC1_000193
-
PubMed: Rubie 2003
-
-
Germline
-
1/140 cases periodic catatonia
-
-
-
Johan den Dunnen
+/.
20
2
c.176G>A
-, r.(?)
p.(Gly59Glu)
-
NA, pathogenic, pathogenic (recessive)
g.50523156C>T
g.50084727C>T
-
-
MLC1_000001
intermediate reduction in plasma membrane expression, submitted through SIB;
ExPASy_017438
PubMed: Ben-Zeev 2002
,
PubMed: Duarri
-
-
Germline, In vitro (cloned), SUMMARY record
yes
-
-
-
-
Johan den Dunnen
,
Rogier Min
+?/.
2
2
c.177G>A
r.(?), r.spl?
p.(Gly59=), p.?
-
likely pathogenic, likely pathogenic (recessive)
g.50523155C>T
g.50084726C>T
-
-
MLC1_000191
-
-
-
-
Germline, SUMMARY record
-
-
-
-
-
Rogier Min
+/., +?/.
5
2, 2i
c.177+1del
r.spl
p.?
-
likely pathogenic, likely pathogenic (recessive), pathogenic (recessive)
g.50523158del
g.50084729del
177delG
-
MLC1_000192
-
PubMed: Patrono 2003
-
-
Germline, SUMMARY record
-
-
-
-
-
Johan den Dunnen
,
Rogier Min
+/., +?/.
9
2i
c.177+1G>T
r.spl
p.?
-
likely pathogenic, likely pathogenic (recessive), pathogenic (recessive)
g.50523154C>A
g.50084725C>A
IVS2+1G>T
-
MLC1_000190
-
PubMed: Kariminejad 2015
,
PubMed: Patrono 2003
-
-
Germline, SUMMARY record
-
-
-
-
-
Johan den Dunnen
,
Rogier Min
+/.
10
2i
c.178-10T>A
r.spl
p.?
-
pathogenic, pathogenic (recessive)
g.50521612A>T
g.50083183A>T
-
-
MLC1_000062
-
PubMed: Boor 2006
,
PubMed: Salinas 2020
RCV000020712.5
-
Germline, SUMMARY record
-
-
-
-
-
Johan den Dunnen
,
Rogier Min
+?/.
2
2i
c.178-2A>G
r.spl
p.?
-
likely pathogenic, likely pathogenic (recessive)
g.50521604T>C
g.50083175T>C
-
-
MLC1_000189
-
-
-
-
Germline, SUMMARY record
-
-
-
-
-
Rogier Min
+/.
1
2i_3i
c.(177+1_178-1)_(267+1_268-1)del
r.178_267del
p.Ser60_Ser89del
-
pathogenic (recessive)
g.(50518827_50521512)_(50521603_50523154 )del
g.(50080398_50083083)_(50083174_50084725)del
del ex3
-
MLC1_000179
-
-
-
-
Germline
-
-
-
-
-
Rogier Min
?/.
2
3
c.184C>T
r.(?)
p.(Leu62Phe)
-
VUS
g.50521596G>A
g.50083167G>A
-
-
MLC1_000188
-
-
-
-
Germline, SUMMARY record
-
-
-
-
-
Rogier Min
+/.
14
3
c.206C>T
r.(?)
p.(Ser69Leu), p.Ser69Leu
-
pathogenic, pathogenic (recessive)
g.50521574G>A
g.50083145G>A
-
-
MLC1_000002
-
PubMed: Boor 2006
,
PubMed: Wang 2011
-
-
Germline, SUMMARY record
-
-
-
-
-
Johan den Dunnen
,
Rogier Min
+/., +?/.
4
3
c.213C>G
r.(?)
p.(Tyr71Ter)
-
likely pathogenic, likely pathogenic (recessive), pathogenic (recessive)
g.50521567G>C
g.50083138G>C
328C>G
-
MLC1_000187
-
PubMed: Leegwater 2001
-
-
Germline, SUMMARY record
-
-
-
-
-
Johan den Dunnen
,
Rogier Min
+?/.
4
3
c.218G>A
r.(?)
p.(Gly73Glu), p.Gly73Glu
-
likely pathogenic, likely pathogenic (recessive)
g.50521562C>T
g.50083133C>T
-
-
MLC1_000068
-
PubMed: Wang 2011
-
-
Germline, SUMMARY record
-
-
-
-
-
Rogier Min
?/.
2
3
c.235G>A
r.(?)
p.(Glu79Lys)
-
VUS
g.50521545C>T
g.50083116C>T
-
-
MLC1_000186
-
-
-
-
Germline, SUMMARY record
-
-
-
-
-
Rogier Min
+?/.
2
3
c.238A>G
r.(?)
p.(Met80Val)
-
likely pathogenic, likely pathogenic (recessive)
g.50521542T>C
g.50083113T>C
-
-
MLC1_000185
-
-
-
-
Germline, SUMMARY record
-
-
-
-
-
Rogier Min
+/., +?/.
4
3
c.240G>A
r.(?)
p.(Met80Ile)
-
likely pathogenic, likely pathogenic (recessive), pathogenic
g.50521540C>T
g.50083111C>T
-
-
MLC1_000003
submitted through SIB;
ExPASy_067763
PubMed: Boor 2006
-
-
Germline, SUMMARY record
-
-
-
-
-
SIB - Livia Famiglietti
,
Rogier Min
+/., +?/., ?/.
10
3
c.249G>T
-, r.(?), r.249g>u
p.(Leu83Phe), p.Leu83Phe
-
likely pathogenic (recessive), NA, pathogenic, pathogenic (recessive), VUS
g.50521531C>A
g.50083102C>A
-
-
MLC1_000184
severe reduction in plasma membrane expression, unknown variant 2nd chromosome
PubMed: Duarri
,
PubMed: Leegwater 2002
,
PubMed: Patrono 2003
-
-
Germline, In vitro (cloned), SUMMARY record
-
-
-
-
-
Johan den Dunnen
,
Rogier Min
+/.
4
3
c.250C>A
r.(?)
p.(Arg84Ser)
-
pathogenic, pathogenic (recessive)
g.50521530G>T
g.50083101G>T
-
-
MLC1_000183
-
PubMed: Kariminejad 2015
-
-
Germline, SUMMARY record
-
-
-
-
-
Johan den Dunnen
,
Rogier Min
+/.
7
3
c.250C>T
r.(?)
p.(Arg84Cys)
-
pathogenic, pathogenic (recessive)
g.50521530G>A
g.50083101G>A
-
-
MLC1_000004
submitted through SIB;
ExPASy_067764
PubMed: Boor 2006
-
-
Germline, SUMMARY record
-
-
-
-
-
SIB - Livia Famiglietti
,
Rogier Min
+/.
6
3
c.251G>A
r.(?)
p.(Arg84His)
-
pathogenic, pathogenic (recessive)
g.50521529C>T
g.50083100C>T
-
-
MLC1_000182
-
-
-
-
Germline, SUMMARY record
-
-
-
-
-
Rogier Min
+?/.
2
3
c.254G>A
r.(?)
p.(Cys85Tyr)
-
likely pathogenic, likely pathogenic (recessive)
g.50521526C>T
g.50083097C>T
-
-
MLC1_000181
-
-
-
-
Germline, SUMMARY record
-
-
-
-
-
Rogier Min
+/., +?/., ?/.
4
3
c.255T>G
-, r.(?)
p.(Cys85Trp)
-
likely pathogenic, NA, pathogenic (recessive), VUS
g.50521525A>C
g.50083096A>C
-
-
MLC1_000180
severe reduction in plasma membrane expression
PubMed: Duarri
,
PubMed: Leegwater 2002
-
-
Germline, In vitro (cloned), SUMMARY record
-
-
-
-
-
Johan den Dunnen
,
Rogier Min
+/., +?/., ?/.
7
3
c.263G>T
-, r.(?)
p.(Gly88Val)
-
likely pathogenic, NA, pathogenic (recessive), VUS
g.50521517C>A
g.50083088C>A
-
-
MLC1_000057
severe reduction in plasma membrane expression
PubMed: Duarri
,
PubMed: Koussa 2005
,
PubMed: Patrono 2003
-
-
Germline, In vitro (cloned), SUMMARY record
yes
-
-
-
-
Johan den Dunnen
,
Rogier Min
+?/.
2
3i
c.267+1G>C
r.spl
p.?
-
likely pathogenic, likely pathogenic (recessive)
g.50521512C>G
g.50083083C>G
-
-
MLC1_000178
-
-
-
-
Germline, SUMMARY record
-
-
-
-
-
Rogier Min
+/.
4
3i_5i, 4
c.268-945_423+725del
r.(268_423del), r.(?)
p.(Cys90HisfsTer39), p.(Cys90_Asn141del)
-
pathogenic, pathogenic (recessive)
g.50517626_50519775del
g.50079197_50081346del
del ex4-5, del ex4-5 (del2149bp)
-
MLC1_000156
-
PubMed: Leegwater 2002
-
-
Germline, SUMMARY record
-
-
-
-
-
Johan den Dunnen
,
Rogier Min
+/.
3
3i
c.268-23A>G
r.spl
p.?
-
pathogenic, pathogenic (recessive)
g.50518849T>C
g.50080420T>C
-
-
MLC1_000177
-
-
-
-
Germline, SUMMARY record
-
-
-
-
-
Rogier Min
+/.
1
-
c.268-1G>A
r.268_321del
p.Cys90_Val107del
-
pathogenic (recessive)
g.50518827C>T
g.50080398C>T
-
-
MLC1_000063
-
PubMed: Boor 2006
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
2
3i_9i
c.(267+1_268-1)_(771+1_772-1)del
r.?
p.?
-
pathogenic, pathogenic (recessive)
g.(50506985_50508955)_(50518827_50521512)del
g.(50068556_50070526)_(50080398_50083083)del
del ex 4-9, del ex4-9
-
MLC1_000117
-
-
-
-
Germline, SUMMARY record
-
-
-
-
-
Rogier Min
+/., ?/.
4
4
c.268T>G
r.(?)
p.(Cys90Gly)
-
pathogenic (recessive), VUS
g.50518826A>C
g.50080397A>C
-
-
MLC1_000176
-
PubMed: Kariminejad 2015
-
-
Germline, SUMMARY record
-
-
-
-
-
Johan den Dunnen
,
Rogier Min
?/.
2
3i_5i
c.(267+1_268+1)_(423+1_424-1)del
r.(?)
p.(Cys90_Asn141del), p.Cys90_Asn141del
-
VUS
g.(50515932_50518346)_(50518827_50521512)del
g.(50077503_50079917)_(50080398_50083083)del
del ex4-5 (268_423del)
-
MLC1_000161
-
-
-
-
Germline
-
-
-
-
-
Rogier Min
+/.
29
4
c.274C>T
-, r.(?), r.274c>u
p.(Pro92Ser), p.Pro92Ser
-
NA, pathogenic, pathogenic (recessive)
g.50518820G>A
g.50080391G>A
-
-
MLC1_000007
intermediate reduction in plasma membrane expression, unknown variant 2nd chromosome,
ExPASy_017439
PubMed: Ben-Zeev 2002
,
PubMed: Capdevila-Nortes 2013
,
PubMed: Duarri
,
PubMed: Leegwater 2002
-
-
Germline, In vitro (cloned), SUMMARY record
-
-
-
-
-
Johan den Dunnen
,
Rogier Min
+?/., ?/.
3
4
c.278C>G
-, r.(?)
p.(Ser93Trp)
-
NA, VUS
g.50518816G>C
g.50080387G>C
-
-
MLC1_000175
severe reduction in plasma membrane expression
PubMed: Duarri
-
-
Germline, In vitro (cloned), SUMMARY record
-
-
-
-
-
Rogier Min
+/.
40
4
c.278C>T
-, r.(?), r.278c>u
p.(Ser93Leu), p.Ser93Leu
-
NA, pathogenic, pathogenic (recessive)
g.50518816G>A
g.50080387G>A
393C>T
-
MLC1_000012
severe reduction in plasma membrane expression,
ExPASy_011699
PubMed: Duarri
,
PubMed: Kariminejad 2015
,
PubMed: Leegwater 2001
,
PubMed: Saijo 2003
,
1 more item
-
-
Germline, In vitro (cloned), SUMMARY record
-
-
-
-
-
Johan den Dunnen
,
Rogier Min
-/., -?/.
2
-
c.279G>A
r.(?)
p.(Ser93=)
-
benign, likely benign
g.50518815C>T
g.50080386C>T
MLC1(NM_015166.3):c.279G>A (p.S93=), MLC1(NM_015166.4):c.279G>A (p.(Ser93=))
-
MLC1_000030
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
,
VKGL-NL_Rotterdam
+/.
7
3i_5i, 4_5i
c.298_423+108del
r.(268_423del), r.268_423del, r.?
p.(Cys90_Asn141del), p.(Val100AspfsTer73), p.Cys90_Asn141del
-
pathogenic, pathogenic (recessive)
g.50518239_50518796del
g.50079810_50080367del
-
-
MLC1_000060
-
PubMed: Boor 2006
-
-
Germline, SUMMARY record
-
-
-
-
-
Johan den Dunnen
,
Rogier Min
+?/.
2
4i
c.321+2T>G
r.spl
p.?
-
likely pathogenic, likely pathogenic (recessive)
g.50518771A>C
g.50080342A>C
-
-
MLC1_000174
-
-
-
-
Germline, SUMMARY record
-
-
-
-
-
Rogier Min
?/.
2
4i
c.321+6T>G
r.spl?
p.?
-
VUS
g.50518767A>C
g.50080338A>C
-
-
MLC1_000173
-
-
-
-
Germline, SUMMARY record
-
-
-
-
-
Rogier Min
-/.
1
-
c.321+50T>C
r.(?)
p.(=)
-
benign
g.50518723A>G
g.50080294A>G
IVS4+50C>T
-
MLC1_000172
-
PubMed: Rubie 2003
-
-
Germline
-
48/55 control individuals
-
-
-
Johan den Dunnen
+?/., ?/.
4
4i
c.322-6T>C
r.(?), r.spl, r.spl?
p.?
-
likely pathogenic (recessive), VUS
g.50518454A>G
g.50080025A>G
-
-
MLC1_000171
-
PubMed: Kariminejad 2015
-
-
Germline, SUMMARY record
-
-
-
-
-
Johan den Dunnen
,
Rogier Min
+?/.
3
4i
c.322-2A>G
r.spl
p.?
-
likely pathogenic, likely pathogenic (recessive)
g.50518450T>C
g.50080021T>C
-
-
MLC1_000170
-
-
-
-
Germline, SUMMARY record
-
-
-
-
-
Rogier Min
+?/.
2
4i
c.322-1G>A
r.spl
p.?
-
likely pathogenic, likely pathogenic (recessive)
g.50518449C>T
g.50080020C>T
-
-
MLC1_000169
-
-
-
-
Germline, SUMMARY record
-
-
-
-
-
Rogier Min
+/.
3
5
c.324del
r.(?)
p.(Asn110ThrfsTer12)
-
pathogenic, pathogenic (recessive)
g.50518447del
g.50080018del
-
-
MLC1_000168
-
-
-
-
Germline, SUMMARY record
-
-
-
-
-
Rogier Min
+/.
3
5
c.337_353delinsG
r.(?), r.337_353delinsg
p.(Ile113GlyfsTer4), p.Ile113Glyfs*4
-
pathogenic, pathogenic (recessive)
g.50518417_50518433delinsC
g.50079988_50080004delinsC
452-468del+g
-
MLC1_000058
-
PubMed: Tsujino 2003
-
-
Germline, SUMMARY record
-
-
-
-
-
Johan den Dunnen
,
Rogier Min
?/.
2
5
c.344T>C
r.(?)
p.(Phe115Ser)
-
VUS
g.50518426A>G
g.50079997A>G
-
-
MLC1_000167
-
-
-
-
Germline, SUMMARY record
-
-
-
-
-
Rogier Min
+/.
13
5
c.353C>G
-, r.(?)
p.(Thr118Arg)
-
NA, pathogenic, pathogenic (recessive)
g.50518417G>C
g.50079988G>C
468C>G
-
MLC1_000013
severe reduction in plasma membrane expression,
ExPASy_011700
PubMed: Duarri
,
PubMed: Leegwater 2001
-
-
Germline, In vitro (cloned), SUMMARY record
-
-
-
-
-
Johan den Dunnen
,
Rogier Min
+/.
16
5
c.353C>T
-, r.(?)
p.(Thr118Met)
-
NA, pathogenic, pathogenic (recessive)
g.50518417G>A
g.50079988G>A
-
-
MLC1_000071
severe reduction in plasma membrane expression
PubMed: Duarri
,
PubMed: Kariminejad 2015
,
PubMed: Leegwater 2002
,
PubMed: Tejido 2004
,
1 more item
-
-
Germline, In vitro (cloned), SUMMARY record
-
-
-
-
-
Johan den Dunnen
,
Rogier Min
+/., +?/.
5
5
c.357dup
r.(?)
p.(Ala120CysfsTer61)
-
likely pathogenic, likely pathogenic (recessive), pathogenic (recessive)
g.50518415dup
g.50079986dup
357insT, 358insT
-
MLC1_000166
-
PubMed: Patrono 2003
-
-
Germline, SUMMARY record
-
-
-
-
-
Johan den Dunnen
,
Rogier Min
?/.
3
5
c.359C>T
r.(?)
p.(Ala120Val)
-
VUS
g.50518411G>A
g.50079982G>A
-
-
MLC1_000165
-
-
-
-
Germline, SUMMARY record
-
-
-
-
-
Rogier Min
-?/.
2
5
c.369T>C
r.(?)
p.(Thr123=)
-
likely benign
g.50518401A>G
g.50079972A>G
-
-
MLC1_000164
-
-
-
-
Germline, SUMMARY record
-
-
-
-
-
Rogier Min
+/., +?/., ?/.
4
5
c.373T>C
-, r.(?)
p.(Cys125Arg)
-
likely pathogenic, NA, pathogenic (recessive), VUS
g.50518397A>G
g.50079968A>G
-
-
MLC1_000008
severe reduction in plasma membrane expression,
1 more item
PubMed: Duarri
,
PubMed: Leegwater 2002
-
-
Germline, In vitro (cloned), SUMMARY record
-
-
-
-
-
Johan den Dunnen
,
Rogier Min
+?/.
2
5
c.387del
r.(?)
p.(Phe129LeufsTer5)
-
likely pathogenic, likely pathogenic (recessive)
g.50518385del
g.50079956del
387delT
-
MLC1_000163
-
-
-
-
Germline, SUMMARY record
-
-
-
-
-
Rogier Min
?/.
2
5
c.388G>A
r.(?)
p.(Gly130Arg)
-
VUS
g.50518382C>T
g.50079953C>T
-
-
MLC1_000162
-
-
-
-
Germline, SUMMARY record
-
-
-
-
-
Rogier Min
+/., +?/., ?/.
5
5
c.422A>G
-, r.(?)
p.(Asn141Ser)
-
likely pathogenic, NA, pathogenic (recessive), VUS
g.50518348T>C
g.50079919T>C
-
-
MLC1_000010
severe reduction in plasma membrane expression,
ExPASy_017441
PubMed: Duarri
,
PubMed: Leegwater 2002
-
-
Germline, In vitro (cloned), SUMMARY record
-
-
-
-
-
Johan den Dunnen
,
Rogier Min
+/., ?/.
7
5
c.423C>A
-, r.(?)
p.(Asn141Lys)
-
NA, pathogenic (recessive), VUS
g.50518347G>T
g.50079918G>T
-
-
MLC1_000009
mild to no reduction in plasma membrane expression,
ExPASy_017440
PubMed: Duarri
,
PubMed: Leegwater 2002
,
PubMed: Patrono 2003
,
PubMed: Xu 2021
-
-
Germline, In vitro (cloned), SUMMARY record
-
-
-
-
-
Johan den Dunnen
,
Rogier Min
+/., +?/.
27
5i
c.423+1G>A
r.(?), r.spl, r.spl?
p.(?), p.?
-
likely pathogenic, likely pathogenic (recessive), pathogenic, pathogenic (recessive)
g.50518346C>T
g.50079917C>T
IVS5+1 A, IVS5+1G>A
-
MLC1_000040
-
PubMed: Bugiani 2003
,
PubMed: Froukh 2020
,
PubMed: Kariminejad 2015
,
PubMed: Leegwater 2002
,
3 more items
RCV001063150.1
-
Germline, SUMMARY record, Unknown
-
-
-
-
-
Johan den Dunnen
,
IMGAG
,
Rogier Min
+?/.
2
5i
c.423+1G>T
r.spl
p.?
-
likely pathogenic, likely pathogenic (recessive)
g.50518346C>A
g.50079917C>A
-
-
MLC1_000160
-
-
-
-
Germline, SUMMARY record
-
-
-
-
-
Rogier Min
+/.
6
5i
c.423+2dup
r.spl
p.?
-
pathogenic, pathogenic (recessive)
g.50518345dup
g.50079916dup
IVS5+3insT
-
MLC1_000159
-
PubMed: Leegwater 2001
-
-
Germline, SUMMARY record
-
-
-
-
-
Johan den Dunnen
,
Rogier Min
?/.
2
5i
c.423+6T>C
r.spl?
p.?
-
VUS
g.50518341A>G
g.50079912A>G
-
-
MLC1_000157
-
-
-
-
Germline, SUMMARY record
-
-
-
-
-
Rogier Min
+/., ?/.
4
5i
c.423+6T>G
r.spl, r.spl?
p.?
-
pathogenic (recessive), VUS
g.50518341A>C
g.50079912A>C
IVS5+6T>G
-
MLC1_000158
-
PubMed: Patrono 2003
-
-
Germline, SUMMARY record
-
-
-
-
-
Johan den Dunnen
,
Rogier Min
+/.
6
5i
c.424-3C>G
r.spl
p.?
-
pathogenic, pathogenic (recessive)
g.50515934G>C
g.50077505G>C
-
-
MLC1_000155
-
-
-
-
Germline, SUMMARY record
-
-
-
-
-
Rogier Min
+?/.
2
5i
c.424-2A>G
r.spl
p.?
-
likely pathogenic, likely pathogenic (recessive)
g.50515933T>C
g.50077504T>C
-
-
MLC1_000154
-
-
-
-
Germline, SUMMARY record
-
-
-
-
-
Rogier Min
?/.
2
5i_7i
c.(423+1_424-1)_(597+1_598-1)del
r.(424_597del), r.(?)
p.(Ile142_Ser199del)
-
VUS
g.(50512762_50515269)_(50515932_50518346)del
g.(50074333_50076840)_(50077503_50079917)del
del ex5-6, del ex5-6 (424_597del)
-
MLC1_000141
-
-
-
-
Germline, SUMMARY record
-
-
-
-
-
Rogier Min
+/.
3
6
c.448del
r.(?)
p.(Leu150SerfsTer11)
-
pathogenic, pathogenic (recessive)
g.50515907del
g.50077478del
-
-
MLC1_000153
-
-
-
-
Germline, SUMMARY record
-
-
-
-
-
Rogier Min
+/.
12
6
c.449_455del
r.(?)
p.(Leu150ArgfsTer9)
-
pathogenic, pathogenic (recessive)
g.50515900_50515906del
g.50077471_50077477del
449_455del (Leu151_Leu152del), 564-570del
-
MLC1_000152
-
PubMed: Kariminejad 2015
,
PubMed: Leegwater 2001
-
-
Germline, SUMMARY record
-
-
-
-
-
Johan den Dunnen
,
Rogier Min
+?/.
2
6
c.452_467del
r.(?), r.452_467del
p.(Leu151ArgfsTer5), p.Leu151ArgfsTer5
-
likely pathogenic, likely pathogenic (recessive)
g.50515888_50515903del
g.50077459_50077474del
-
-
MLC1_000149
-
-
-
-
Germline, SUMMARY record
-
-
-
-
-
Rogier Min
+/., +?/.
3
6
c.456_460dup
r.(?)
p.(Leu154ArgfsTer9)
-
likely pathogenic, likely pathogenic (recessive), pathogenic (recessive)
g.50515895_50515899dup
g.50077466_50077470dup
575insGGAGC
-
MLC1_000150
-
PubMed: Leegwater 2001
,
PubMed: Leegwater 2002
-
-
Germline, SUMMARY record
-
-
-
-
-
Johan den Dunnen
,
Rogier Min
+/., +?/.
4
6
c.459del
r.(?)
p.(Glu153AspfsTer8)
-
likely pathogenic, likely pathogenic (recessive), pathogenic (recessive)
g.50515896del
g.50077467del
459delG, 460delG
-
MLC1_000151
-
PubMed: Kariminejad 2015
-
-
Germline, SUMMARY record
-
-
-
-
-
Johan den Dunnen
,
Rogier Min
+/.
9
6
c.470C>A
-, r.(?)
p.(Ala157Glu)
-
NA, pathogenic, pathogenic (recessive)
g.50515885G>T
g.50077456G>T
585C>A, 660C>A
-
MLC1_000148
intermediate reduction in plasma membrane expression, no variant 2nd chromosome
PubMed: Duarri
,
PubMed: Gorospe 2004
,
PubMed: Rubie 2003
-
-
Germline, In vitro (cloned), SUMMARY record
-
-
-
-
-
Johan den Dunnen
,
Rogier Min
-/., -?/.
4
6
c.512G>T
r.(?)
p.(Cys171Phe)
-
benign, likely benign
g.50515843C>A
g.50077414C>A
627G>T, 702G>T
-
MLC1_000054
polymorphic variant
PubMed: Gorospe 2004
,
PubMed: Leegwater 2002
,
PubMed: Rubie 2003
,
PubMed: Wang 2011
-
-
Germline
-
0.125, 18/100 control individuals
-
-
-
Johan den Dunnen
+/.
2
6
c.514_515del
r.(?)
p.(Lys172GlufsTer8)
-
pathogenic, pathogenic (recessive)
g.50515840_50515841del
g.50077411_50077412del
-
-
MLC1_000147
-
-
-
-
Germline, SUMMARY record
-
-
-
-
-
Rogier Min
-/.
1
-
c.525+21C>T
r.(?)
p.(=)
-
benign
g.50515809G>A
g.50077380G>A
IVS6+22C>T
-
MLC1_000146
-
PubMed: Rubie 2003
-
-
Germline
-
13/55 control individuals
-
-
-
Johan den Dunnen
?/.
1
-
c.526-1G>T
r.spl?
p.?
-
VUS
g.50515342C>A
-
MLC1(NM_015166.4):c.526-1G>T
-
MLC1_000208
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
?/.
1
-
c.545C>A
r.(?)
p.(Ala182Asp)
-
VUS
g.50515322G>T
-
MLC1(NM_015166.4):c.545C>A (p.(Ala182Asp))
-
MLC1_000207
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
?/.
1
-
c.561A>C
r.(?)
p.(Glu187Asp)
-
VUS
g.50515306T>G
g.50076877T>G
MLC1(NM_015166.3):c.561A>C (p.E187D)
-
MLC1_000036
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
?/.
1
-
c.578G>A
r.(?)
p.(Arg193Gln)
-
VUS
g.50515289C>T
g.50076860C>T
MLC1(NM_015166.3):c.578G>A (p.R193Q)
-
MLC1_000035
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
+/.
2
7
c.580del
r.(?)
p.(Val194SerfsTer2)
-
pathogenic, pathogenic (recessive)
g.50515289del
g.50076860del
578delG
-
MLC1_000145
-
-
-
-
Germline, SUMMARY record
-
-
-
-
-
Rogier Min
-/.
6
8, 8i
c.594C>T
r.(?)
p.(Tyr198=)
-
benign
g.50515273G>A
g.50076844G>A
709C>T (Y199)
-
MLC1_000144
-
PubMed: Rubie 2003
-
-
Germline, SUMMARY record
-
29/100 control individuals
-
-
-
Johan den Dunnen
,
Rogier Min
+/.
28
7
c.594_597del
r.(?), r.594_597del
p.(Tyr198*), p.(Tyr198Ter), p.Tyr198Ter
-
pathogenic, pathogenic (recessive)
g.50515271_50515274del
g.50076842_50076845del
594delCTCA, 709_712del, Y198stop
-
MLC1_000074
no variant 2nd chromosome, unknown variant 2nd chromosome
PubMed: Leegwater 2001
,
PubMed: Leegwater 2002
,
PubMed: Wang 2011
-
-
Germline, SUMMARY record
-
-
-
-
-
Johan den Dunnen
,
Rogier Min
?/.
2
7
c.595T>C
r.(?)
p.(Ser199Pro)
-
VUS
g.50515272A>G
g.50076843A>G
-
-
MLC1_000143
-
-
-
-
Germline, SUMMARY record
-
-
-
-
-
Rogier Min
?/.
2
7
c.596C>T
r.(?)
p.(Ser199Leu)
-
VUS
g.50515271G>A
g.50076842G>A
-
-
MLC1_000142
-
-
-
-
Germline, SUMMARY record
-
-
-
-
-
Rogier Min
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