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%)
Effect
Reported
Exon
DNA change (cDNA)
RNA change
Protein
Classification method
Clinical classification
DNA change (genomic) (hg19)
DNA change (hg38)
Published as
ISCN
DB-ID
Variant remarks
Reference
ClinVar ID
dbSNP ID
Origin
Segregation
Frequency
Re-site
VIP
Methylation
Owner
+?/.
1
-
c.(?_11668+196)_*313{0}
r.?
p.?
ACMG
likely pathogenic
g.(?_73826844)_(73839058_?)del
g.(?_73599717)_(73611931_?)del
arr([GRCh37] 2p13.1(73,826,844-73,839,058)x1), compound heterozygous
-
ALMS1_000856
-
PubMed: Perea-Romero 2021
-
-
Germline
yes
-
-
-
-
LOVD
+/.
1
-
c.643eC>T
r.(?)
p.(Arg2145Ter)
-
likely pathogenic
g.73680087C>T
g.73452960C>T
-
-
ALMS1_000628
-
PubMed: Holtan 2020
-
-
Germline
-
1/899 cases
-
-
-
Global Variome, with Curator vacancy
+?/.
1
-
c.54_55insTAG
r.(?)
p.(Glu19Ter)
-
likely pathogenic
g.73613050_73613051insTAG
g.73385922_73385923insTAG
c.54_55insTAG
-
ALMS1_000634
-
PubMed: Neubauer 2021
-
-
Unknown
-
-
-
-
-
Cordula Haas
-?/., ?/.
2
-
c.57_74dup
r.(?)
p.(Glu23_Glu28dup)
-
likely benign, VUS
g.73613053_73613070dup
-
ALMS1(NM_001378454.1):c.57_74dup (p.(Glu23_Glu28dup))
-
ALMS1_000885
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record, Unknown
-
-
-
-
-
VKGL-NL_Leiden , MobiDetails
?/.
1
-
c.75_77dup
r.(?)
p.(Glu28dup)
-
VUS
g.73613071_73613073dup
g.73385943_73385945dup
36_44delinsGGAGGAGGAGGA
-
ALMS1_000284
-
-
-
-
De novo
-
-
-
-
-
Johan den Dunnen
-?/.
1
-
c.103_105dup
r.(?)
p.(Ala35dup)
-
likely benign
g.73613099_73613101dup
g.73385971_73385973dup
ALMS1(NM_015120.4):c.106_108dupGCG (p.A36dup)
-
ALMS1_000794
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
-?/.
1
-
c.246G>A
r.(?)
p.(Ala82=)
-
likely benign
g.73613242G>A
g.73386114G>A
ALMS1(NM_015120.4):c.249G>A (p.A83=)
-
ALMS1_000795
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+/.
1
-
c.296C>A
r.(?)
p.(Ser99Ter)
-
likely pathogenic
g.73613292C>A
g.73386164C>A
-
-
ALMS1_000626
-
PubMed: Holtan 2020
-
-
Germline
-
1/899 cases
-
-
-
Global Variome, with Curator vacancy
-?/.
1
-
c.302G>A
r.(?)
p.(Gly101Asp)
-
likely benign
g.73613298G>A
g.73386170G>A
ALMS1(NM_015120.4):c.302G>A (p.(Gly102Asp))
-
ALMS1_000796
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
-?/.
1
1
c.323A>G
r.(?)
p.(Lys108Arg)
-
likely benign
g.73613319A>G
g.73386191A>G
A323G
-
ALMS1_000637
-
PubMed: Kominami 2019 , PubMed: Kominami 2017 , Journal: Kominami 2017
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
1
-
c.324+4066_10384+3639delinsCACTT
r.?
p.?
-
pathogenic
g.73617386_73789908delinsCACTT
g.73390258_73562781delinsCACTT
del exon16_17 / del exon2_15 73617393-73789869del
-
ALMS1_000701
172,476 bp deletion, 5 bp insertion
PubMed: Lindstrand 2016
-
-
Germline
-
-
-
-
-
LOVD
?/.
1
-
c.451-5T>G
r.?
p.?
-
VUS
g.73646246T>G
g.73419118T>G
c.451-5T>G
-
ALMS1_000866
0/1266 control chromosomes
PubMed: Xu 2015
-
-
Germline
-
2/314 case chromosomes
-
-
-
LOVD
+?/.
1
-
c.547del
r.(?)
p.(Glu183LysfsTer2)
-
likely pathogenic
g.73646347del
g.73419219del
ALMS1, variant 1: c.550del/p.V184*, variant 2: c.6305C>A/p.S2102*
-
ALMS1_000775
1 more item
PubMed: Weisschuh 2020
-
-
Unknown
?
-
-
-
-
LOVD
?/.
1
-
c.568C>A
r.(?)
p.(Leu190Met)
-
VUS
g.73646368C>A
g.73419240C>A
ALMS1 nucleotide 1, protein 1:c.571C>A, p.Leu191Met
-
ALMS1_000783
homozygous, ACMG unclassified - no access to supplementary table 2
PubMed: Hull 2020
-
-
Germline
?
-
-
-
-
LOVD
-?/.
1
-
c.611A>C
r.(?)
p.(Glu204Ala)
-
likely benign
g.73646411A>C
g.73419283A>C
ALMS1(NM_015120.4):c.614A>C (p.(Glu205Ala))
-
ALMS1_000797
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
-?/.
1
-
c.650T>C
r.(?)
p.(Ile217Thr)
-
likely benign
g.73649988T>C
g.73422860T>C
ALMS1(NM_015120.4):c.650T>C (p.(Ile218Thr))
-
ALMS1_000828
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
-?/.
1
-
c.668C>A
r.(?)
p.(Ser223Tyr)
-
likely benign
g.73650006C>A
-
ALMS1(NM_015120.4):c.671C>A (p.(Pro224His))
-
ALMS1_000878
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
-?/.
1
-
c.717G>A
r.(?)
p.(Ala239=)
-
likely benign
g.73650055G>A
g.73422927G>A
ALMS1(NM_015120.4):c.720G>A (p.A240=)
-
ALMS1_000798
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
-?/.
1
-
c.718C>T
r.(?)
p.(Pro240Ser)
-
likely benign
g.73650056C>T
g.73422928C>T
ALMS1(NM_015120.4):c.721C>T (p.P241S)
-
ALMS1_000829
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
?/.
1
-
c.719C>A
r.(?)
p.(Pro240His)
ACMG
VUS
g.73650057C>A
g.73422929C>A
716C>A Ala239Glu)
-
ALMS1_000635
ACMG PM2, BP4
PubMed: Zenteno 2020
-
-
Germline
-
1/143 cases
-
-
-
Johan den Dunnen
?/.
1
-
c.738A>C
r.(?)
p.(Gln246His)
-
VUS
g.73650076A>C
g.73422948A>C
ALMS1(NM_015120.4):c.741A>C (p.(Gln247His))
-
ALMS1_000844
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+?/.
1
-
c.764G>A
r.(?)
p.(Arg255Lys)
-
likely pathogenic
g.73650102G>A
g.73422974G>A
c.767G>A
-
ALMS1_000712
-
PubMed: Patel 2016
-
-
Germline
-
-
-
-
-
LOVD
+/.
1
-
c.797G>A
r.(?)
p.(Trp266Ter)
-
pathogenic (recessive)
g.73651590G>A
g.73424462G>A
797G>A (Trp266*)
-
ALMS1_000872
-
PubMed: Consugar 2015
-
-
Germline
yes
-
-
-
-
LOVD
+/., +?/.
6
5
c.805C>T
r.(?)
p.(Arg269Ter)
ACMG
likely pathogenic, pathogenic, pathogenic (recessive)
g.73651598C>T
g.73424470C>T
808C>T (R269X), c.808C>T
-
ALMS1_000281
-
Sharon, submitted, PubMed: Lazar 2015 , Journal: Lazar 2015 , PubMed: Lazar 2015 ; Journal: Lazar 2015 , 3 more items
-
-
Germline
yes
1/2420 IRD families
-
-
-
Global Variome, with Curator vacancy , Johan den Dunnen , Dror Sharon , Pieter Klap
+?/.
1
-
c.833_834del
r.(?)
p.(Phe278SerfsTer16)
-
likely pathogenic
g.73651626_73651627del
g.73424498_73424499del
c.834_835delAT, p.Phe278SerfsTer16
-
ALMS1_000771
different transcript, ENST00000264448.6(ALMS1):c.833_834del, p.(Phe278Serfs*16), heterozygous
PubMed: Zampaglione 2020
-
-
Unknown
?
-
-
-
-
LOVD
+?/.
1
-
c.846dup
r.(?)
p.(Glu283ArgfsTer12)
-
likely pathogenic
g.73651639dup
g.73424511dup
c.849dup
-
ALMS1_000713
-
PubMed: Patel 2016
-
-
Germline
-
-
-
-
-
LOVD
-?/.
1
-
c.901A>C
r.(?)
p.(Ile301Leu)
-
likely benign
g.73651694A>C
g.73424566A>C
ALMS1(NM_015120.4):c.901A>C (p.(Ile302Leu))
-
ALMS1_000830
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+?/.
2
-
c.964G>T
r.(?)
p.(Glu322Ter)
-
likely pathogenic
g.73651757G>T
g.73424629G>T
ALMS1, variant 1: c.967G>T/p.E323*, variant 2: c.5145T>G/p.Y1715*
-
ALMS1_000776
solved, compound heterozygous
PubMed: Weisschuh 2020
-
-
Unknown
?
-
-
-
-
LOVD
+?/.
1
-
c.1043G>A
r.(?)
p.(Trp348Ter)
-
likely pathogenic (recessive)
g.73651836G>A
g.73424708G>A
1043G>A (p.W348*)
-
ALMS1_000858
-
PubMed: Weisschuh 2016
-
-
Germline
-
-
-
-
-
LOVD
+?/.
1
-
c.1051C>T
r.(?)
p.(Arg351Ter)
-
likely pathogenic (recessive)
g.73651844C>T
g.73424716C>T
c.1054C>T
-
ALMS1_000706
-
PubMed: Xu 2016 , PubMed: Huang 2016
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+?/.
1
-
c.1144C>T
r.(?)
p.(His382Tyr)
-
likely pathogenic
g.55955604G>A
g.54195844G>A
p.(His3882Tyr)
-
ALMS1_000875
expression cloning mini-gene splicing assay shows no effect on splicing
PubMed: Castro Sanchez 2015 , PubMed: Alvarez-Satta 2017
-
-
Germline
-
-
-
-
-
LOVD
+/.
2
-
c.1196_1202del
r.(?)
p.(Thr399LysfsTer11)
ACMG
pathogenic
g.73651989_73651995del
g.73424861_73424867del
1196_1202delCACAGGA, ALMS1 c.1199_1205del, p.(Thr400Lysfs*11)
-
ALMS1_000438
compound heterozygous, probably causal
PubMed: Zhu 2022
-
-
Germline/De novo (untested), Unknown
?
-
-
-
-
IMGAG
-?/.
1
-
c.1338+19A>G
r.(=)
p.(=)
-
likely benign
g.73653700A>G
g.73426572A>G
ALMS1(NM_015120.4):c.1341+19A>G
-
ALMS1_000845
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
-?/.
1
-
c.1353G>A
r.(?)
p.(Ser451=)
-
likely benign
g.73659340G>A
g.73432212G>A
ALMS1(NM_001378454.1):c.1353G>A (p.(Ser451Ser))
-
ALMS1_000852
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
?/.
1
-
c.1433-2A>G
r.spl?
p.?
-
VUS
g.73675088A>G
-
ALMS1(NM_015120.4):c.1436-2A>G
-
ALMS1_000879
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
+?/.
1
-
c.1529T>A
r.(?)
p.(Leu510Ter)
ACMG
likely pathogenic (recessive)
g.73675186T>A
g.73448056T>A
c.1529T>A
-
ALMS1_000827
-
-
-
-
Germline
-
-
-
-
-
Viktoriia Zabnenkova
+?/.
1
-
c.1625T>A
r.(?)
p.(Leu542Ter)
-
likely pathogenic
g.73675279T>A
g.73448152T>A
ALMS1, variant 1: c.1628T>A/p.L543*, variant 2: c.10149dup/p.S3384Qfs*7
-
ALMS1_000777
solved, compound heterozygous
PubMed: Weisschuh 2020
-
-
Germline
yes
-
-
-
-
LOVD
+?/.
1
-
c.1671del
r.(?)
p.(Pro558LeufsTer37)
-
likely pathogenic
g.73675325del
g.73448198del
c.1674del
-
ALMS1_000714
-
PubMed: Patel 2016
-
-
Germline
-
-
-
-
-
LOVD
+/.
2
-
c.1727C>G
r.(?)
p.(Ser576Ter)
-
pathogenic, pathogenic (recessive)
g.73675381C>G
g.73448254C>G
1730C>G, c.1730C>G
-
ALMS1_000757
-
Villafuerte-de la Cruz RA, et al., 2023. Submitted, PubMed: Redin-2012
ClinVar-801720
rs756389027
Germline
yes
-
-
-
-
Rocio Villafuerte-de la Cruz
+?/.
1
-
c.1732del
r.(?)
p.(Arg578GlyfsTer17)
-
likely pathogenic
g.73675386del
g.73448259del
ALMS1 c.1729delA, p.Arg577GlyfsTer17
-
ALMS1_000325
heterozygous
PubMed: Turro 2020
-
-
Germline/De novo (untested)
?
-
-
-
-
LOVD
-?/.
1
-
c.1871A>G
r.(?)
p.(His624Arg)
-
likely benign
g.73675525A>G
g.73448398A>G
c.1868A>G
-
ALMS1_000328
17 heterozygous, no homozygous; Clinindb (India)
PubMed: Narang 2020 , Journal: Narang 2020
-
rs41291187
Germline
-
17/2792 individuals
-
-
-
Mohammed Faruq
+?/.
1
-
c.1894dup
r.(?)
p.(Tyr632LeufsTer9)
-
likely pathogenic
g.73675548dup
g.73448421dup
ALMS1, variant 1: c.1897dup/p.Y633Lfs*9, variant 2: c.6571_6574del/p.S2191Mfs*15
-
ALMS1_000778
solved, compound heterozygous
PubMed: Weisschuh 2020
-
-
Germline
yes
-
-
-
-
LOVD
-?/.
1
-
c.1899A>G
r.(?)
p.(Gln633=)
-
likely benign
g.73675553A>G
g.73448426A>G
ALMS1(NM_015120.4):c.1902A>G (p.Q634=)
-
ALMS1_000799
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
+/.
1
-
c.2036A>G
r.(?)
p.(Tyr679Cys)
-
pathogenic
g.73675690A>G
g.73448563A>G
-
-
ALMS1_000254
-
PubMed: DDDS 2015 , Journal: DDDS 2015
-
-
Germline
-
-
-
-
-
Johan den Dunnen
?/.
1
-
c.2038C>G
r.(?)
p.(Arg680Gly)
-
VUS
g.73675692C>G
g.73448565C>G
ALMS1(NM_015120.4):c.2041C>G (p.R681G)
-
ALMS1_000849
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Groningen
+?/.
1
-
c.2067del
r.(?)
p.(Asp690IlefsTer4)
-
likely pathogenic
g.73675721del
g.73448594del
2064del (D689IfsX4)
-
ALMS1_000859
-
PubMed: Wang 2015
-
-
Germline
-
-
-
-
-
LOVD
+?/.
3
-
c.2087C>A
r.(?)
p.(Ser696Ter)
-
likely pathogenic, likely pathogenic (recessive)
g.73675741C>A
g.73448614C>A
2084C>A (S695X), c.2090C>A
-
ALMS1_000707
-
PubMed: Wang 2015 , PubMed: Xu 2016 , PubMed: Huang 2016
-
-
Germline
-
-
-
-
-
Johan den Dunnen
-?/.
1
-
c.2122A>G
r.(?)
p.(Thr708Ala)
-
likely benign
g.73675776A>G
g.73448649A>G
ALMS1(NM_015120.4):c.2125A>G (p.(Thr709Ala))
-
ALMS1_000843
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+/.
1
-
c.2212del
r.(?)
p.(Glu738ArgfsTer41)
-
pathogenic (recessive)
g.73675866del
g.73448739del
2215delC
-
ALMS1_000699
-
PubMed: Porto 2017
-
-
Germline
-
-
-
-
-
LOVD
+/.
1
-
c.2294C>G
r.(?)
p.(Ser765*)
-
pathogenic
g.73675948C>G
g.73448821C>G
-
-
ALMS1_000800
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/.
1
-
c.2296C>T
r.(?)
p.(Gln766Ter)
-
pathogenic (recessive)
g.73675950C>T
g.73448823C>T
2293C>T
-
ALMS1_000873
-
PubMed: Aliferis 2012
-
-
Germline
-
-
-
-
-
LOVD
+?/.
1
-
c.2308_2309del
r.(?)
p.(Pro770Ter)
-
likely pathogenic
g.73675962_73675963del
g.73448835_73448836del
ALMS1, variant 1: c.2317_2318del/p.I773Ffs*13, variant 2: c.2311_2312del/p.I771Ffs*13
-
ALMS1_000779
1 more item
PubMed: Weisschuh 2020
-
-
Unknown
?
-
-
-
-
LOVD
+?/.
1
-
c.2314_2315del
r.(?)
p.(Ile772PhefsTer13)
-
likely pathogenic
g.73675968_73675969del
g.73448841_73448842del
ALMS1, variant 1: c.2317_2318del/p.I773Ffs*13, variant 2: c.2311_2312del/p.I771Ffs*13
-
ALMS1_000434
solved, compound heterozygous
PubMed: Weisschuh 2020
-
-
Unknown
?
-
-
-
-
LOVD
?/.
3
-
c.2416G>C
r.(?)
p.(Val806Leu)
-
VUS
g.73676070G>C
g.73448943G>C
c.2413G>C (V805L)
-
ALMS1_000656
30/1266 control chromosomes
PubMed: Xu 2015
-
rs138921247
Germline
-
8/314 case chromosomes
-
-
-
LOVD
+/.
1
8
c.2533G>T
r.(?)
p.(Gly846Ter)
-
pathogenic (recessive)
g.73676187G>T
g.73449060G>T
-
-
ALMS1_000445
-
-
-
-
Germline
-
-
-
-
-
Marta de Castro-Miró
+/.
1
-
c.2643dup
r.(?)
p.(Glu882*)
-
pathogenic
g.73676297dup
g.73449170dup
ALMS1(NM_015120.4):c.2646dupT (p.E883*)
-
ALMS1_000846
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+?/.
1
-
c.2663C>G
r.(?)
p.(Ser888Ter)
ACMG
likely pathogenic
g.73676317C>G
g.73449190C>G
ALMS1, c.2660C>G, p.Ser887*, compound heterozygous
-
ALMS1_000788
different transcript: ENST00000264448.6(ALMS1):c.2660C>G, p.Ser887*, compound heterozygous
PubMed: Perea-Romero 2021
-
-
Germline
yes
-
-
-
-
LOVD
+?/.
1
-
c.2726C>G
r.(?)
p.(Ser909Ter)
-
likely pathogenic
g.73676380C>G
g.73449253C>G
c.2729C>G
-
ALMS1_000715
-
PubMed: Patel 2016
-
-
Germline
-
-
-
-
-
LOVD
+?/.
1
-
c.2758C>T
r.(?)
p.(Gln920Ter)
-
likely pathogenic
g.73676412C>T
g.73449285C>T
ALMS1 p.Q920X, p.R2928X
-
ALMS1_000773
different transcript NM_001378454.1(ALMS1):c.2758C>T, p.Q920X
PubMed: Hirano 2020
-
-
Germline
yes
-
-
-
-
LOVD
?/.
1
-
c.2775dup
r.(?)
p.(Leu926Serfs*4)
-
VUS
g.73676429dup
g.73449302dup
ALMS1(NM_015120.4):c.2772dup (p.(Leu927Serfs*4))
-
ALMS1_000838
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+/., ?/.
2
8
c.2819T>A
r.(?)
p.(Leu940Ter)
-
pathogenic, VUS
g.73676473T>A
g.73449346T>A
c.2816T>A(p.L939*), 1 more item
-
ALMS1_000774
heterozygous, ACMG unclassified - no access to supplementary table 2
PubMed: Hull 2020 , PubMed: Wang 2016
-
-
Germline
?
-
-
-
-
LOVD
+/.
1
-
c.2954_2955insCTGGACTGCTATT
r.(?)
p.(Gly986TrpfsTer8)
-
pathogenic (recessive)
g.73676608_73676609insCTGGACTGCTATT
g.73449481_73449482insCTGGACTGCTATT
2:73676607C>CCTGGACTGCTATT ENST00000264448.6:c.2958_2959insCTATTCTGGACTG (Thr987LeufsTer6)
-
ALMS1_000638
-
PubMed: Carss 2017
-
-
Germline
-
-
-
-
-
LOVD
+?/.
1
-
c.2955_2956insCTATTCTGGACTG
r.(?)
p.(Gly986LeufsTer8)
-
likely pathogenic
g.73676609_73676610insCTATTCTGGACTG
g.73449482_73449483insCTATTCTGGACTG
ALMS1 c.2958_2959insCTATTCTGGACTG, p.Thr987LeufsTer6
-
ALMS1_000780
1 more item
PubMed: Turro 2020
-
-
Germline/De novo (untested)
?
-
-
-
-
LOVD
+/.
1
-
c.2991_2992del
r.(?)
p.(Val998ThrfsTer8)
ACMG
pathogenic
g.73676645_73676646del
g.73449518_73449519del
ALMS1 NM_015120: g.63760_63761delAG, c.2988_2989delAG, p.V997Tfs*8
-
ALMS1_000758
different transcript: ENST00000264448.6(ALMS1):c.2988_2989del
PubMed: Xu 2020
-
-
Germline
yes
-
-
-
-
LOVD
+/.
1
8
c.2999C>G
r.(?)
p.(Ser1000Ter)
-
pathogenic
g.73676653C>G
g.73449526C>G
c.2996C>G
-
ALMS1_000725
-
PubMed: Wang-2013
-
-
Unknown
-
-
-
-
-
LOVD
+/.
1
-
c.3056dup
r.(?)
p.(Asp1019GlufsTer2)
-
pathogenic (recessive)
g.73676710dup
g.73449583dup
3059dupA
-
ALMS1_000700
-
PubMed: Porto 2017
-
-
Germline
-
-
-
-
-
LOVD
+?/.
1
-
c.3184C>T
r.(?)
p.(Gln1062Ter)
-
likely pathogenic
g.73676838C>T
g.73449711C>T
3181C>T (Q1061X)
-
ALMS1_000863
-
PubMed: Wang 2015
-
-
Germline
-
-
-
-
-
LOVD
-?/.
1
-
c.3255G>T
r.(?)
p.(Gln1085His)
-
likely benign
g.73676909G>T
g.73449782G>T
ALMS1(NM_015120.4):c.3258G>T (p.Q1086H)
-
ALMS1_000801
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
-?/.
1
-
c.3305A>C
r.(?)
p.(Lys1102Thr)
-
likely benign
g.73676959A>C
g.73449832A>C
ALMS1(NM_015120.4):c.3308A>C (p.K1103T)
-
ALMS1_000840
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
+/.
1
8
c.3343del
r.(?)
p.(His1115IlefsTer7)
-
pathogenic
g.73676997del
g.73449870del
c.[3340del]
-
ALMS1_000759
-
PubMed: Redin-2012
-
-
Germline
-
-
-
-
-
LOVD
?/.
1
-
c.3596G>A
r.(?)
p.(Gly1199Asp)
-
VUS
g.73677250G>A
g.73450123G>A
ALMS1(NM_015120.4):c.3599G>A (p.G1200D)
-
ALMS1_000802
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
+/.
1
-
c.3656C>G
r.(?)
p.(Ser1219Ter)
-
pathogenic (recessive)
g.73677310C>G
g.73450183C>G
3653C>G
-
ALMS1_000870
-
PubMed: Liu 2015
-
-
Germline
-
-
-
-
-
LOVD
+/.
1
-
c.3895C>T
r.(?)
p.(Gln1299*)
-
pathogenic
g.73677549C>T
g.73450422C>T
ALMS1(ENST00000264448.6):c.3892C>T
-
ALMS1_000831
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Nijmegen
+/., +?/.
4
8
c.3899C>A
r.(?)
p.(Ser1300Ter)
ACMG
likely pathogenic, likely pathogenic (recessive), pathogenic, pathogenic (recessive)
g.73677553C>A
g.73450426C>A
3896C>A (Ser1299*), ALMS1 NM_015120: g.64668C>A, c.3896C>A, p.S1299X, c.3896C>A, 1 more item
-
ALMS1_000760
different transcript: ENST00000264448.6(ALMS1):c.3896C>A
PubMed: Consugar 2015 , PubMed: Liu-2020 , PubMed: Wang 2016 , PubMed: Xu 2020
-
-
Germline
yes
-
-
-
-
LOVD
+/.
1
8
c.3922G>T
r.(?)
p.(Glu1308Ter)
-
pathogenic (recessive)
g.73677576G>T
g.73450449G>T
c.3919G>T
-
ALMS1_000446
-
-
-
-
Germline
-
-
-
-
-
Marta de Castro-Miró
-/.
1
-
c.3936T>C
r.(?)
p.(Asn1312=)
-
benign
g.73677590T>C
g.73450463T>C
ALMS1(NM_015120.4):c.3939T>C (p.N1313=)
-
ALMS1_000803
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
?/.
1
-
c.3977T>C
r.(?)
p.(Ile1326Thr)
-
VUS
g.73677631T>C
-
ALMS1(NM_015120.4):c.3980T>C (p.(Ile1327Thr))
-
ALMS1_000880
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+?/.
1
-
c.4147dup
r.(?)
p.(Gln1383ProfsTer17)
ACMG
likely pathogenic
g.73677801dup
g.73450674dup
ALMS1 c.4150dup, p.(Thr1384Asnfs*15)
-
ALMS1_000702
different transcript: single heterozygous variant (recessive)
PubMed: Jespersgaar 2019
-
-
Germline
?
-
-
-
-
LOVD
+/.
1
8
c.4153dup
r.(?)
p.(Thr1385AsnfsTer15)
ACMG
pathogenic (recessive)
g.73677807dup
g.73450680dup
c.4156dup
-
ALMS1_000702
ACMG: Class 5 (PVS1, PM3, PS4_SUP, PM2_SUP)
PMID: 25706677, 25296579, 23188138, 28432734,
ClinVar-000210127
-
Germline
yes
-
-
-
-
Andreas Laner
-?/.
1
-
c.4204A>G
r.(?)
p.(Thr1402Ala)
-
likely benign
g.73677858A>G
g.73450731A>G
ALMS1(NM_015120.4):c.4201A>G (p.(Thr1403Ala))
-
ALMS1_000832
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
?/.
1
-
c.4244G>A
r.(?)
p.(Gly1415Asp)
-
VUS
g.73677898G>A
-
ALMS1(NM_015120.4):c.4247G>A (p.(Arg1416Gln))
-
ALMS1_000881
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+/.
1
-
c.4249del
r.(?)
p.(Arg1417GlyfsTer55)
-
pathogenic (recessive)
g.73677903del
g.73450776del
c.4252del
-
ALMS1_000665
-
PubMed: Sanchez-Navarro 2018
-
-
Germline
-
-
-
-
-
LOVD
-?/.
1
-
c.4291C>A
r.(?)
p.(His1431Asn)
-
likely benign
g.73677945C>A
-
ALMS1(NM_001378454.1):c.4288C>A (p.(His1430Asn))
-
ALMS1_000886
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
?/.
2
-
c.4329G>T
r.(?)
p.(Leu1443Phe)
-
VUS
g.73677983G>T
g.73450856G>T
c.4326G>T (L1442F)
-
ALMS1_000498
11/1266 control chromosomes
PubMed: Xu 2015
-
rs192499639
Germline
-
2/314 case chromosomes
-
-
-
LOVD
?/.
1
-
c.4339C>G
r.(?)
p.(His1447Asp)
-
VUS
g.73677993C>G
g.73450866C>G
ALMS1(NM_015120.4):c.4336C>G (p.H1446D)
-
ALMS1_000805
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Rotterdam
-?/.
1
-
c.4344A>G
r.(?)
p.(Leu1448=)
-
likely benign
g.73677998A>G
g.73450871A>G
ALMS1(NM_015120.4):c.4347A>G (p.L1449=)
-
ALMS1_000806
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht
-?/.
1
-
c.4400G>T
r.(?)
p.(Gly1467Val)
-
likely benign
g.73678054G>T
g.73450927G>T
ALMS1(NM_015120.4):c.4403G>T (p.G1468V)
-
ALMS1_000807
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_AMC
+/.
1
-
c.4474G>T
r.(?)
p.(Glu1492Ter)
-
pathogenic (recessive)
g.73678128G>T
g.73451001G>T
c.4477G>T
-
ALMS1_000666
-
PubMed: Sanchez-Navarro 2018
-
-
Germline
-
-
-
-
-
LOVD
+/.
1
-
c.4566dup
r.(?)
p.(Tyr1523LeufsTer5)
-
pathogenic
g.73678220dup
g.73451093dup
ALMS1 c.4569dup, p.(Arg120Gly)
-
ALMS1_000784
compound heterozygous
PubMed: Bell 2021
-
-
Germline
yes
-
-
-
-
LOVD
+/.
1
-
c.4601C>A
r.(?)
p.(Tyr1534Ter)
-
pathogenic (recessive)
g.73678256C>A
g.73451129C>A
4599C>A
-
ALMS1_000871
-
PubMed: Liu 2015
-
-
Germline
-
-
-
-
-
LOVD
+/.
1
-
c.4645_4661del
r.(?)
p.(Arg1549TrpfsTer4)
ACMG
pathogenic
g.73678299_73678315del
g.73451172_73451188del
ALMS1 NM_015120: g.65413_65429delCAGAGTTTCTTCTGCTC, c.4641_4657delCAGAGTTTCTGCTC, p.R1548Wfs*4
-
ALMS1_000761
different transcript: ENST00000264448.6(ALMS1):c.4642_4658del
PubMed: Xu 2020
-
-
Germline
yes
-
-
-
-
LOVD
?/.
1
-
c.4730C>T
r.(?)
p.(Pro1577Leu)
-
VUS
g.73678384C>T
g.73451257C>T
ALMS1(NM_015120.4):c.4727C>T (p.(Pro1578Leu))
-
ALMS1_000808
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Leiden
+?/.
1
-
c.4743C>G
r.(?)
p.(Tyr1581Ter)
-
likely pathogenic (dominant)
g.73678397C>G
g.73451270C>G
c.4746C>G
-
ALMS1_000610
-
PubMed: Lee 2019
ClinVar-000930568.1
-
Germline
-
-
-
-
-
Johan den Dunnen
+?/.
1
-
c.4747C>T
r.(?)
p.(Gln1583Ter)
-
likely pathogenic
g.73678401C>T
g.73451274C>T
c.4750C>T, p.(Gln1584*)
-
ALMS1_000749
compound heterozygous
PubMed: Wang 2019
-
-
Germline
yes
-
-
-
-
LOVD
?/.
2
-
c.4885C>T
r.(?)
p.(Arg1629Trp)
-
VUS
g.73678539C>T
g.73451412C>T
c.4882C>T (R1628W)
-
ALMS1_000590
1/1266 control chromosomes
PubMed: Xu 2015
-
rs201874722
Germline
-
2/314 case chromosomes
-
-
-
LOVD
+?/.
3
-
c.4888C>T
r.(?)
p.(Gln1630Ter)
-
likely pathogenic, likely pathogenic (recessive)
g.73678542C>T
g.73451415C>T
c.4891C>T, c.4891C>T, p.(Gln1631*)
-
ALMS1_000708
compound heterozygous
PubMed: Wang 2019 , PubMed: Xu 2016 , PubMed: Huang 2016
-
-
Germline
yes
-
-
-
-
Johan den Dunnen
+/., +?/.
2
-
c.4904_4907del
r.(?)
p.(Ser1635IlefsTer2)
-
likely pathogenic, pathogenic
g.73678558_73678561del
g.73451431_73451434del
c.4907_4910del p.L1636fs, c.4907_4910del, p.(Ser1636Ilefs*2)
-
ALMS1_000703
compound heterozygous
PubMed: Wang 2016 , PubMed: Wang 2019
-
-
De novo, Germline
yes
-
-
-
-
LOVD
+?/.
1
-
c.4914_4917del
r.(?)
p.(Asn1638LysfsTer4)
-
likely pathogenic (recessive)
g.73678568_73678571del
g.73451441_73451444del
c.4917_4920del
-
ALMS1_000709
-
PubMed: Xu 2016 , PubMed: Huang 2016
-
-
Germline
-
-
-
-
-
Johan den Dunnen
+/.
2
-
c.5025C>G
r.(?)
p.(Tyr1675Ter)
ACMG
pathogenic
g.73678679C>G
g.73451552C>G
ALMS1 c.[5022C>G];[10825_10826del], V1: c.5022C>G, (p.Tyr1674Ter), 1 more item
-
ALMS1_000789
different transcript NM_015120.2(ALMS1):c.5022C>G, p.(Tyr1674Ter) ; heterozygous, 1 more item
PubMed: Chen 2021 , PubMed: Chen 2021
-
-
Germline, Unknown
?, yes
Taiwan Biobank: 0; GnomAD_exome_East: 0; GnomAD_All: 0
-
-
-
LOVD
-?/.
1
-
c.5118A>G
r.(?)
p.(Ser1706=)
-
likely benign
g.73678772A>G
-
ALMS1(NM_015120.4):c.5121A>G (p.S1707=)
-
ALMS1_000887
VKGL data sharing initiative Nederland
-
-
-
CLASSIFICATION record
-
-
-
-
-
VKGL-NL_Utrecht