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 |
- |
n.*6T>C |
r.*6u>c |
- |
- |
likely pathogenic |
g.35657742A>G |
g.35657745A>G |
*5T>C |
- |
RMRP_000017 |
1 more item |
PubMed: Kavadas et al. 2008 |
- |
- |
SUMMARY record |
yes |
- |
- |
- |
- |
Anne Polvi |
| +?/+? |
1 |
- |
n.-10_-4delCTGAGGAins28 |
r.? |
- |
- |
likely pathogenic |
g.35658019_35658025delins28 |
- |
-11_-5delCTGAGGAins28bp |
- |
RMRP_000024 |
1 CHH family (com-het) with undetermined ethnicity |
PubMed: Kavadas et al. 2008 |
- |
- |
SUMMARY record |
yes |
- |
- |
- |
- |
Anne Polvi |
| +/+ |
1 |
- |
n.-10_-9insTACTCTGTGAAGTACTCTGTGAAGCTGA |
r.0 |
- |
- |
pathogenic |
g.35658024_35658025insTCAGCTTCACAGAGTACTTCACAGAGTA |
g.35658027_35658028insTCAGCTTCACAGAGTACTTCACAGAGTA |
insertion TACTCTGTGAAGTACTCTGTGAAGCTGA at -10 (including 2 times duplication) |
- |
RMRP_000003 |
1 CHH patient |
PubMed: Roifman et al. 2006 |
- |
- |
SUMMARY record |
yes |
- |
- |
- |
- |
Anne Polvi |
| +/+ |
1 |
- |
n.-13_-12insATCTGTG |
r.0 |
- |
- |
pathogenic |
g.35658027_35658028insCACAGAT |
g.35658030_35658031insCACAGAT |
insertion ATCTGTG at -13 |
- |
RMRP_000013 |
1 CHH patient with undetermined ethnicity (com-het) |
PubMed: Roifman et al. 2006 |
- |
- |
SUMMARY record |
yes |
- |
- |
- |
- |
Anne Polvi |
| +?/+? |
1 |
- |
n.-13_-2dupAAGCTGAGGACG |
r.? |
- |
- |
likely pathogenic |
g.35658018_35658029dup |
g.35658021_35658032dup |
dup(aagctgaggacg) at -2; g.-14_-3dupAAGCTGAGGACG |
- |
RMRP_000028 |
1 Swiss and 1 Swiss-Danish CHH family (both com-het) |
PubMed: Bonafe et al. 2002, PubMed: Bonafe et al. 2005 |
- |
- |
SUMMARY record |
yes |
0/60 CON |
- |
- |
- |
Anne Polvi |
| +?/+? |
1 |
- |
n.-13_-6dupAAGCTGAG |
r.? |
- |
- |
likely pathogenic |
g.35658022_35658029dup |
g.35658025_35658032dup |
dup AAGCTGAG at -6 |
- |
RMRP_000020 |
1 Mexican CHH family (com-het) |
PubMed: Ridanpää et al. 2002 |
- |
- |
SUMMARY record |
yes |
- |
- |
- |
- |
Anne Polvi |
| +?/+? |
1 |
- |
n.-13_1dupAAGCTGAGGACGTG |
r.? |
- |
- |
likely pathogenic |
g.35658018_35658031dup |
g.35658021_35658034dup |
dup AAGCTGAGGACGTG at 1 |
- |
RMRP_000036 |
1 American CHH family (com-het) |
PubMed: Ridanpää et al. 2002 |
- |
- |
SUMMARY record |
yes |
0/280 CON |
- |
- |
- |
Anne Polvi |
| +?/+? |
1 |
- |
n.-14_3dupGAAGCTGAGGACGTGGT |
r.? |
- |
- |
likely pathogenic |
g.35658015_35658031dup |
g.35658018_35658034dup |
17-bp duplication (GAAGCTGAGGACGTGGT) at +3; 17-bp dup at +3 |
- |
RMRP_000039 |
6 Japanese CHH families (com-het) |
PubMed: Nakashima et al. 2003, PubMed: Hirose et al. 2006 |
- |
- |
SUMMARY record |
yes |
0/65 JAP CON |
- |
- |
- |
Anne Polvi |
| +?/+? |
1 |
- |
n.-15_-6dupTGAAGCTGAG |
r.? |
- |
- |
likely pathogenic |
g.35658022_35658031dup |
g.35658025_35658034dup |
dup TGAAGCTGAG at -6 |
- |
RMRP_000032 |
1 French CHH family (com-het) |
PubMed: Ridanpää et al. 2002 |
- |
- |
SUMMARY record |
yes |
- |
- |
- |
- |
Anne Polvi |
| +?/+? |
1 |
- |
n.-15_1dupTGAAGCTGAGGACGTG |
r.? |
- |
- |
likely pathogenic |
g.35658017_35658032dup |
g.35658020_35658035dup |
16-bp dup at +1 |
- |
RMRP_000035 |
1 Japanese CHH family (com-het) |
PubMed: Hirose et al. 2006 |
- |
- |
SUMMARY record |
yes |
0/65 JAP CON |
- |
- |
- |
Anne Polvi |
| +?/+? |
1 |
- |
n.-18_-17insCTCACTACTC |
r.? |
- |
- |
likely pathogenic |
g.35658039_35658040insGAGGAGTAGT |
g.35658042_35658043insGAGGAGTAGT |
M29916.1: g.726_727insCTCACTACTC |
- |
RMRP_000103 |
1 more item |
PubMed: Crahes et al. 2013 |
- |
- |
SUMMARY record |
yes |
- |
- |
- |
- |
Anne Polvi |
| +?/+? |
1 |
- |
n.-19_-13dupTCTGTGA |
r.? |
- |
- |
likely pathogenic |
g.35658028_35658034dup |
g.35658031_35658037dup |
dup TCTGTGA at -13; g.-20_-14dupTCTGTGA; -14_-20dupTCTGTGA; -20-14dup |
- |
RMRP_000011 |
1 more item |
1 more item |
- |
- |
SUMMARY record |
yes |
0/280 CON |
- |
- |
- |
Anne Polvi |
| +?/+? |
1 |
- |
n.-19_-3dupTCTGTGAAGCTGAGGAC |
r.? |
- |
- |
likely pathogenic |
g.35658020_35658036dup |
g.35658023_35658039dup |
dupTCTGTGAAGCTGAGGAC at g.-3; -20_-4dupTCTGTGAAGCTGAGGAC |
- |
RMRP_000026 |
1 English and 1 Swiss CHH family (both com-het) |
PubMed: Ridanpää et al. 2001, PubMed: Ridanpää et al. 2002, PubMed: Bonafe et al. 2005 |
- |
- |
SUMMARY record |
yes |
0/280 CON |
- |
- |
- |
Anne Polvi |
| +?/+? |
1 |
- |
n.-20_-13CTCTGTGA[3] |
r.? |
- |
- |
likely pathogenic |
g.35658028_35658035[3] |
- |
-21_-14trip |
- |
RMRP_000021 |
1 CHH family (com-het) with undetermined ethnicity |
PubMed: Kavadas et al. 2008 |
- |
- |
SUMMARY record |
yes |
- |
- |
- |
- |
Anne Polvi |
| +/+ |
1 |
- |
n.-20_-19insTCTGTGAAGCTGGGGAC |
r.0 |
- |
- |
pathogenic |
g.35658036_35658037insCCCCAGCTTCACAGAGT |
g.35658039_35658040insCCCCAGCTTCACAGAGT |
n.-21_-20insTCTGTGAAGCTGGGGAC |
- |
RMRP_000001 |
2 Japanese CHH families (com-het) |
PubMed: Nakashima et al. 2003, PubMed: Hirose et al. 2006 |
- |
- |
SUMMARY record |
yes |
0/65 JAP CON |
- |
- |
- |
Anne Polvi |
| +?/+? |
1 |
- |
n.-20_1dupCTCTGTGAAGCTGAGGACGTG |
r.? |
- |
- |
likely pathogenic |
g.35658015_35658035dup |
g.35658018_35658038dup |
-1_-21dupCTCTGTGAAGCTGAGGACGTG |
- |
RMRP_000034 |
1 American CHH patient (com-het) |
PubMed: Hermanns el al. 2006 |
- |
- |
SUMMARY record |
yes |
- |
- |
- |
- |
Anne Polvi |
| +?/+? |
1 |
- |
n.-21_-9dupACTCTGTGAAGCT |
r.? |
- |
- |
VUS |
g.35658026_35658038dup |
g.35658029_35658041dup |
g.-22_-10dupACTCTGTGAAGCT |
- |
RMRP_000015 |
1 French CHH family (com-het) |
PubMed: Bonafe et al. 2005 |
- |
- |
SUMMARY record |
yes |
0/100 CAU CON |
- |
- |
- |
Anne Polvi |
| +?/+? |
1 |
- |
n.-22-6dupTACTCTGTGAAGCTGAG |
r.? |
- |
- |
likely pathogenic |
g.35658021_35658037dup |
g.35658024_35658040dup |
-23-8dupTACTCTGTGAAGCTGAG |
- |
RMRP_000016 |
1 CHH patient (com-het) with undetermined ethnicity |
PubMed: Fuente et al. 2011 |
- |
- |
SUMMARY record |
yes |
- |
- |
- |
- |
Anne Polvi |
| +?/+? |
1 |
- |
n.-22_-13dupTACTCTGTGA |
r.? |
- |
- |
likely pathogenic |
g.35658028_35658037dup |
g.35658031_35658040dup |
dupTACTCTGTGA at -13; g.-23_-14dupTACTCTGTGA |
- |
RMRP_000004 |
2 Finnish CHH families (com-het) and 1 French CHH family (com-het) |
PubMed: Ridanpää et al. 2002, PubMed: Bonafe et al. 2005 |
- |
- |
SUMMARY record |
yes |
0/280 CON |
- |
- |
- |
Anne Polvi |
| +?/+? |
1 |
- |
n.-22_-14dupTACTCTGTG |
r.? |
- |
- |
likely pathogenic |
g.35658029_35658037dup |
g.35658032_35658040dup |
g.-23_-15dupTACTCTGTG |
- |
RMRP_000010 |
1 French CHH family (com-het) |
PubMed: Bonafe et al. 2005 |
- |
- |
SUMMARY record |
yes |
0/100 CAU CON |
- |
- |
- |
Anne Polvi |
| +?/+? |
1 |
- |
n.-22_-3dupTACTCTGTGAAGCTGAGGAC |
r.? |
- |
- |
likely pathogenic |
g.35658020_35658039dup |
g.35658023_35658042dup |
20 bp duplication (TACTCTGTGAAGCTGAGGAC) at nt -3; n.-4_-23dupTACTCTGTGAAGCTGAGGAC |
- |
RMRP_000025 |
1 Japanese CHH family and 1 German CHH patient (all com-het) |
PubMed: Harada et al. 2005, PubMed: Hermanns el al. 2006 |
- |
- |
SUMMARY record |
yes |
- |
- |
- |
- |
Anne Polvi |
| +?/+? |
1 |
- |
n.-23_-14dupCTACTCTGTG |
r.? |
- |
- |
likely pathogenic |
g.35658029_35658038dup |
g.35658032_35658041dup |
-15_-24dupCTACTCTGTG |
- |
RMRP_000009 |
1 American CHH patient (com-het) |
PubMed: Hermanns el al. 2006 |
- |
- |
SUMMARY record |
yes |
- |
- |
- |
- |
Anne Polvi |
| +/+ |
1 |
- |
n.-24_-10ACTACTCTGTGAAGC[3] |
r.0 |
- |
- |
pathogenic |
g.35658025_35658039[3] |
- |
Two times dupACTACTCTGTGAAGC at -10 |
- |
RMRP_000030 |
1 Swiss CHH family (com-het) |
PubMed: Ridanpää et al. 2001, PubMed: Ridanpää et al. 2002 |
- |
- |
SUMMARY record |
yes |
- |
- |
- |
- |
Anne Polvi |
| +?/+? |
1 |
- |
n.-24_-10dupACTACTCTGTGAAGC |
r.? |
- |
- |
likely pathogenic |
g.35658025_35658039dup |
g.35658028_35658042dup |
g.-25_-11 dupACTACTCTGTGAAGC; -25_-11dup; -11_-25dupACTACTCTGTGAAGC |
- |
RMRP_000014 |
1 German and 1 Spanish CHH patient and 1 CHH family with undetermined ethnicity (all com-het) |
PubMed: Hermanns el al. 2006, PubMed: Munoz-Robles et al. 2006, PubMed: Kavadas et al. 2008 |
- |
- |
SUMMARY record |
yes |
- |
- |
- |
- |
Anne Polvi |
| +?/+? |
1 |
- |
n.-24_-12dup |
r.? |
- |
- |
likely pathogenic |
g.35658027_35658039dup |
g.35658030_35658042dup |
-25_-13dup |
- |
RMRP_000012 |
1 CHH family (com-het) with undetermined ethnicity |
PubMed: Kavadas et al. 2008 |
- |
- |
SUMMARY record |
yes |
- |
- |
- |
- |
Anne Polvi |
| +?/+? |
1 |
- |
n.-24_-18dupACTACTC |
r.? |
- |
- |
likely pathogenic |
g.35658033_35658039dup |
g.35658036_35658042dup |
g.-19_-25 dupACTACTC |
- |
RMRP_000002 |
1 Thai CHH patient (hom) |
PubMed: Vatanavicharn et al. 2010 |
- |
- |
SUMMARY record |
yes |
- |
- |
- |
- |
Anne Polvi |
| +?/+? |
1 |
- |
n.-24_-4dupACTACTCTGTGAAGCTGAGGA |
r.? |
- |
- |
likely pathogenic |
g.35658019_35658039dup |
g.35658022_35658042dup |
g.-25_-5dupACTACTCTGTGAAGCTGAGGA; -25_-5dup; 6_-25dupACTACTCTGTGAAGCTGAGA |
- |
RMRP_000023 |
1 German CHH family and 2 Belgian siblings, 1 CHH family with undetermined ethnicity (all com-het) |
PubMed: Bonafe et al. 2005, PubMed: Hermanns el al. 2006, PubMed: Kavadas et al. 2008 |
- |
- |
SUMMARY record |
yes |
0/100 CAU CON |
- |
- |
- |
Anne Polvi |
| +?/+? |
1 |
- |
n.-24_-9ACTACTCTGTGAAGCT[3] |
r.? |
- |
- |
likely pathogenic |
g.35658024_35658039[3] |
- |
g.-25_-10tripACTACTCTGTGAAGCT |
- |
RMRP_000031 |
1 Italian CHH family (com-het) |
PubMed: Bonafe et al. 2005 |
- |
- |
SUMMARY record |
yes |
0/100 CAU CON |
- |
- |
- |
Anne Polvi |
| +?/+? |
1 |
- |
n.-25_-4dupTACTACTCTGTGAAGCTGAGGA |
r.? |
- |
- |
likely pathogenic |
g.35658020_35658041dup |
g.35658023_35658044dup |
-5_-26dupTACTACTCTGTGAAGCTGAGAA |
- |
RMRP_000022 |
1 American CHH patient (com-het) |
PubMed: Hermanns el al. 2006 |
- |
- |
SUMMARY record |
yes |
- |
- |
- |
- |
Anne Polvi |
| +?/+? |
1 |
- |
n.-3_1dupCGTG |
r.? |
- |
- |
likely pathogenic |
g.35658015_35658018dup |
g.35658018_35658021dup |
-4_-1dup |
- |
RMRP_000038 |
1 CHH family (com-het) with undetermined ethnicity |
PubMed: Kavadas et al. 2008 |
- |
- |
SUMMARY record |
yes |
- |
- |
- |
- |
Anne Polvi |
| +?/+? |
1 |
- |
n.-4_-3insGGACGTGGTT |
r.? |
- |
- |
likely pathogenic |
g.35658018_35658019insAACCACGTCC |
g.35658021_35658022insAACCACGTCC |
ins GGACGTGGTT at -4 |
- |
RMRP_000027 |
1 French CHH family (com-het) |
PubMed: Ridanpää et al. 2002 |
- |
- |
SUMMARY record |
yes |
0/280 CON |
- |
- |
- |
Anne Polvi |
| +/+ |
1 |
- |
n.-6_-5insCCTGAG |
r.0 |
- |
- |
pathogenic |
g.35658025_35658026insGCTCAG |
g.35658028_35658029insGCTCAG |
insCCTGAG at -6 |
- |
RMRP_000033 |
1 German CHH family (com-het) |
PubMed: Ridanpää et al. 2001, PubMed: Ridanpää et al. 2002 |
- |
- |
SUMMARY record |
yes |
0/280 CON |
- |
- |
- |
Anne Polvi |
| +?/+? |
1 |
- |
n.-6_4dupGGACGTGGTT |
r.? |
- |
- |
likely pathogenic |
g.35658012_35658021dup |
g.35658015_35658024dup |
dup GGACGTGGTT at 4 |
- |
RMRP_000040 |
1 Brazilian CHH family (com-het) |
PubMed: Ridanpää et al. 2002 |
- |
- |
SUMMARY record |
yes |
0/280 CON |
- |
- |
- |
Anne Polvi |
| +?/+? |
1 |
- |
n.-7_1dupAGGACGTG |
r.? |
- |
- |
likely pathogenic |
g.35658017_35658024dup |
g.35658020_35658027dup |
g.-8_-1dupAGGACGTG |
- |
RMRP_000037 |
1 Canadian CHH family (com-het) |
PubMed: Bonafe et al. 2005 |
- |
- |
SUMMARY record |
yes |
0/100 CAU CON |
- |
- |
- |
Anne Polvi |
| +?/+? |
1 |
- |
n.-8_-1dupGAGGACGT |
r.? |
- |
- |
likely pathogenic |
g.35658017_35658024dup |
g.35658020_35658027dup |
-9_-2dup |
- |
RMRP_000029 |
1 CHH family (com-het) with undetermined ethnicity |
PubMed: Kavadas et al. 2008 |
- |
- |
SUMMARY record |
yes |
- |
- |
- |
- |
Anne Polvi |
| +?/+? |
1 |
- |
n.5C>T |
r.5c>u |
- |
- |
likely pathogenic |
g.35658011G>A |
g.35658014G>A |
4T; g.4C>T; 4C>T |
- |
RMRP_000018 |
1 more item |
1 more item |
- |
- |
SUMMARY record |
yes |
0/280 CON |
- |
- |
- |
Anne Polvi |
| +?/+? |
1 |
- |
n.10T>C |
r.10u>c |
- |
- |
likely pathogenic |
g.35658006A>G |
g.35658009A>G |
9T>C |
- |
RMRP_000019 |
1 German CHH patient (com-het) |
PubMed: Hermanns el al. 2006 |
- |
- |
SUMMARY record |
yes |
- |
- |
- |
- |
Anne Polvi |
| ?/? |
1 |
- |
n.12A>G |
r.12a>g |
- |
- |
VUS |
g.35658004T>C |
g.35658007T>C |
g.11A>G reported previously as polymorphisms |
- |
RMRP_000041 |
- |
PubMed: Kwan et al. 2012 |
- |
- |
SUMMARY record |
yes |
- |
- |
- |
- |
Anne Polvi |
| +?/+? |
1 |
- |
n.15G>T |
r.15g>u |
- |
- |
likely pathogenic |
g.35658001C>A |
g.35658004C>A |
14G>T |
- |
RMRP_000042 |
1 American CHH patient (com-het) |
PubMed: Hermanns el al. 2006 |
- |
- |
SUMMARY record |
yes |
- |
- |
- |
- |
Anne Polvi |
| +?/+? |
1 |
- |
n.19G>C |
r.19g>c |
- |
- |
likely pathogenic |
g.35657997C>G |
g.35658000C>G |
g.G18>C |
- |
RMRP_000043 |
1 CHH patient (com-het) with undetermined ethnicity |
PubMed: Fuente et al. 2011 |
- |
- |
SUMMARY record |
yes |
- |
- |
- |
- |
Anne Polvi |
| +?/+? |
1 |
- |
n.28G>A |
r.28g>a |
- |
- |
likely pathogenic |
g.35657988C>T |
g.35657991C>T |
g.G27>A |
- |
RMRP_000044 |
1 CHH patient (hom) with undetermined ethnicity |
PubMed: Fuente et al. 2011 |
- |
- |
SUMMARY record |
yes |
- |
- |
- |
- |
Anne Polvi |
| +?/+? |
1 |
- |
n.36C>T |
r.36c>u |
- |
- |
likely pathogenic |
g.35657980G>A |
g.35657983G>A |
g.35C>T |
- |
RMRP_000045 |
1 French CHH family (com-het) |
PubMed: Bonafe et al. 2005 |
- |
- |
SUMMARY record |
yes |
0/100 CAU CON |
- |
- |
- |
Anne Polvi |
| +?/+? |
1 |
- |
n.41G>A |
r.41g>a |
- |
- |
likely pathogenic |
g.35657975C>T |
g.35657978C>T |
g.40G>A |
- |
RMRP_000046 |
1 Dutch CHH family (com-het) |
PubMed: Bonafe et al. 2005 |
- |
- |
SUMMARY record |
yes |
0/100 CAU CON |
- |
- |
- |
Anne Polvi |
| +?/+? |
1 |
- |
n.46_54dupTGTTCCTCC |
r.46_54dupuguuccucc |
- |
- |
likely pathogenic |
g.35657962_35657970dup |
g.35657965_35657973dupGGAGGAACA |
g.45_53dupTGTTCCTCC |
- |
RMRP_000047 |
1 more item |
PubMed: Bonafe et al. 2005 |
- |
- |
SUMMARY record |
yes |
0/100 CAU CON |
- |
- |
- |
Anne Polvi |
| ?/. |
1 |
- |
n.47G>A |
r.(?) |
- |
- |
VUS |
g.35657969C>T |
g.35657972C>T |
RMRP(NR_003051.3):n.47G>A |
- |
CA9_000010 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Rotterdam |
| -?/. |
3 |
- |
n.54C>T |
r.(?) |
- |
- |
likely benign |
g.35657962G>A |
g.35657965G>A |
RMRP(NR_003051.3):n.54C>T |
- |
CA9_000019 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Rotterdam, VKGL-NL_Groningen, VKGL-NL_Utrecht |
| +?/+? |
1 |
- |
n.57_58insTTCCGCCT |
r.57_58insuuccgccu |
- |
- |
likely pathogenic |
g.35657958_35657959insAGGCGGAA |
g.35657961_35657962insAGGCGGAA |
ins TTCCGCCT at 57 |
- |
RMRP_000048 |
1 more item |
PubMed: Ridanpää et al. 2002 |
- |
- |
SUMMARY record |
yes |
0/280 CON |
- |
- |
- |
Anne Polvi |
| ?/. |
1 |
- |
n.58_59insA |
r.(?) |
- |
- |
VUS |
g.35657957_35657958insT |
g.35657960_35657961insT |
RMRP(NR_003051.3):n.58_59insA |
- |
CA9_000009 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Rotterdam |
| +?/+? |
1 |
- |
n.62G>A |
r.62g>a |
- |
- |
likely pathogenic |
g.35657954C>T |
g.35657957C>T |
g.G61>A |
- |
RMRP_000049 |
1 CHH patient (com-het) with undetermined ethnicity |
PubMed: Fuente et al. 2011 |
- |
- |
SUMMARY record |
yes |
- |
- |
- |
- |
Anne Polvi |
| +/., +?/+? |
3 |
- |
n.64C>T |
r.(?), r.64c>u |
- |
- |
likely pathogenic, pathogenic |
g.35657952G>A |
g.35657955G>A |
63T; g.63C>T, RMRP(NR_003051.3):n.64C>T |
- |
RMRP_000050 |
VKGL data sharing initiative Nederland, 1 more item |
PubMed: Ridanpää et al. 2002, PubMed: Bonafe et al. 2005, PubMed: Kuijpers et al. 2003 |
- |
- |
CLASSIFICATION record, SUMMARY record |
yes |
0/280 CON |
- |
- |
- |
Anne Polvi, VKGL-NL_Rotterdam, VKGL-NL_Nijmegen |
| +?/+? |
1 |
- |
n.65T>C |
r.65u>c |
- |
- |
likely pathogenic |
g.35657951A>G |
g.35657954A>G |
g.64T>C |
- |
RMRP_000051 |
1 Italian CHH family (com-het) |
PubMed: Bonafe et al. 2005 |
- |
- |
SUMMARY record |
yes |
0/100 CAU CON |
- |
- |
- |
Anne Polvi |
| ?/. |
1 |
- |
n.69G>T |
r.(?) |
- |
- |
VUS |
g.35657947C>A |
g.35657950C>A |
RMRP(NR_003051.3):n.69G>T |
- |
CA9_000008 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Rotterdam |
| +?/+? |
1 |
- |
n.69_70delinsTT |
r.69_70delinsuu |
- |
- |
likely pathogenic |
g.35657946_35657947delinsAA |
g.35657949_35657950delinsAA |
g.68_69delinsTT |
- |
RMRP_000052 |
1 CHH patient (com-het) with undetermined ethnicity |
PubMed: Horn et al. 2010 |
- |
- |
SUMMARY record |
yes |
- |
- |
- |
- |
Anne Polvi |
| ?/. |
1 |
- |
n.70G>T |
r.(?) |
- |
- |
VUS |
g.35657946C>A |
g.35657949C>A |
RMRP(NR_003051.3):n.70G>T |
- |
CA9_000007 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Rotterdam |
| +/+, +/., +?/. |
4 |
- |
n.71A>G |
r.(?), r.71a>g |
-, p.0 |
- |
likely pathogenic, pathogenic |
g.35657945T>C |
g.35657948T>C |
70A>G, RMPR c.71A>G (RNA gene - no protein produced), RMRP(NR_003051.3):n.71A>G |
- |
RMRP_000005 |
homozygous, VKGL data sharing initiative Nederland, 1 more item |
PubMed: Alabdullatif 2017, 1 more item |
- |
- |
CLASSIFICATION record, Germline, SUMMARY record |
yes |
10/845 FIN CON |
- |
- |
- |
Anne Polvi, VKGL-NL_Rotterdam, VKGL-NL_Nijmegen |
| +?/+? |
1 |
- |
n.77C>T |
r.77c>u |
- |
- |
likely pathogenic |
g.35657939G>A |
g.35657942G>A |
g.76C>T |
- |
RMRP_000053 |
1 CHH patient (com-het) with undetermined ethnicity |
PubMed: Horn et al. 2010 |
- |
- |
SUMMARY record |
yes |
- |
- |
- |
- |
Anne Polvi |
| +?/+? |
1 |
- |
n.78C>T |
r.78c>u |
- |
- |
likely pathogenic |
g.35657938G>A |
g.35657941G>A |
g.77C>T |
- |
RMRP_000054 |
1 CHH patient (com-het) with undetermined ethnicity |
PubMed: Bacchetta et al. 2009 |
- |
- |
SUMMARY record |
yes |
- |
- |
- |
- |
Anne Polvi |
| +?/+? |
1 |
- |
n.80G>A |
r.80g>a |
- |
- |
likely pathogenic |
g.35657936C>T |
g.35657939C>T |
79A |
- |
RMRP_000055 |
1 American CHH family (com-het) |
PubMed: Ridanpää et al. 2002 |
- |
- |
SUMMARY record |
yes |
0/280 CON |
- |
- |
- |
Anne Polvi |
| +?/+? |
1 |
- |
n.81G>A |
r.81g>a |
- |
- |
likely pathogenic |
g.35657935C>T |
g.35657938C>T |
80G>A |
- |
RMRP_000056 |
1 American CHH patient (com-het) |
PubMed: Hermanns el al. 2006 |
- |
- |
SUMMARY record |
yes |
- |
- |
- |
- |
Anne Polvi |
| +?/+? |
1 |
- |
n.90C>G |
r.90c>g |
- |
- |
likely pathogenic |
g.35657926G>C |
g.35657929G>C |
89C>G |
- |
RMRP_000057 |
1 American CHH patient (com-het) |
PubMed: Hermanns el al. 2006 |
- |
- |
SUMMARY record |
yes |
- |
- |
- |
- |
Anne Polvi |
| +?/+? |
1 |
- |
n.92G>A |
r.92g>a |
- |
- |
likely pathogenic |
g.35657924C>T |
g.35657927C>T |
91G>A |
- |
RMRP_000058 |
1 more item |
PubMed: Hermanns el al. 2006 |
- |
- |
SUMMARY record |
yes |
- |
- |
- |
- |
Anne Polvi |
| +?/+? |
1 |
- |
n.93dupA |
r.93dupa |
- |
- |
likely pathogenic |
g.35657923dup |
g.35657926dupT |
g.92_93insA |
- |
RMRP_000059 |
1 more item |
PubMed: Bonafe et al. 2005 |
- |
- |
SUMMARY record |
yes |
0/100 CAU CON |
- |
- |
- |
Anne Polvi |
| +?/+? |
1 |
- |
n.94G>C |
r.94g>c |
- |
- |
likely pathogenic |
g.35657922C>G |
g.35657925C>G |
g.93G>C |
- |
RMRP_000060 |
1 Dutch CHH family (com-het) |
PubMed: Bonafe et al. 2005 |
- |
- |
SUMMARY record |
yes |
0/100 CAU CON |
- |
- |
- |
Anne Polvi |
| +?/+? |
1 |
- |
n.95_96delAG |
r.95_96delag |
- |
- |
likely pathogenic |
g.35657920_35657921delCT |
g.35657923_35657924delCT |
del AG at 94 |
- |
RMRP_000061 |
1 more item |
PubMed: Ridanpää et al. 2002 |
- |
- |
SUMMARY record |
yes |
0/280 CON |
- |
- |
- |
Anne Polvi |
| ?/. |
1 |
- |
n.96G>T |
r.(?) |
- |
- |
VUS |
g.35657920C>A |
g.35657923C>A |
RMRP(NR_003051.3):n.96G>T |
- |
CA9_000018 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Rotterdam |
| +?/+? |
1 |
- |
n.97_98dupTG |
r.97_98dupug |
- |
- |
likely pathogenic |
g.35657918_35657919dup |
g.35657921_35657922dupCA |
98dupTG; dup TG at 98; g.96_97dupTG |
- |
RMRP_000063 |
1 more item |
PubMed: Ridanpää et al. 2002, PubMed: Ridanpää et al. 2001, PubMed: Bonafe et al. 2005 |
- |
- |
SUMMARY record |
yes |
0/280 CON |
- |
- |
- |
Anne Polvi |
| +?/+? |
1 |
- |
n.98G>A |
r.98g>a |
- |
- |
likely pathogenic |
g.35657918C>T |
g.35657921C>T |
g.97G>A |
- |
RMRP_000062 |
1 German CHH family and 1 American CHH patient (all com-het) |
PubMed: Bonafe et al. 2005, PubMed: Hermanns el al. 2006 |
- |
- |
SUMMARY record |
yes |
0/100 CAU CON |
- |
- |
- |
Anne Polvi |
| +?/. |
1 |
- |
n.100C>T |
r.(?) |
- |
- |
likely pathogenic |
g.35657916G>A |
- |
RMRP(NR_003051.3):n.100C>T |
- |
RMRP_000105 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Groningen |
| +/., +?/+? |
2 |
- |
n.102C>T |
r.(?), r.102c>u |
- |
- |
likely pathogenic, pathogenic |
g.35657914G>A |
g.35657917G>A |
101C>T |
- |
RMRP_000102 |
VKGL data sharing initiative Nederland, 1 more item |
PubMed: Hermanns el al. 2006 |
- |
- |
CLASSIFICATION record, SUMMARY record |
yes |
- |
- |
- |
- |
Anne Polvi, VKGL-NL_Nijmegen |
| ?/. |
1 |
- |
n.110C>T |
r.(?) |
- |
- |
VUS |
g.35657906G>A |
- |
RMRP(NR_003051.3):n.110C>T |
- |
RMRP_000110 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Groningen |
| +?/+? |
1 |
- |
n.117A>G |
r.117a>g |
- |
- |
likely pathogenic |
g.35657899T>C |
g.35657902T>C |
116A>G |
- |
RMRP_000064 |
1 American CHH patient (com-het) |
PubMed: Hermanns el al. 2006 |
- |
- |
SUMMARY record |
yes |
- |
- |
- |
- |
Anne Polvi |
| +?/+? |
1 |
- |
n.119A>G |
r.119a>g |
- |
- |
likely pathogenic |
g.35657897T>C |
g.35657900T>C |
118G |
- |
RMRP_000065 |
1 German CHH patient (com-het) |
PubMed: Ridanpää et al. 2002 |
- |
- |
SUMMARY record |
yes |
0/280 CON |
- |
- |
- |
Anne Polvi |
| +?/+? |
1 |
- |
n.125C>T |
r.125c>u |
- |
- |
likely pathogenic |
g.35657891G>A |
g.35657894G>A |
124C>T |
- |
RMRP_000066 |
1 American CHH patient (com-het) |
PubMed: Hermanns el al. 2006 |
- |
- |
SUMMARY record |
yes |
- |
- |
- |
- |
Anne Polvi |
| +/., +?/+? |
2 |
- |
n.127C>T |
r.(?), r.127c>u |
- |
- |
likely pathogenic, pathogenic |
g.35657889G>A |
g.35657892G>A |
126T; g126C>T |
- |
RMRP_000067 |
1 Arabian CHH family (hom), 1 Italian and 1 Turkish CHH family (both com-het), 1 more item |
PubMed: Ridanpää et al. 2002, PubMed: Bonafe et al. 2005 |
- |
- |
CLASSIFICATION record, SUMMARY record |
yes |
0/280 CON |
- |
- |
- |
Anne Polvi, VKGL-NL_Nijmegen |
| +?/+? |
1 |
- |
n.128G>A |
r.128g>a |
- |
- |
likely pathogenic |
g.35657888C>T |
g.35657891C>T |
g.127G>A |
- |
RMRP_000068 |
1 Italian and 1 Canadian CHH family (both com-het) |
PubMed: Bonafe et al. 2005 |
- |
- |
SUMMARY record |
yes |
0/100 CAU CON |
- |
- |
- |
Anne Polvi |
| -?/., ?/? |
2 |
- |
n.128G>C |
r.(?), r.128g>c |
- |
- |
likely benign, VUS |
g.35657888C>G |
g.35657891C>G |
127G>C, RMRP(NR_003051.3):n.128G>C |
- |
RMRP_000069 |
1 American CHH patient (het), VKGL data sharing initiative Nederland |
PubMed: Hermanns el al. 2006 |
- |
- |
CLASSIFICATION record, SUMMARY record |
yes |
0/200 CON |
- |
- |
- |
Anne Polvi, VKGL-NL_Rotterdam |
| ?/. |
1 |
- |
n.146C>T |
r.(?) |
- |
- |
VUS |
g.35657870G>A |
- |
RMRP(NR_003051.3):n.146C>T |
- |
RMRP_000108 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Groningen |
| +/., +?/+? |
2 |
- |
n.147G>A |
r.(?), r.147g>a |
- |
- |
likely pathogenic, pathogenic |
g.35657869C>T |
g.35657872C>T |
146A; g.146G>A, RMRP(NR_003051.3):n.147G>A |
- |
RMRP_000070 |
VKGL data sharing initiative Nederland, 1 more item |
PubMed: Ridanpää et al. 2002, PubMed: Bonafe et al. 2005, PubMed: Kavadas et al. 2008 |
- |
- |
CLASSIFICATION record, SUMMARY record |
yes |
0/280 CON |
- |
- |
- |
Anne Polvi, VKGL-NL_Rotterdam |
| +?/+? |
1 |
- |
n.147G>C |
r.147g>c |
- |
- |
likely pathogenic |
g.35657869C>G |
g.35657872C>G |
g.146G>C |
- |
RMRP_000071 |
1 Italian CHH family (com-het) |
PubMed: Bonafe et al. 2005 |
- |
- |
SUMMARY record |
yes |
0/100 CAU CON |
- |
- |
- |
Anne Polvi |
| +?/+? |
1 |
- |
n.153A>G |
r.153a>g |
- |
- |
likely pathogenic |
g.35657863T>C |
g.35657866T>C |
152G |
- |
RMRP_000072 |
1 Canadian CHH family (com-het) |
PubMed: Ridanpää et al. 2002 |
- |
- |
SUMMARY record |
yes |
0/280 CON |
- |
- |
- |
Anne Polvi |
| +?/+? |
1 |
- |
n.155G>C |
r.155g>c |
- |
- |
likely pathogenic |
g.35657861C>G |
g.35657864C>G |
154G>C |
- |
RMRP_000073 |
1 CHH family (com-het) with undetermined ethnicity |
PubMed: Kavadas et al. 2008 |
- |
- |
SUMMARY record |
yes |
- |
- |
- |
- |
Anne Polvi |
| +?/+? |
1 |
- |
n.155G>T |
r.155g>u |
- |
- |
likely pathogenic |
g.35657861C>A |
g.35657864C>A |
154T |
- |
RMRP_000006 |
1 Finnish CHH family (com-het) |
PubMed: Ridanpää et al. 2002 |
- |
- |
SUMMARY record |
yes |
0/280 CON |
- |
- |
- |
Anne Polvi |
| -/., -?/. |
3 |
- |
n.157G>C |
r.(?) |
- |
- |
benign, likely benign |
g.35657859C>G |
g.35657862C>G |
RMRP(NR_003051.3):n.157G>C |
- |
CA9_000006 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Groningen, VKGL-NL_Utrecht, VKGL-NL_Nijmegen |
| ?/. |
1 |
- |
n.159A>G |
r.(?) |
- |
- |
VUS |
g.35657857T>C |
g.35657860T>C |
RMRP(NR_003051.3):n.159A>G |
- |
CA9_000017 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Rotterdam |
| +?/+? |
1 |
- |
n.169G>A |
r.169g>a |
- |
- |
likely pathogenic |
g.35657847C>T |
g.35657850C>T |
168G>A |
- |
RMRP_000075 |
1 Japanese CHH family (com-het) |
PubMed: Hirose et al. 2006 |
- |
- |
SUMMARY record |
yes |
0/65 JAP CON |
- |
- |
- |
Anne Polvi |
| ?/. |
1 |
- |
n.176G>A |
r.(?) |
- |
- |
VUS |
g.35657840C>T |
- |
RMRP(NR_003051.3):n.176G>A |
- |
RMRP_000107 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Groningen |
| -/. |
3 |
- |
n.178C>T |
r.(?) |
- |
- |
benign |
g.35657838G>A |
g.35657841G>A |
RMRP(NR_003051.3):n.178C>T |
- |
CA9_000005 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Groningen, VKGL-NL_Utrecht, VKGL-NL_Nijmegen |
| +?/+? |
1 |
- |
n.180_181insC |
r.180_181insc |
- |
- |
likely pathogenic |
g.35657835_35657836insG |
g.35657838_35657839insG |
179_180insC |
- |
RMRP_000077 |
1 more item |
PubMed: Hermanns el al. 2006 |
- |
- |
SUMMARY record |
yes |
- |
- |
- |
- |
Anne Polvi |
| +?/+? |
1 |
- |
n.181G>A |
r.181g>a |
- |
- |
likely pathogenic |
g.35657835C>T |
g.35657838C>T |
180A |
- |
RMRP_000076 |
2 Mexican CHH family, 2 German and 2 American CHH patients (all com-het) |
PubMed: Ridanpää et al. 2002, PubMed: Hermanns el al. 2006 |
- |
- |
SUMMARY record |
yes |
0/280 CON |
- |
- |
- |
Anne Polvi |
| +?/+? |
1 |
- |
n.183G>A |
r.183g>a |
- |
- |
likely pathogenic |
g.35657833C>T |
g.35657836C>T |
182G>A |
- |
RMRP_000078 |
1 Japanese CHH family (com-het) |
PubMed: Nakashima et al. 2003, PubMed: Hirose et al. 2006 |
- |
- |
SUMMARY record |
yes |
0/65 JAP CON |
- |
- |
- |
Anne Polvi |
| +?/+? |
1 |
- |
n.183G>C |
r.183g>c |
- |
- |
likely pathogenic |
g.35657833C>G |
g.35657836C>G |
182C |
- |
RMRP_000079 |
1 Dutch, 1 English and 1 Japanese CHH family (all com-het) |
PubMed: Ridanpää et al. 2002, PubMed: Hirose et al. 2006 |
- |
- |
SUMMARY record |
yes |
0/280 CON |
- |
- |
- |
Anne Polvi |
| +?/+? |
1 |
- |
n.183G>T |
r.183g>u |
- |
- |
likely pathogenic |
g.35657833C>A |
g.35657836C>A |
g.182G>T |
- |
RMRP_000080 |
1 German CHH family and 1 German CHH patient (all com-het) |
PubMed: Bonafe et al. 2005, PubMed: Hermanns el al. 2006 |
- |
- |
SUMMARY record |
yes |
0/100 CAU CON |
- |
- |
- |
Anne Polvi |
| +?/+? |
1 |
- |
n.194G>A |
r.194g>a |
- |
- |
likely pathogenic |
g.35657822C>T |
g.35657825C>T |
193G>A; 193A; g.193G>A |
- |
RMRP_000081 |
1 more item |
1 more item |
- |
- |
SUMMARY record |
yes |
0/205 CON |
- |
- |
- |
Anne Polvi |
| +?/+? |
1 |
- |
n.195dupT |
r.195dupu |
- |
- |
likely pathogenic |
g.35657821dup |
g.35657824dupA |
InsT195; 194-195insT |
- |
RMRP_000082 |
1 more item |
PubMed: Kuijpers et al. 2003, PubMed: Hermanns el al. 2006 |
- |
- |
SUMMARY record |
yes |
- |
- |
- |
- |
Anne Polvi |
| +?/+? |
1 |
- |
n.196C>T |
r.196c>u |
- |
- |
likely pathogenic |
g.35657820G>A |
g.35657823G>A |
195T; 195C>T; g.195C>T |
- |
RMRP_000083 |
1 more item |
1 more item |
- |
- |
SUMMARY record |
yes |
0/280 CON |
- |
- |
- |
Anne Polvi |
| +?/+? |
1 |
- |
n.212C>G |
r.212c>g |
- |
- |
likely pathogenic |
g.35657804G>C |
g.35657807G>C |
C211G |
- |
RMRP_000007 |
1 Finnish, 1 American and 1 Polish CHH family and 1 German CHH patient (all com-het) |
PubMed: Ridanpää et al. 2002, PubMed: Hermanns el al. 2006 |
- |
- |
SUMMARY record |
yes |
- |
- |
- |
- |
Anne Polvi |
| ?/. |
1 |
- |
n.213G>A |
r.(?) |
- |
- |
VUS |
g.35657803C>T |
g.35657806C>T |
- |
- |
CA9_000016 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Nijmegen |
| +?/+? |
1 |
- |
n.214C>G |
r.214c>g |
- |
- |
likely pathogenic |
g.35657802G>C |
g.35657805G>C |
g.213C>G |
- |
RMRP_000084 |
1 German CHH family and 1 German CHH patient (all com-het) |
PubMed: Bonafe et al. 2005, PubMed: Hermanns el al. 2006 |
- |
- |
SUMMARY record |
yes |
0/100 CAU CON |
- |
- |
- |
Anne Polvi |
| +?/+? |
1 |
- |
n.215A>T |
r.215a>u |
- |
- |
likely pathogenic |
g.35657801T>A |
g.35657804T>A |
214T |
- |
RMRP_000085 |
1 German CHH patient (com-het) |
PubMed: Ridanpää et al. 2002 |
- |
- |
SUMMARY record |
yes |
0/280 CON |
- |
- |
- |
Anne Polvi |
| -?/. |
1 |
- |
n.217_218insGCAGTGCGTGTCCGCGCACCAACCACACGGGGCTCATTCTCAGCGCGGCTGTTTTTTGTTCCCGAGACCA |
r.(?) |
- |
- |
likely benign |
g.35657798_35657799insTGGTCTCGGGAACAAAAAACAGCCGCGCTGAGAATGAGCCCCGTGTGGTTGGTGCGCGGACACGCACTGC |
- |
RMRP(NR_003051.3):n.217_218ins70 |
- |
RMRP_000109 |
VKGL data sharing initiative Nederland |
- |
- |
- |
CLASSIFICATION record |
- |
- |
- |
- |
- |
VKGL-NL_Groningen |