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%)

 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
|
| +/+ |
02c |
c.-43_499+1del |
r.spl? |
p.? |
- |
pathogenic |
g.23282109_23284530del |
g.22812900_22815321del |
197del543 (cDNA): removes the first methionine at position 242 as well as 167 codons downstream; c.-45_499del p.M1_C167del |
- |
SLC7A7_000001 |
1 Italian LPI family (hom) |
PubMed: Bosrsani et al. 1999 |
- |
- |
SUMMARY record |
yes |
- |
- |
- |
- |
Anne Polvi |
| +/+ |
03 |
c.1A>C |
r.(1a>c) |
p.(Met1?) |
- |
pathogenic |
g.23282607T>G |
g.22813398T>G |
242A>C: M1L |
- |
SLC7A7_000002 |
1 Italian (Puglia) LPI family (hom) |
PubMed: Sperandeo et al. 2000 |
- |
rs121908676 |
SUMMARY record |
yes |
- |
- |
- |
- |
Anne Polvi |
| +?/+? |
03 |
c.14C>T |
r.(14c>u) |
p.(Thr5Ile) |
- |
likely pathogenic |
g.23282594G>A |
g.22813385G>A |
- |
- |
SLC7A7_000003 |
1 Italian LPI family (hom); Variants c.14C>T and c.158C>T segregate together so it is unclear which is (or both are) causative |
PubMed: Sperandeo et al. 2008 |
- |
- |
SUMMARY record |
yes |
- |
- |
- |
- |
Anne Polvi |
| -?/. |
- |
c.84G>A |
r.(?) |
p.(Pro28=) |
- |
likely benign |
g.23282524C>T |
- |
SLC7A7(NM_001126106.2):c.84G>A (p.P28=) |
- |
SLC7A7_000092 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Rotterdam |
| -/. |
- |
c.96G>A |
r.(?) |
p.(Lys32=) |
- |
benign |
g.23282512C>T |
g.22813303C>T |
SLC7A7(NM_001126106.4):c.96G>A (p.K32=) |
- |
SLC7A7_000075 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_AMC |
| +/+ |
03 |
c.106_108del |
r.(?) |
p.(Glu36del) |
- |
pathogenic |
g.23282502_23282504del |
g.22813293_22813295del |
deletion of nucleotides 104–106 from the first methionine of the SLC7A7 cDNA: E36del; c.104_106delGGA p.E36del |
- |
SLC7A7_000004 |
1 Greek LPI patient (com-het); Functional studies: mutated protein failed to show transport activity |
PubMed: Sperandeo et al. 2005 |
- |
- |
SUMMARY record |
yes |
- |
- |
- |
- |
Anne Polvi |
| +/+ |
03 |
c.149T>A |
r.149u>a |
p.(Met50Lys) |
- |
pathogenic |
g.23282459A>T |
g.22813250A>T |
435T>A: M50K; p.M50K (c.149T>A) |
- |
SLC7A7_000005 |
1 Italian LPI family (het); Functional studies confirmed disease-causative role |
PubMed: Palacín et al. 2001, PubMed: Sperandeo et al. 2005 |
- |
- |
SUMMARY record |
yes |
- |
- |
- |
- |
Anne Polvi |
| +?/+? |
03 |
c.158C>T |
r.(158c>u) |
p.(Ser53Leu) |
- |
likely pathogenic |
g.23282450G>A |
g.22813241G>A |
- |
- |
SLC7A7_000052 |
1 Italian LPI family (hom); Variants c.14C>T and c.158C>T segregate together so it is unclear which is (or both are) causative |
PubMed: Sperandeo et al. 2008 |
- |
- |
SUMMARY record |
yes |
- |
- |
- |
- |
Anne Polvi |
| -/. |
- |
c.159G>A |
r.(?) |
p.(Ser53=) |
- |
benign |
g.23282449C>T |
g.22813240C>T |
SLC7A7(NM_001126106.4):c.159G>A (p.S53=) |
- |
SLC7A7_000061 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Groningen |
| -/. |
- |
c.159G>A |
r.(?) |
p.(Ser53=) |
- |
benign |
g.23282449C>T |
g.22813240C>T |
SLC7A7(NM_001126106.4):c.159G>A (p.S53=) |
- |
SLC7A7_000061 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Nijmegen |
| -/. |
- |
c.159G>A |
r.(?) |
p.(Ser53=) |
- |
benign |
g.23282449C>T |
g.22813240C>T |
SLC7A7(NM_001126106.4):c.159G>A (p.S53=) |
- |
SLC7A7_000061 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_AMC |
| +/+ |
03 |
c.161G>T |
r.161g>u |
p.(Gly54Val) |
- |
pathogenic |
g.23282447C>A |
g.22813238C>A |
447G>T (exon 3): G54V |
- |
SLC7A7_000006 |
1 Latvian and 1 Estonian (both hom) LPI family; Functional analysis: loss of function |
PubMed: Mykkänen et al. 2000 |
- |
rs121908677 |
SUMMARY record |
yes |
- |
- |
- |
- |
Anne Polvi |
| +/. |
- |
c.161G>T |
r.(?) |
p.(Gly54Val) |
- |
pathogenic |
g.23282447C>A |
g.22813238C>A |
- |
- |
SLC7A7_000006 |
15 heterozygous, no homozygous; Clinindb (India) |
PubMed: Narang 2020, Journal: Narang 2020 |
- |
rs121908677 |
Germline |
- |
15/2655 individuals |
- |
- |
- |
Mohammed Faruq |
| +?/. |
- |
c.173C>A |
r.(?) |
p.(Ser58Tyr) |
- |
likely pathogenic |
g.23282435G>T |
- |
SLC7A7(NM_001126106.4):c.173C>A (p.S58Y) |
- |
SLC7A7_000074 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_VUmc |
| +/+ |
03 |
c.215_218del |
r.(?) |
p.(Ser72Trpfs*97) |
- |
pathogenic |
g.23282395_23282398del |
g.22813186_22813189del |
455delCTCT: Frameshift after Leu71, termination at codon 167; c.211_214delCTCT p.S72WfsX97 |
- |
SLC7A7_000009 |
1 Italian (Molise) LPI family (hom) |
PubMed: Sperandeo et al. 2000 |
- |
- |
SUMMARY record |
yes |
- |
- |
- |
- |
Anne Polvi |
| +?/? |
03 |
c.220_222dup |
r.(?) |
p.(Val74dup) |
- |
likely pathogenic |
g.23282386_23282388dup |
g.22813177_22813179dup |
c.223insGTC, p.Val74_Ile75insVal |
- |
SLC7A7_000054 |
2 Turkish patients (1 hom and 1 het) with LPI |
PubMed: Güzel-Ozantürk et al. 2013 |
- |
- |
SUMMARY record |
yes |
- |
- |
- |
- |
Anne Polvi |
| -/. |
- |
c.234C>T |
r.(?) |
p.(Val78=) |
- |
benign |
g.23282374G>A |
- |
SLC7A7(NM_001126106.2):c.234C>T (p.V78=), SLC7A7(NM_001126106.4):c.234C>T (p.V78=) |
- |
SLC7A7_000091 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_AMC |
| -?/. |
- |
c.234C>T |
r.(?) |
p.(Val78=) |
- |
likely benign |
g.23282374G>A |
- |
SLC7A7(NM_001126106.2):c.234C>T (p.V78=), SLC7A7(NM_001126106.4):c.234C>T (p.V78=) |
- |
SLC7A7_000091 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Rotterdam |
| -/. |
- |
c.234C>T |
r.(?) |
p.(Val78=) |
- |
benign |
g.23282374G>A |
- |
SLC7A7(NM_001126106.2):c.234C>T (p.V78=), SLC7A7(NM_001126106.4):c.234C>T (p.V78=) |
- |
SLC7A7_000091 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Utrecht |
| +?/+? |
03 |
c.235G>A |
r.(235g>a) |
p.(Gly79Arg) |
- |
likely pathogenic |
g.23282373C>T |
g.22813164C>T |
235G>A |
- |
SLC7A7_000053 |
1 Malaysian LPI family (hom) |
PubMed: Habib et al. 2013 |
- |
- |
SUMMARY record |
yes |
- |
- |
- |
- |
Anne Polvi |
| -?/. |
- |
c.250G>A |
r.(?) |
p.(Val84Ile) |
- |
likely benign |
g.23282358C>T |
- |
SLC7A7(NM_001126106.2):c.250G>A (p.V84I) |
- |
SLC7A7_000095 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Utrecht |
| +/+ |
03 |
c.254_255del |
r.254_255deluu |
p.(Phe85Trpfs*13) |
- |
pathogenic |
g.23282354_23282355del |
g.22813145_22813146del |
539delTT (exon 3) Frameshift after V84; c.253_254delTT p.F85WfsX13 |
- |
SLC7A7_000010 |
1 Turkish LPI family (hom) |
PubMed: Mykkänen et al. 2000 |
- |
- |
SUMMARY record |
yes |
- |
- |
- |
- |
Anne Polvi |
| ?/. |
- |
c.269A>T |
r.(?) |
p.(Tyr90Phe) |
- |
VUS |
g.23282339T>A |
g.22813130T>A |
- |
- |
SLC7A7_000073 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Nijmegen |
| ?/. |
- |
c.272C>A |
r.(?) |
p.(Ala91Glu) |
- |
VUS |
g.23282336G>T |
g.22813127G>T |
SLC7A7(NM_001126106.4):c.272C>A (p.A91E) |
- |
SLC7A7_000072 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Groningen |
| -?/. |
- |
c.272C>T |
r.(?) |
p.(Ala91Val) |
- |
likely benign |
g.23282336G>A |
g.22813127G>A |
SLC7A7(NM_001126106.2):c.272C>T (p.A91V), SLC7A7(NM_001126106.4):c.272C>T (p.A91V) |
- |
SLC7A7_000071 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_AMC |
| -/. |
- |
c.272C>T |
r.(?) |
p.(Ala91Val) |
- |
benign |
g.23282336G>A |
g.22813127G>A |
- |
- |
SLC7A7_000071 |
7 heterozygous, no homozygous; Clinindb (India) |
PubMed: Narang 2020, Journal: Narang 2020 |
- |
rs11568438 |
Germline |
- |
7/2795 individuals |
- |
- |
- |
Mohammed Faruq |
| -?/. |
- |
c.272C>T |
r.(?) |
p.(Ala91Val) |
- |
likely benign |
g.23282336G>A |
- |
SLC7A7(NM_001126106.2):c.272C>T (p.A91V), SLC7A7(NM_001126106.4):c.272C>T (p.A91V) |
- |
SLC7A7_000071 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Utrecht |
| +?/+? |
03 |
c.282_283insTGG |
r.(?) |
p.(Gly94_Thr95insTrp) |
- |
likely pathogenic |
g.23282325_23282326insCCA |
g.22813116_22813117insCCA |
c.283insTGG, p.Glu94_Thr95insTrp |
- |
SLC7A7_000055 |
1 Turkish patient with LPI (het) |
PubMed: Güzel-Ozantürk et al. 2013 |
- |
- |
SUMMARY record |
yes |
- |
- |
- |
- |
Anne Polvi |
| ?/. |
- |
c.335G>A |
r.(?) |
p.(Gly112Glu) |
- |
VUS |
g.23282273C>T |
g.22813064C>T |
SLC7A7(NM_001126106.4):c.335G>A (p.G112E) |
- |
SLC7A7_000070 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Groningen |
| -/. |
- |
c.342C>T |
r.(?) |
p.(Phe114=) |
- |
benign |
g.23282266G>A |
- |
SLC7A7(NM_001126106.4):c.342C>T (p.F114=) |
- |
SLC7A7_000081 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_AMC |
| +?/+? |
03 |
c.346_349del |
r.(?) |
p.(Ala116Serfs*53) |
- |
likely pathogenic |
g.23282262_23282265del |
g.22813053_22813056del |
c.344_347delTTGC, p.Leu115LeufsX53 |
- |
SLC7A7_000056 |
1 Turkish patient (hom) with LPI |
PubMed: Güzel-Ozantürk et al. 2013 |
- |
- |
SUMMARY record |
yes |
- |
- |
- |
- |
Anne Polvi |
| ?/. |
- |
c.359T>A |
r.(?) |
p.(Leu120His) |
- |
VUS |
g.23282249A>T |
- |
SLC7A7(NM_001126106.2):c.359T>A (p.L120H) |
- |
SLC7A7_000086 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Rotterdam |
| ?/. |
- |
c.363G>T |
r.(?) |
p.(Trp121Cys) |
- |
VUS |
g.23282245C>A |
g.22813036C>A |
- |
- |
SLC7A7_000065 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Nijmegen |
| +?/+? |
03 |
c.371T>C |
r.(370u>c) |
p.(Leu124Pro) |
- |
likely pathogenic |
g.23282237A>G |
g.22813028A>G |
c.370T>C p.L124P |
- |
SLC7A7_000007 |
2 Greek LPI families (com-het) |
PubMed: Sperandeo et al. 2008, PubMed: Font-Llitjos et al. 2009 |
- |
- |
SUMMARY record |
yes |
0/50 CON |
- |
- |
- |
Anne Polvi |
| +?/+? |
03 |
c.418G>C |
r.(418g>c) |
p.(Ala140Pro) |
- |
likely pathogenic |
g.23282190C>G |
g.22812981C>G |
c.418G>C p.A140P |
- |
SLC7A7_000008 |
1 Italian LPI family (com-het) |
PubMed: Sperandeo_2008 |
- |
- |
SUMMARY record |
yes |
- |
- |
- |
- |
Anne Polvi |
| +/+ |
03 |
c.454T>C |
r.(454u>c) |
p.(Phe152Leu) |
- |
pathogenic |
g.23282154A>G |
g.22812945A>G |
from the first methionine, 453T>C: F152L |
- |
SLC7A7_000011 |
1 Greek LPI patient (com-het); Functional studies: mutated protein showed moderately reduced transport activity |
PubMed: Sperandeo 2005 |
- |
- |
SUMMARY record |
yes |
- |
- |
- |
- |
Anne Polvi |
| -/. |
- |
c.456C>T |
r.(?) |
p.(Phe152=) |
- |
benign |
g.23282152G>A |
g.22812943G>A |
SLC7A7(NM_001126106.4):c.456C>T (p.F152=) |
- |
SLC7A7_000069 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_AMC |
| ?/. |
- |
c.475C>T |
r.(?) |
p.(Arg159Cys) |
- |
VUS |
g.23282133G>A |
g.22812924G>A |
- |
- |
SLC7A7_000076 |
conflicting interpretations of pathogenicity; 1 heterozygous, no homozygous; Clinindb (India) |
PubMed: Narang 2020, Journal: Narang 2020 |
- |
rs11568437 |
Germline |
- |
1/2793 individuals |
- |
- |
- |
Mohammed Faruq |
| -/. |
- |
c.498T>C |
r.(?) |
p.(Ile166=) |
- |
benign |
g.23282110A>G |
g.22812901A>G |
SLC7A7(NM_001126106.4):c.498T>C (p.I166=) |
- |
SLC7A7_000060 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Groningen |
| -/. |
- |
c.498T>C |
r.(?) |
p.(Ile166=) |
- |
benign |
g.23282110A>G |
g.22812901A>G |
SLC7A7(NM_001126106.4):c.498T>C (p.I166=) |
- |
SLC7A7_000060 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Nijmegen |
| -/. |
- |
c.498T>C |
r.(?) |
p.(Ile166=) |
- |
benign |
g.23282110A>G |
g.22812901A>G |
SLC7A7(NM_001126106.4):c.498T>C (p.I166=) |
- |
SLC7A7_000060 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_AMC |
| +/+ |
i03 |
c.499+1G>A |
r.-41_499del |
p.? |
- |
pathogenic |
g.23282108C>T |
g.22812899C>T |
IVS3 +1G>A in donor of intron 3; IVS3 +1G>A: exon 3 skipping |
- |
SLC7A7_000012 |
2 Japanese LPI families (com-het) |
PubMed: Sperandeo et al. 2000, PubMed: Kamada et al. 2001 |
- |
- |
SUMMARY record |
yes |
- |
- |
- |
- |
Anne Polvi |
| +/+ |
i03- |
c.500-4294_1908+1028del |
r.? |
p.? |
- |
pathogenic |
g.23241434_23253569del |
g.22772225_22784360del |
c.500-4294_1908+1028 del12136: p.L168_N511delfsX19 |
- |
SLC7A7_000016 |
1 Spanish LPI family (com-het) |
PubMed: Font-Llitjos et al. 2009 |
- |
- |
SUMMARY record |
yes |
0/50 CON |
- |
- |
- |
Anne Polvi |
| -?/. |
- |
c.500-20C>G |
r.(=) |
p.(=) |
- |
likely benign |
g.23249280G>C |
- |
SLC7A7(NM_001126106.2):c.500-20C>G |
- |
SLC7A7_000085 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Utrecht |
| +/+ |
i03-i04 |
c.500-?_625+?del |
r.500_625del |
p.(Cys167*) |
- |
pathogenic |
g.(?_23249135)_(23249260_?)del |
g.22779926_22780051del |
786del125: Frameshift after I166 (EX4); c.499+?_629-?, genomic rearrangement causing the deletion of the entire exon 4 at the cDNA level; c.500-?_625+?del: p.C167_G209delinsX |
- |
SLC7A7_000013 |
1 Pakistani LPI family (hom). |
PubMed: Palacín et al. 2001, PubMed: Sperandeo et al. 2005 |
- |
- |
SUMMARY record |
yes |
- |
- |
- |
- |
Anne Polvi |
| +/+ |
31_5i |
c.500-?_770+?del |
r.500_770del |
p.(Cys167*) |
- |
pathogenic |
g.(?_23248002)_(23249260_?)del |
g.22778793_22780051del |
IVS3-IVS5del9.7kb (intron 3 - intron 5) : exon 4 and 5 deletion in cDNAIVS3-IVS5del9.7kb (intron 3 - intron 5) : exon 4 and 5 deletion in cDNAIVS3-IVS5del9.7kb (intron 3 - intron 5) : exon 4 and 5 del |
- |
SLC7A7_000014 |
2 Japanese LPI families (1 hom and 1 com-het), 1 Japanese LPI family (com-het); Deletion from intron 3 to intron 5 > deletion of exons 4 and 5 |
PubMed: Shoji et al. 2002, PubMed: Kamada et al. 2001 |
- |
- |
SUMMARY record |
yes |
0/50 JAP CON |
- |
- |
- |
Anne Polvi |
| -?/. |
- |
c.516T>C |
r.(?) |
p.(Ile172=) |
- |
likely benign |
g.23249244A>G |
- |
SLC7A7(NM_001126106.2):c.516T>C (p.I172=) |
- |
SLC7A7_000088 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Rotterdam |
| -?/. |
- |
c.519C>T |
r.(?) |
p.(Asn173=) |
- |
likely benign |
g.23249241G>A |
- |
SLC7A7(NM_001126106.2):c.519C>T (p.N173=) |
- |
SLC7A7_000084 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Rotterdam |
| +/+ |
04 |
c.545dup |
r.(?) |
p.(Val183Glyfs*9) |
- |
pathogenic |
g.23249215dup |
g.22780006dup |
786insT: Frameshift after Leu182, termination at codon 190; c.545_546insT |
- |
SLC7A7_000017 |
1 Italian (Sicilia) LPI family (hom) |
PubMed: Sperandeo et al. 2000 |
- |
- |
SUMMARY record |
yes |
- |
- |
- |
- |
Anne Polvi |
| +/+ |
04 |
c.563C>T |
r.563c>u |
p.(Thr188Ile) |
- |
pathogenic |
g.23249197G>A |
g.22779988G>A |
849C>T: T188I; p.T188I (c.563C>T) |
- |
SLC7A7_000018 |
1 Greek LPI family (com-het); Functional studies confirmed disease-causative role |
PubMed: Palacín et al. 2001, PubMed: Sperandeo et al. 2005 |
- |
- |
SUMMARY record |
yes |
- |
- |
- |
- |
Anne Polvi |
| +?/+? |
04 |
c.571A>G |
r.(571a>g) |
p.(Lys191Glu) |
- |
likely pathogenic |
g.23249189T>C |
g.22779980T>C |
c.571A>G p.K191E |
- |
SLC7A7_000019 |
1 German LPI family (com-het) |
PubMed: Sperandeo_2008 |
- |
- |
SUMMARY record |
yes |
- |
- |
- |
- |
Anne Polvi |
| -/. |
- |
c.585G>A |
r.(?) |
p.(Leu195=) |
- |
benign |
g.23249175C>T |
g.22779966C>T |
SLC7A7(NM_001126106.4):c.585G>A (p.L195=) |
- |
SLC7A7_000078 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_AMC |
| -?/. |
- |
c.620G>A |
r.(?) |
p.(Gly207Asp) |
- |
likely benign |
g.23249140C>T |
- |
SLC7A7(NM_001126106.2):c.620G>A (p.G207D) |
- |
SLC7A7_000094 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Utrecht |
| +/+ |
04 |
c.622C>T |
r.(622c>u) |
p.(Gln208*) |
- |
pathogenic |
g.23249138G>A |
g.22779929G>A |
c.622C>T p.Q208X |
- |
SLC7A7_000020 |
1 German LPI family (com-het) |
PubMed: Sperandeo_2008 |
- |
- |
SUMMARY record |
yes |
- |
- |
- |
- |
Anne Polvi |
| +/+ |
i04 |
c.625+1G>A |
r.500_625del |
p.Cys167* |
- |
pathogenic |
g.23249134C>T |
g.22779925C>T |
911+1G>A; IVS4+1G>A |
- |
SLC7A7_000021 |
1 Turkish (hom) and 1 Korean (hom) LPI family, 3 Japanese (com-het) LPI families; Mutation causes exon 4 skipping |
PubMed: Mykkanen et al. 2000, PubMed: Noguchi et al.v2000, PubMed: Ko et al. 2012 |
- |
- |
SUMMARY record |
yes |
0/50 JAP CON |
- |
- |
- |
Anne Polvi |
| +?/+? |
i04 |
c.625+1G>C |
r.spl? |
p.? |
- |
likely pathogenic |
g.23249134C>G |
g.22779925C>G |
c.625+1G>C: p.C167_G209delinsX |
- |
SLC7A7_000022 |
1 English/Argentinean LPI family (com-het) |
PubMed: Font-Llitjos et al. 2009 |
- |
- |
SUMMARY record |
yes |
0/50 CON |
- |
- |
- |
Anne Polvi |
| -/. |
- |
c.660T>C |
r.(?) |
p.(Gly220=) |
- |
benign |
g.23248112A>G |
g.22778903A>G |
SLC7A7(NM_001126106.4):c.660T>C (p.G220=) |
- |
SLC7A7_000068 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_AMC |
| -/. |
- |
c.660T>C |
r.(?) |
p.(Gly220=) |
- |
benign |
g.23248112A>G |
- |
SLC7A7(NM_001126106.4):c.660T>C (p.G220=) |
- |
SLC7A7_000068 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Nijmegen |
| +?/+? |
05 |
c.713C>T |
r.713c>u |
p.(Ser238Phe) |
- |
likely pathogenic |
g.23248059G>A |
g.22778850G>A |
TCC>TTC: S238F |
- |
SLC7A7_000023 |
8 Japanese LPI families (3 hom and 5 com-het) |
PubMed: Shoji et al. 2002 |
- |
- |
SUMMARY record |
yes |
0/50 JAP CON |
- |
- |
- |
Anne Polvi |
| -?/. |
- |
c.720A>C |
r.(?) |
p.(Ser240=) |
- |
likely benign |
g.23248052T>G |
- |
SLC7A7(NM_001126106.2):c.720A>C (p.S240=) |
- |
SLC7A7_000087 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Rotterdam |
| -?/. |
- |
c.720A>C |
r.(?) |
p.(Ser240=) |
- |
likely benign |
g.23248052T>G |
- |
SLC7A7(NM_001126106.2):c.720A>C (p.S240=) |
- |
SLC7A7_000087 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Utrecht |
| +/+ |
05 |
c.726G>A |
r.(726g>a) |
p.(Trp242*) |
- |
pathogenic |
g.23248046C>T |
g.22778837C>T |
967G>A: W242X; 1012G>A: W242X |
- |
SLC7A7_000024 |
2 Italian, 1 North African and 3 Moroccan LPI families (all hom) |
PubMed: Sperandeo et al. 2000, PubMed: Mykkanen et al. 2000 |
- |
rs121908679 |
SUMMARY record |
yes |
- |
- |
- |
- |
Anne Polvi |
| +/. |
- |
c.726G>A |
r.(?) |
p.(Trp242Ter) |
- |
pathogenic |
g.23248046C>T |
g.22778837C>T |
SLC7A7(NM_001126106.2):c.726G>A (p.W242*) |
- |
SLC7A7_000024 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Nijmegen |
| +/. |
- |
c.726G>A |
r.(?) |
p.(Trp242Ter) |
- |
pathogenic |
g.23248046C>T |
- |
SLC7A7(NM_001126106.2):c.726G>A (p.W242*) |
- |
SLC7A7_000024 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Rotterdam |
| +?/. |
- |
c.749A>T |
r.(?) |
p.(Glu250Val) |
- |
likely pathogenic |
g.23248023T>A |
g.22778814T>A |
- |
- |
SLC7A7_000077 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Nijmegen |
| +?/+? |
05 |
c.753G>T |
r.(753g>u) |
p.(Glu251Asp) |
- |
likely pathogenic |
g.23248019C>A |
g.22778810C>A |
c.753G>T p.E251D |
- |
SLC7A7_000025 |
1 Italian LPI family (hom) |
PubMed: Sperandeo_2008 |
- |
- |
SUMMARY record |
yes |
- |
- |
- |
- |
Anne Polvi |
| +/+ |
i05- |
c.771-848_1908+718del4647 |
r.? |
p.? |
- |
pathogenic |
g.23241729_23246375del |
g.22772520_22777166del |
c.771-848_1908+718del4647: p.N258_N511delinsX |
- |
SLC7A7_000026 |
1 Spanish LPI family (hom) |
PubMed: Font-Llitjos et al. 2009 |
- |
- |
SUMMARY record |
yes |
0/50 CON |
- |
- |
- |
Anne Polvi |
| +?/+? |
06 |
c.782T>C |
r.(782u>c) |
p.(Leu261Pro) |
- |
likely pathogenic |
g.23245516A>G |
g.22776307A>G |
c.782T>C p.L261P |
- |
SLC7A7_000027 |
1 Italian LPI family (com-het) |
PubMed: Sperandeo_2008 |
- |
- |
SUMMARY record |
yes |
- |
- |
- |
- |
Anne Polvi |
| +/+ |
06 |
c.820dup |
r.(?) |
p.(Tyr274Leufs*21) |
- |
pathogenic |
g.23245478dup |
g.22776269dup |
c.820dupT: p.Y274fsX21 |
- |
SLC7A7_000028 |
1 English/Argentinean LPI family (com-het) |
PubMed: Font-Llitjos et al. 2009 |
- |
- |
SUMMARY record |
yes |
0/50 CON |
- |
- |
- |
Anne Polvi |
| +?/+? |
i06 |
c.894+1G>T |
r.spl? |
p.? |
- |
likely pathogenic |
g.23245403C>A |
g.22776194C>A |
IVS6 +1G>T in donor of intron 6: no RNA studies |
- |
SLC7A7_000029 |
1 Japanese LPI family (com-het) |
PubMed: Sperandeo et al. 2000 |
- |
- |
SUMMARY record |
yes |
- |
- |
- |
- |
Anne Polvi |
| -?/. |
- |
c.894+6G>A |
r.(=) |
p.(=) |
- |
likely benign |
g.23245398C>T |
- |
SLC7A7(NM_001126106.2):c.894+6G>A |
- |
SLC7A7_000080 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Rotterdam |
| -/. |
- |
c.894+103A>G |
r.(=) |
p.(=) |
- |
benign |
g.23245301T>C |
g.22776092T>C |
- |
- |
SLC7A7_000063 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Nijmegen |
| +?/+? |
i06 |
c.895-2A>G |
r.spl? |
p.? |
- |
likely pathogenic |
g.23245147T>C |
g.22775938T>C |
A-to-G transition in a splice acceptor sequence 6 (IVS6-2A G) |
- |
SLC7A7_000031 |
1 Lithuanian LPI patient (hom) |
PubMed: Cimbalistiene et al. 2007 |
- |
- |
SUMMARY record |
yes |
- |
- |
- |
- |
Anne Polvi |
| +/+ |
i06 |
c.895-2A>T |
r.895_904delacuuuugcag |
p.Thr299Ilefs*10 |
- |
pathogenic |
g.23245147T>A |
g.22775938T>A |
genomic change 1181-2A>T leads to alternative splicing: c.1181delACTTTTGCAG |
- |
SLC7A7_000030 |
Finnish Major LPI mutation: 35 Finnish LPI patients (hom); Functional analysis: loss of function |
PubMed: Torrens et al. 1999, PubMed: Bosrsani et al. 1999 |
- |
rs146582474 |
SUMMARY record |
yes |
- |
- |
- |
- |
Anne Polvi |
| +/. |
- |
c.895-2A>T |
r.spl? |
p.? |
ACMG |
pathogenic |
g.23245147T>A |
g.22775938T>A |
- |
- |
SLC7A7_000030 |
- |
PubMed: Trujillano 2017 |
- |
- |
Germline |
- |
- |
- |
- |
- |
Daniel Trujillano |
| -?/. |
- |
c.954A>T |
r.(?) |
p.(Leu318Phe) |
- |
likely benign |
g.23245086T>A |
- |
SLC7A7(NM_001126106.2):c.954A>T (p.L318F) |
- |
SLC7A7_000093 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Utrecht |
| +/+ |
07 |
c.998G>T |
r.[998g>a, 895_998del, 895_998del; 1096_1245del, 771_998del; 1096_1245del] |
p.[Arg333Met, Thr299Alafs*13, Thr299Alafs*13, Asn258_Arg333del; Gly366_Lys415del] |
- |
pathogenic |
g.23245042C>A |
g.22775833C>A |
p.R333M (c.997G>T) |
- |
SLC7A7_000032 |
1 Turkish LPI family (hom); Several different transcripts: a full length transcript with the R333M mutation and three shorter transcripts with skipping of exon 7 or exons 7 and 9 or exons 6, 7 and 9. |
PubMed: Sperandeo et al. 2005 |
- |
- |
SUMMARY record |
yes |
- |
- |
- |
- |
Anne Polvi |
| +?/+? |
i07 |
c.998+1G>T |
r.spl? |
p? |
- |
likely pathogenic |
g.23245041C>A |
g.22775832C>A |
1284+1G>T |
- |
SLC7A7_000033 |
3 Japanese LPI families (all com-het) |
PubMed: Shoji et al. 2002 |
- |
- |
SUMMARY record |
yes |
0/50 JAP CON |
- |
- |
- |
Anne Polvi |
| +/. |
- |
c.998+1G>T |
r.spl? |
p.? |
- |
pathogenic |
g.23245041C>A |
g.22775832C>A |
- |
- |
SLC7A7_000033 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Nijmegen |
| -?/. |
- |
c.999G>A |
r.(?) |
p.(Arg333=) |
- |
likely benign |
g.23244749C>T |
g.22775540C>T |
SLC7A7(NM_001126106.2):c.999G>A (p.R333=), SLC7A7(NM_001126106.4):c.999G>A (p.R333=) |
- |
SLC7A7_000066 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Groningen |
| -?/. |
- |
c.999G>A |
r.(?) |
p.(Arg333=) |
- |
likely benign |
g.23244749C>T |
- |
SLC7A7(NM_001126106.2):c.999G>A (p.R333=), SLC7A7(NM_001126106.4):c.999G>A (p.R333=) |
- |
SLC7A7_000066 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Rotterdam |
| -?/. |
- |
c.999G>A |
r.(?) |
p.(Arg333=) |
- |
likely benign |
g.23244749C>T |
- |
SLC7A7(NM_001126106.2):c.999G>A (p.R333=), SLC7A7(NM_001126106.4):c.999G>A (p.R333=) |
- |
SLC7A7_000066 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Utrecht |
| +/+ |
08 |
c.1001T>G |
r.1001u>g |
p.Leu334Arg |
- |
pathogenic |
g.23244747A>C |
g.22775538A>C |
c.1287T>G: L334R |
- |
SLC7A7_000034 |
1 Spanish LPI family (com-het) and 1 Chilean sibpair (hom) with LPI; Mutation nearly abolished (approximately 10% residual activity) sodium-independent L-arginine and sodium-dependent L-leucine transport activity |
PubMed: Torrens et al. 1999, PubMed: Font-Llitjos et al. 2009 |
- |
rs72552272 |
SUMMARY record |
yes |
0/50 CON |
- |
- |
- |
Anne Polvi |
| +/+ |
08 |
c.1005_1008del |
r.1005_1008delcuuu |
p.Phe335Leufs*15 |
- |
pathogenic |
g.23244743_23244746del |
g.22775534_22775537del |
1291delCTTT: framesift |
- |
SLC7A7_000035 |
3 Spanish LPI families (1 hom and 2 com-het) and 1 Japanese LPI family (com-het) |
PubMed: Torrens et al. 1999, PubMed: Mykkanen et al. 2000, PubMed: Shoji et al. 2002 |
- |
- |
SUMMARY record |
yes |
- |
- |
- |
- |
Anne Polvi |
| +?/+? |
09 |
c.1013G>A |
r.1013g>a |
p.(Gly338Asp) |
- |
likely pathogenic |
g.23244735C>T |
g.22775526C>T |
1299G→A (exon 8): G338D |
- |
SLC7A7_000036 |
1 Swedish LPI family (hom) |
PubMed: Mykkanen et al. 2000 |
- |
- |
SUMMARY record |
yes |
- |
- |
- |
- |
Anne Polvi |
| ?/. |
- |
c.1055A>G |
r.(?) |
p.(His352Arg) |
- |
VUS |
g.23244693T>C |
- |
SLC7A7(NM_001126106.2):c.1055A>G (p.H352R) |
- |
SLC7A7_000079 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Rotterdam |
| +?/+? |
09 |
c.1093A>T |
r.(1093a>u) |
p.(Asn365Tyr) |
- |
likely pathogenic |
g.23244655T>A |
g.22775446T>A |
c.1093A>T p.N365Y |
- |
SLC7A7_000037 |
1 German LPI family (hom) |
PubMed: Sperandeo_2008 |
- |
- |
SUMMARY record |
yes |
- |
- |
- |
- |
Anne Polvi |
| +?/+? |
09 |
c.1098dup |
r.(?) |
p.(Ile367Tyrfs*17) |
- |
likely pathogenic |
g.23243710dup |
g.22774501dup |
c.1099insT, p.Ile367TyrfsX16 |
- |
SLC7A7_000057 |
2 Turkish patients (hom) with LPI |
PubMed: Güzel-Ozantürk et al. 2013 |
- |
- |
SUMMARY record |
yes |
- |
- |
- |
- |
Anne Polvi |
| ?/. |
- |
c.1128A>C |
r.(?) |
p.(Glu376Asp) |
- |
VUS |
g.23243680T>G |
- |
SLC7A7(NM_001126106.2):c.1128A>C (p.E376D) |
- |
SLC7A7_000090 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Rotterdam |
| +/+ |
09 |
c.1147_1151dup |
r.(1147_1151dupaacua) |
p.(Tyr384*) |
- |
pathogenic |
g.23243657_23243661dup |
g.22774448_22774452dup |
1438duplAACTA (exon 9): Y384X |
- |
SLC7A7_000038 |
1 Canadian LPI family (het) |
PubMed: Mykkanen et al. 2000 |
- |
- |
SUMMARY record |
yes |
- |
- |
- |
- |
Anne Polvi |
| +/+ |
09 |
c.1158C>A |
r.(1158c>a) |
p.(Ser386Arg) |
- |
pathogenic |
g.23243650G>T |
g.22774441G>T |
1399C>A: S386R |
- |
SLC7A7_000039 |
2 Italian (Calabria) LPI families (hom) |
PubMed: Sperandeo et al. 2000, PubMed: Sperandeo et al. 2005 |
- |
- |
SUMMARY record |
yes |
- |
- |
- |
- |
Anne Polvi |
| +/+ |
09 |
c.1185_1188del |
r.(?) |
p.(Ser396Leufs*122) |
- |
pathogenic |
g.23243622_23243625del |
g.22774413_22774416del |
1425delTTCT: Frameshift after Leu395, termination at codon 516; 1471delTTCT: Frameshift after L395; c.1185_1188delTTCT: p.S396fsX121 |
- |
SLC7A7_000040 |
1 Italian, 1 Tunisian, 1 German and 1 Spanish LPI family (all hom) and 1 French-Algerian LPI family 1 (com-het); Functional studies confirmed disease-causative role |
PubMed: Sperandeo et al. 2000, PubMed: Mykkanen et al. 2000, PubMed: Sperandeo et al. 2005, PubMed: Font-Llitjos et al. 2009 |
- |
- |
SUMMARY record |
yes |
0/50 CON |
- |
- |
- |
Anne Polvi |
| +/. |
- |
c.1185_1188del |
r.(?) |
p.(Ser396LeufsTer122) |
- |
pathogenic |
g.23243622_23243625del |
- |
SLC7A7(NM_001126106.4):c.1185_1188delTTCT (p.S396Lfs*122) |
- |
SLC7A7_000040 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_VUmc |
| +/+ |
09 |
c.1228C>T |
r.1228c>u |
p.(Arg410*) |
- |
pathogenic |
g.23243580G>A |
g.22774371G>A |
CGA>TGA: R410X |
- |
SLC7A7_000041 |
12 Japanese LPI families (7 hom and 5 com-het) and 1 Moroccan LPI family (hom) |
PubMed: Noguchi et al.v2000, PubMed: Shoji et al. 2002, PubMed: Font-Llitjos et al. 2009 |
- |
rs121908678 |
SUMMARY record |
yes |
0/50 JAP CON |
- |
- |
- |
Anne Polvi |
| +/. |
- |
c.1228C>T |
r.(?) |
p.(Arg410Ter) |
- |
pathogenic |
g.23243580G>A |
g.22774371G>A |
SLC7A7(NM_001126106.2):c.1228C>T (p.R410*) |
- |
SLC7A7_000041 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Rotterdam |
| ?/. |
- |
c.1235G>A |
r.(?) |
p.(Arg412His) |
- |
VUS |
g.23243573C>T |
- |
SLC7A7(NM_003982.4):c.1235G>A (p.(Arg412His)) |
- |
SLC7A7_000099 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Leiden |
| -?/. |
- |
c.1245+10C>T |
r.(=) |
p.(=) |
- |
likely benign |
g.23243553G>A |
- |
SLC7A7(NM_001126106.2):c.1245+10C>T |
- |
SLC7A7_000089 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Rotterdam |
| -?/. |
- |
c.1246-24_1246-20del |
r.(=) |
p.(=) |
- |
likely benign |
g.23243345_23243349del |
- |
SLC7A7(NM_001126106.2):c.1246-24_1246-20delCAGTT |
- |
SLC7A7_000096 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Utrecht |
| +/+ |
10 |
c.1262del |
r.1262delc |
p.(Pro421Argfs*98) |
- |
pathogenic |
g.23243311del |
g.22774102del |
1548delC (exon 10) Frameshift after F420 |
- |
SLC7A7_000042 |
1 Arabic LPI family (hom); Functional analysis: loss of function |
PubMed: Mykkanen et al. 2000 |
- |
- |
SUMMARY record |
yes |
- |
- |
- |
- |
Anne Polvi |
| +?/+? |
10 |
c.1273T>C |
r.(1273u>c) |
p.(Cys425Arg) |
- |
likely pathogenic |
g.23243298A>G |
g.22774089A>G |
c.1273T>C: p.C425R |
- |
SLC7A7_000043 |
1 Spanish LPI family (com-het) |
PubMed: Font-Llitjos et al. 2009 |
- |
- |
SUMMARY record |
yes |
0/50 CON |
- |
- |
- |
Anne Polvi |