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 = exons 3 to 7, 8i_9 = border intron 8/exon 9.
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.
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
Variant/VariO/DNA: VariO of DNA
Variant/VariO/protein: VariO of protein
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.
DNA change (genomic) (hg19): Description of variant at DNA level, based on the genomic DNA reference sequence (following HGVS recommendations).
- g.12345678C>T
- g.12345678_12345890del
- g.12345678_12345890dup
Reference: Reference to publication describing the variant, including links to OMIM (when available), PubMed or or other source, e.g. "den Dunnen ASHG2003 P2346".
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.
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).
Template: Template(s) used to detect the sequence variant; DNA = genomic DNA, RNA = RNA (cDNA).
All options:
- DNA
- RNA = RNA (cDNA)
- Protein
- ? = unknown
Technique: Technique(s) used to identify the sequence variant.
All options:
- ? = Unknown
- arrayCGH = array for Comparative Genomic Hybridisation
- arraySEQ = array for resequencing
- arraySNP = array for SNP typing
- arrayCNV = array for Copy Number Variation (SNP and CNV probes)
- BESS = Base Excision Sequence Scanning
- CMC = Chemical Mismatch Cleavage
- CSCE = Conformation Sensitive Capillary Electrophoresis
- DGGE = Denaturing-Gradient Gel-Electrophoresis
- DHPLC = Denaturing High-Performance Liquid Chromatography
- DOVAM = Detection Of Virtually All Mutations (SSCA variant)
- ddF = dideoxy Fingerprinting
- DSCA = Double-Strand DNA Conformation Analysis
- EMC = Enzymatic Mismatch Cleavage
- HD = HeteroDuplex analysis
- MCA = high-resolution Melting Curve Analysis (hrMCA)
- IHC = Immuno-Histo-Chemistry
- MAPH = Multiplex Amplifiable Probe Hybridisation
- MLPA = Multiplex Ligation-dependent Probe Amplification
- SEQ-NG = Next-Generation Sequencing
- SEQ-NG-H = Next-Generation Sequencing - Helicos
- SEQ-NG-I = Next-Generation Sequencing - Illumina/Solexa
- SEQ-NG-R = Next-Generation Sequencing - Roche/454
- SEQ-NG-S = Next-Generation Sequencing - SOLiD
- Northern = Northern blotting
- PCR = Polymerase Chain Reaction
- PCRdig = PCR + restriction enzyme digestion
- PCRlr = PCR, long-range
- PCRm = PCR, multiplex
- PCRq = PCR, quantitative
- PAGE = Poly-Acrylamide Gel-Electrophoresis
- PTT = Protein Truncation Test
- PFGE = Pulsed-Field Gel-Electrophoresis (+Southern)
- RT-PCR = Reverse Transcription and PCR
- SEQ = SEQuencing
- SBE = Single Base Extension
- SSCA = Single-Strand DNA Conformation polymorphism Analysis (SSCP)
- SSCAf = SSCA, fluorescent (SSCP)
- Southern = Southern blotting
- TaqMan = TaqMan assay
- Western = Western Blotting
Reference: Reference to publication describing the individual/family, possibly giving more phenotypic details than listed in this database entry, including link to PubMed or other source, e.g. "den Dunnen ASHG2003 P2346". References in the "Country:City" format indicate that the variant was submitted directly to this database by the laboratory indicated.
Ethnic origin: The ethnic origin of the individual; e.g. African, Caucasian, gypsy, jew (Ashkenazi).
Remarks: Remarks about the individual.
Effect
|
Exon
|
DNA change (cDNA)
|
RNA change
|
Protein
|
Variant/VariO/DNA
|
Variant/VariO/protein
|
Allele
|
DNA change (genomic) (hg19)
|
Reference
|
DB-ID
|
Frequency
|
Template
|
Technique
|
Disease
|
Reference
|
Ethnic origin
|
Remarks
|
Panel size
|
Owner
|
./. |
1i |
c.17+1G>C |
r.spl? |
p.? |
VariO:0136 DNA substitution; VariO:0316 transversion |
- |
Unknown |
g.6640614C>G |
Kousi et al 2009. Brain 132: 810-819 |
TPP1_000001 |
- |
? |
? |
NCL |
- |
- |
- |
1 |
Y Yang |
./. |
1i |
c.18-3C>G |
r.spl? |
p.? |
VariO:0136 DNA substitution; VariO:0316 transversion |
- |
Unknown |
g.6640501G>C |
Kousi M1, Lehesjoki AE, Mole SE.(2012) |
TPP1_000069 |
- |
? |
? |
NCL |
Kousi M1, Lehesjoki AE, Mole SE.(2012) |
UK |
- |
1 |
Y Yang |
./. |
2i |
c.89+1G>A |
r.(=) |
p.(=) |
- |
- |
Unknown |
g.6640426C>T |
Saini AG et al. (2016) |
TPP1_000093 |
- |
? |
? |
NCL |
Saini AG et al. (2016) |
- |
- |
1 |
XY Liu |
./. |
2i |
c.89+4A>G |
r.spl? |
p.? |
VariO:0136 DNA substitution; VariO:0313 transition; VariO:0315 purine transition |
- |
Unknown |
g.6640423T>C |
Noher de HAc et al.2005. Neuronal ceroid lipofuscinoses (Batten disease) in LatinAmerica –an update, pp: 103-16. |
TPP1_000002 |
- |
? |
? |
NCL |
- |
- |
- |
1 |
Y Yang |
./. |
2 |
c.98_99insC |
r.(?) |
p.(Gly34Argfs*11) |
VariO:0142 DNA insertion |
VariO:0023 amphigoric amino acid indel |
Unknown |
g.6640137_6640138insG |
Kousi M1, Lehesjoki AE, Mole SE.(2012) |
TPP1_000070 |
- |
? |
? |
NCL |
Kousi M1, Lehesjoki AE, Mole SE.(2012) |
UK |
- |
1 |
Y Yang |
./. |
3 |
c.184T>A |
r.(?) |
p.(Ser62Thr) |
VariO:0136 DNA substitution; VariO:0316 transversion |
VariO:0021 amino acid substitution |
Unknown |
g.6640052A>T |
Kousi M1, Lehesjoki AE, Mole SE.(2012) |
TPP1_000071 |
- |
? |
? |
NCL |
Kousi M1, Lehesjoki AE, Mole SE.(2012) |
Turkey |
- |
1 |
Y Yang |
./. |
3 |
c.184_185delTC |
r.(?) |
p.(Ser62Glyfs*25) |
VariO:0141 DNA deletion |
VariO:0023 amphigoric amino acid indel |
Unknown |
g.6640051_6640052delGA |
Lam et al. 2001 Am J Med Genet 99:161-163 |
TPP1_000003 |
- |
? |
? |
NCL |
- |
- |
- |
1 |
Y Yang |
./. |
3 |
c.196C>T |
r.(?) |
p.(Gln66*) |
VariO:0136 DNA substitution; VariO:0313 transition; VariO:0314 pyrimidine transition |
VariO:0015 protein truncation |
Unknown |
g.6640040G>A |
Sleat et al. 1999 Am J Hum Genet 64:1511-1523;Noher de HAc et al. 2005. Neuronal ceroid lipofuscinoses (Batten disease) in Latin America –an update, pp: 103-16.;A. Burkina pers comm |
TPP1_000004 |
- |
? |
? |
NCL |
- |
- |
- |
1 |
Y Yang |
./. |
3 |
c.225A>G |
r.(=) |
p.(=) |
VariO:0136 DNA substitution; VariO:0313 transition; VariO:0315 purine transition |
- |
Unknown |
g.6640011T>C |
Sleat et al. 1999 Am J Hum Genet 64:1511-1523;Taschner pers comm |
TPP1_000005 |
- |
? |
? |
NCL |
- |
- |
- |
1 |
Y Yang |
./. |
3 |
c.229G>A |
r.(?) |
p.(Gly77Arg) |
VariO:0136 DNA substitution; VariO:0313 transition; VariO:0315 purine transition |
VariO:0021 amino acid substitution |
Unknown |
g.6640007C>T |
Sleat et al. 1999 Am J Hum Genet 64:1511-1523 |
TPP1_000006 |
- |
? |
? |
NCL |
- |
- |
- |
1 |
Y Yang |
./. |
4 |
c.237C>A |
r.(?) |
p.(Tyr79*) |
VariO:0136 DNA substitution; VariO:0316 transversion |
VariO:0015 protein truncation |
Unknown |
g.6639000G>T |
Kousi M1, Lehesjoki AE, Mole SE.(2012) |
TPP1_000072 |
- |
? |
? |
NCL |
Kousi M1, Lehesjoki AE, Mole SE.(2012) |
Czech Republic |
- |
1 |
Y Yang |
./. |
4 |
c.237C>G |
r.(?) |
p.(Tyr79*) |
VariO:0136 DNA substitution; VariO:0316 transversion |
VariO:0015 protein truncation |
Unknown |
g.6639000G>C |
Elleder pers comm |
TPP1_000007 |
- |
? |
? |
NCL |
- |
- |
- |
1 |
Y Yang |
./. |
4 |
c.311T>A |
r.(?) |
p.(Leu104*) |
VariO:0136 DNA substitution; VariO:0316 transversion |
VariO:0015 protein truncation |
Unknown |
g.6638926A>T |
Kohan et al. 2008. Human Genetics 123: 537-555 (553);Kohan et al 2009 Clin Genet 76: 372-382;Moore et al 2008 Clin Genet. 74:213-22 |
TPP1_000008 |
- |
? |
? |
NCL |
- |
- |
- |
1 |
Y Yang |
./. |
4 |
c.360dup |
r.(?) |
p.(Thr121Aspfs*17) |
VariO:0142 DNA insertion |
VariO:0023 amphigoric amino acid indel |
Unknown |
g.6638877dup |
Zhong et al. 2000 Genetics in Medicine 2: 312-318 |
TPP1_000010 |
- |
? |
? |
NCL |
- |
- |
- |
1 |
Y Yang |
./. |
4 |
c.379C>T |
r.(?) |
p.(Arg127*) |
VariO:0136 DNA substitution; VariO:0313 transition; VariO:0314 pyrimidine transition |
VariO:0015 protein truncation |
Unknown |
g.6638858G>A |
Sleat et al. 1999 Am J Hum Genet 64:1511-1523;GOS, UK pers comm |
TPP1_000011 |
- |
? |
? |
NCL |
- |
- |
- |
1 |
Y Yang |
./. |
4 |
c.380G>A |
r.(?) |
p.(Arg127Gln) |
VariO:0136 DNA substitution; VariO:0313 transition; VariO:0315 purine transition |
VariO:0021 amino acid substitution |
Unknown |
g.6638857C>T |
Zhong et al. 2000 Genetics in Medicine 2: 312-318;Steinfeld et al. 2002 Am J Med Genet 112:347-353;P. Ray pers comm |
TPP1_000009 |
- |
? |
? |
NCL |
- |
- |
- |
1 |
Y Yang |
./. |
4i |
c.380+55G>A |
r.(=) |
p.(=) |
VariO:0136 DNA substitution; VariO:0313 transition; VariO:0315 purine transition |
- |
Unknown |
g.6638802C>T |
Mole et al. Eur J Paed Neurol 5 (Suppl A):7-102001 |
TPP1_000012 |
- |
? |
? |
NCL |
- |
- |
- |
1 |
Y Yang |
./. |
4i |
c.381-6C>T |
r.(=) |
p.(=) |
VariO:0136 DNA substitution; VariO:0313 transition; VariO:0314 pyrimidine transition |
- |
Unknown |
g.6638665G>A |
Tessa et al pers comm |
TPP1_000013 |
- |
? |
? |
NCL |
- |
- |
- |
1 |
Y Yang |
./. |
4i |
c.381-1G>C |
r.spl? |
p.? |
VariO:0136 DNA substitution; VariO:0316 transversion |
- |
Unknown |
g.6638660C>G |
Kousi M1, Lehesjoki AE, Mole SE.(2012) |
TPP1_000073 |
- |
? |
? |
NCL |
Kousi M1, Lehesjoki AE, Mole SE.(2012) |
Canada |
- |
1 |
Y Yang |
./. |
5 |
c.457T>G |
r.(?) |
p.(Ser153Ala) |
VariO:0136 DNA substitution; VariO:0316 transversion |
VariO:0021 amino acid substitution |
Unknown |
g.6638583A>C |
Caillaud et al. 1999 Hum Genet 65 suppl:A232 |
TPP1_000014 |
- |
? |
? |
NCL |
- |
- |
- |
1 |
Y Yang |
./. |
5 |
c.497_498insA |
r.(?) |
p.(His166Glnfs*22) |
VariO:0142 DNA insertion |
VariO:0023 amphigoric amino acid indel |
Unknown |
g.6638542_6638543insT |
Kousi M1, Lehesjoki AE, Mole SE.(2012) |
TPP1_000074 |
- |
? |
? |
NCL |
Kousi M1, Lehesjoki AE, Mole SE.(2012) |
Turkey |
- |
1 |
Y Yang |
./. |
5i |
c.509-1G>A |
r.spl? |
p.? |
VariO:0136 DNA substitution; VariO:0313 transition; VariO:0315 purine transition |
- |
Unknown |
g.6638385C>T |
Sleat et al. 1999 Am J Hum Genet 64:1511-1523;Hartikainen et al. 1999 Mol Genet Mab 67:162-168 |
TPP1_000016 |
- |
? |
? |
NCL |
- |
- |
- |
1 |
Y Yang |
./. |
5i |
c.509-1G>C |
r.spl? |
p.? |
VariO:0136 DNA substitution; VariO:0316 transversion |
- |
Unknown |
g.6638385C>G |
Sleat et al. 1997 Science 277:1802-1805;Zhong et al. 1998 Clin Genet 54:234-238;Sleat et al. 1999 Am J Hum Genet 64:1511-1523;Hartikainen et al. 1999 Mol Genet Mab 67:162-168;Caillaud et al. 1999 Hum Genet 65 suppl:A232 |
TPP1_000015 |
- |
? |
? |
NCL |
- |
- |
- |
1 |
Y Yang |
./. |
6 |
c.605C>T |
r.(?) |
p.(Pro202Leu) |
VariO:0136 DNA substitution; VariO:0313 transition; VariO:0314 pyrimidine transition |
VariO:0021 amino acid substitution |
Unknown |
g.6638288G>A |
Zhong pes comm;Lobel pers comm;Steinfeld et al. 2002 Am J Med Genet 112:347-354 |
TPP1_000017 |
- |
? |
? |
NCL |
- |
- |
- |
1 |
Y Yang |
./. |
6 |
c.616C>T |
r.(?) |
p.(Arg206Cys) |
VariO:0136 DNA substitution; VariO:0313 transition; VariO:0314 pyrimidine transition |
VariO:0021 amino acid substitution |
Unknown |
g.6638277G>A |
Berry-Kravis et al . 2000 Ann Neurol 47: 254-257 |
TPP1_000018 |
- |
? |
? |
NCL |
- |
- |
- |
1 |
Y Yang |
./. |
6 |
c.617G>A |
r.(?) |
p.(Arg206His) |
VariO:0136 DNA substitution; VariO:0313 transition; VariO:0315 purine transition |
VariO:0021 amino acid substitution |
Unknown |
g.6638276C>T |
A. Burkina pers comm |
TPP1_000019 |
- |
? |
? |
NCL |
- |
- |
- |
1 |
Y Yang |
./. |
6 |
c.622C>T |
r.(?) |
p.(Arg208*) |
VariO:0136 DNA substitution; VariO:0313 transition; VariO:0314 pyrimidine transition |
VariO:0015 protein truncation |
Unknown |
g.6638271G>A |
Sleat et al. 1997 Science 277:1802-1805;Zhong et al. 1998 Clin Genet 54:234-238;Sleat et al. 1999 Am J Hum Genet 64:1511-1523;Hartikainen et al. 1999 Mol Genet Mab 67:162-168 |
TPP1_000020 |
- |
? |
? |
NCL |
- |
- |
- |
1 |
Y Yang |
./. |
6 |
c.625T>C |
r.(?) |
p.(Tyr209His) |
VariO:0136 DNA substitution; VariO:0313 transition; VariO:0314 pyrimidine transition |
VariO:0021 amino acid substitution |
Unknown |
g.6638268A>G |
Kousi M1, Lehesjoki AE, Mole SE.(2012) |
TPP1_000075 |
- |
? |
? |
NCL |
Kousi M1, Lehesjoki AE, Mole SE.(2012) |
UK |
- |
1 |
Y Yang |
./. |
6 |
c.640C>T |
r.(?) |
p.(Gln214*) |
VariO:0136 DNA substitution; VariO:0313 transition; VariO:0314 pyrimidine transition |
VariO:0015 protein truncation |
Unknown |
g.6638253G>A |
Kousi M1, Lehesjoki AE, Mole SE.(2012) |
TPP1_000076 |
- |
? |
? |
NCL |
Kousi M1, Lehesjoki AE, Mole SE.(2012) |
Italy |
- |
1 |
Y Yang |
./. |
6 |
c.646G>A |
r.(?) |
p.(Val216Met) |
VariO:0136 DNA substitution; VariO:0313 transition; VariO:0315 purine transition |
VariO:0021 amino acid substitution |
Unknown |
g.6638247C>T |
Xin pers comm |
TPP1_000021 |
- |
? |
? |
NCL |
- |
- |
- |
1 |
Y Yang |
./. |
6i |
c.688-10C>T |
r.(=) |
p.(=) |
VariO:0136 DNA substitution; VariO:0313 transition; VariO:0314 pyrimidine transition |
- |
Unknown |
g.6638100G>A |
Ju et al. 2002 J Med Genet 39:822-825 |
TPP1_000022 |
- |
? |
? |
NCL |
- |
- |
- |
1 |
Y Yang |
./. |
7 |
c.713C>G |
r.(?) |
p.(Ser238*) |
VariO:0136 DNA substitution; VariO:0316 transversion |
VariO:0015 protein truncation |
Unknown |
g.6638065G>C |
Sleat pers comm |
TPP1_000023 |
- |
? |
? |
NCL |
- |
- |
- |
1 |
Y Yang |
./. |
7 |
c.775delC |
r.(?) |
p.(Arg259Valfs*17) |
VariO:0141 DNA deletion |
VariO:0023 amphigoric amino acid indel |
Unknown |
g.6638003delG |
Ray pers comm;Goldberg-Stern et al. 2009. Pediatr Neurol 41: 297-300 |
TPP1_000024 |
- |
? |
? |
NCL |
- |
- |
- |
1 |
Y Yang |
./. |
7 |
c.790C>T |
r.(?) |
p.(Gln264*) |
VariO:0136 DNA substitution; VariO:0313 transition; VariO:0314 pyrimidine transition |
VariO:0015 protein truncation |
Unknown |
g.6637988G>A |
Kousi M1, Lehesjoki AE, Mole SE.(2012) |
TPP1_000077 |
- |
? |
? |
NCL |
Kousi M1, Lehesjoki AE, Mole SE.(2012) |
Italy |
- |
1 |
Y Yang |
./. |
7 |
c.797G>A |
r.(?) |
p.(Arg266Gln) |
VariO:0136 DNA substitution; VariO:0313 transition; VariO:0315 purine transition |
VariO:0021 amino acid substitution |
Unknown |
g.6637981C>T |
Kousi M1, Lehesjoki AE, Mole SE.(2012) |
TPP1_000078 |
- |
? |
? |
NCL |
Kousi M1, Lehesjoki AE, Mole SE.(2012) |
Spain |
- |
1 |
Y Yang |
./. |
7 |
c.822_837del |
r.(?) |
p.(Leu275*) |
VariO:0141 DNA deletion |
VariO:0015 protein truncation |
Unknown |
g.6637941_6637956del |
Kousi M1, Lehesjoki AE, Mole SE.(2012) |
TPP1_000079 |
- |
? |
? |
NCL |
Kousi M1, Lehesjoki AE, Mole SE.(2012) |
UK |
- |
1 |
Y Yang |
./. |
7 |
c.827A>T |
r.(?) |
p.(Asp276Val) |
VariO:0136 DNA substitution; VariO:0316 transversion |
VariO:0021 amino acid substitution |
Unknown |
g.6637951T>A |
Kohan et al 2009 Clin Genet 76: 372-382 |
TPP1_000025 |
- |
? |
? |
NCL |
- |
- |
- |
1 |
Y Yang |
./. |
7 |
c.829G>A |
r.(?) |
p.(Val277Met) |
VariO:0136 DNA substitution; VariO:0313 transition; VariO:0315 purine transition |
VariO:0021 amino acid substitution |
Unknown |
g.6637949C>T |
Ju et al. 2002 J Med Genet 39:822-825 |
TPP1_000026 |
- |
? |
? |
NCL |
- |
- |
- |
1 |
Y Yang |
./. |
7 |
c.833A>C |
r.(?) |
p.(Gln278Pro) |
VariO:0136 DNA substitution; VariO:0316 transversion |
VariO:0021 amino acid substitution |
Unknown |
g.6637945T>G |
Ju et al. 2002 J Med Genet 39:822-825 |
TPP1_000027 |
- |
? |
? |
NCL |
- |
- |
- |
1 |
Y Yang |
./. |
7 |
c.843G>T |
r.(?) |
p.(Met281Ile) |
VariO:0136 DNA substitution; VariO:0316 transversion |
VariO:0021 amino acid substitution |
Unknown |
g.6637935C>A |
A. Burkina pers comm |
TPP1_000028 |
- |
? |
? |
NCL |
- |
- |
- |
1 |
Y Yang |
./. |
7 |
c.851G>T |
r.(?) |
p.(Gly284Val) |
VariO:0136 DNA substitution; VariO:0316 transversion |
VariO:0021 amino acid substitution |
Unknown |
g.6637927C>A |
Zhong et al. 2000 Genetics in Medicine 2: 312-318;Ju et al. 2002 J Med Genet 39:822-825Moore et al 2008 Clin Genet. 74:213-22 |
TPP1_000031 |
- |
? |
? |
NCL |
- |
- |
- |
1 |
Y Yang |
./. |
7 |
c.857A>G |
r.(?) |
p.(Asn286Ser) |
VariO:0136 DNA substitution; VariO:0313 transition; VariO:0315 purine transition |
VariO:0021 amino acid substitution |
Unknown |
g.6637921T>C |
Steinfeld et al. 2002 Am J Med Genet 112:347-354 |
TPP1_000030 |
- |
? |
? |
NCL |
- |
- |
- |
1 |
Y Yang |
./. |
7 |
c.860T>A |
r.(?) |
p.(Ile287Asn) |
VariO:0136 DNA substitution; VariO:0316 transversion |
VariO:0021 amino acid substitution |
Unknown |
g.6637918A>T |
Sleat et al. 1999 Am J Hum Genet 64:1511-1523 |
TPP1_000029 |
- |
? |
? |
NCL |
- |
- |
- |
1 |
Y Yang |
./. |
7i |
c.886+17T>C |
r.(=) |
p.(=) |
VariO:0136 DNA substitution; VariO:0313 transition; VariO:0314 pyrimidine transition |
- |
Unknown |
g.6637875A>G |
Ju et al. 2002 J Med Genet 39:822-825 |
TPP1_000032 |
- |
? |
? |
NCL |
- |
- |
- |
1 |
Y Yang |
./. |
7i |
c.886+41G>C |
r.(=) |
p.(=) |
VariO:0136 DNA substitution; VariO:0316 transversion |
- |
Unknown |
g.6637851C>G |
Ju et al. 2002 J Med Genet 39:822-825 |
TPP1_000033 |
- |
? |
? |
NCL |
- |
- |
- |
1 |
Y Yang |
./. |
7i |
c.887-18A>G |
r.(=) |
p.(=) |
VariO:0136 DNA substitution; VariO:0313 transition; VariO:0315 purine transition |
- |
Unknown |
g.6637752T>C |
Sleat et al. 1999 Am J Hum Genet 64:1511-1523 |
TPP1_000035 |
- |
? |
? |
NCL |
- |
- |
- |
1 |
Y Yang |
./. |
7i |
c.887-10A>G |
r.(=) |
p.(=) |
VariO:0136 DNA substitution; VariO:0313 transition; VariO:0315 purine transition |
- |
Unknown |
g.6637744T>C |
Bessa et al. 2008. Mol Genet Mab 93:66-73;De HAc pers comm |
TPP1_000034 |
- |
? |
? |
NCL |
- |
- |
- |
1 |
Y Yang |
./. |
8 |
c.969_976del |
r.(?) |
p.(Ser324Argfs*2) |
VariO:0141 DNA deletion |
VariO:0023 amphigoric amino acid indel |
Unknown |
g.6637645_6637652del |
Sleat et al. 1999 Am J Hum Genet 64:1511-1523 |
TPP1_000090 |
- |
? |
? |
NCL |
- |
- |
- |
1 |
Y Yang |
./. |
8 |
c.984_986delTGA |
r.(?) |
p.(Asp328del) |
VariO:0141 DNA deletion |
VariO:0016 sequence retaining amino acid deletion |
Unknown |
g.6637635_6637637delTCA |
A. Burkina pers comm |
TPP1_000037 |
- |
? |
? |
NCL |
- |
- |
- |
1 |
Y Yang |
./. |
8 |
c.987_989delinsCTC |
r.(?) |
p.(Glu329_Asp330delinsAspSer) |
VariO:0143 DNA indel |
VariO:0029 sequence retaining amino acid indel |
Unknown |
g.6637632_6637634delinsGAG |
Mila pers comm |
TPP1_000036 |
- |
? |
? |
NCL |
- |
- |
- |
1 |
Y Yang |
./. |
8 |
c.1015C>T |
r.(?) |
p.(Arg339Trp) |
VariO:0136 DNA substitution; VariO:0313 transition; VariO:0314 pyrimidine transition |
VariO:0021 amino acid substitution |
Unknown |
g.6637606G>A |
C. Caillaud pers comm;A. Burkina pers comm |
TPP1_000038 |
- |
? |
? |
NCL |
- |
- |
- |
1 |
Y Yang |
./. |
8 |
c.1016G>A |
r.(?) |
p.(Arg339Gln) |
VariO:0136 DNA substitution; VariO:0313 transition; VariO:0315 purine transition |
VariO:0021 amino acid substitution |
Unknown |
g.6637605C>T |
Kousi M1, Lehesjoki AE, Mole SE.(2012) |
TPP1_000080 |
- |
? |
? |
NCL |
Kousi M1, Lehesjoki AE, Mole SE.(2012) |
Spain |
- |
1 |
Y Yang |
./. |
8 |
c.1027G>A |
r.(?) |
p.(Glu343Lys) |
VariO:0136 DNA substitution; VariO:0313 transition; VariO:0315 purine transition |
VariO:0021 amino acid substitution |
Unknown |
g.6637594C>T |
Sleat et al. 1999 Am J Hum Genet 64:1511-1523 |
TPP1_000039 |
- |
? |
? |
NCL |
- |
- |
- |
1 |
Y Yang |
./. |
8 |
c.1057A>C |
r.(?) |
p.(Thr353Pro) |
VariO:0136 DNA substitution; VariO:0316 transversion |
VariO:0021 amino acid substitution |
Unknown |
g.6637564T>G |
Steinfeld et al. 2002 Am J Med Geent 112:347-354 |
TPP1_000040 |
- |
? |
? |
NCL |
- |
- |
- |
1 |
Y Yang |
./. |
8 |
c.1062delG |
r.(?) |
p.(Leu355Serfs*72) |
VariO:0141 DNA deletion |
VariO:0023 amphigoric amino acid indel |
Unknown |
g.6637559delC |
Kousi M1, Lehesjoki AE, Mole SE.(2012) |
TPP1_000081 |
- |
? |
? |
NCL |
Kousi M1, Lehesjoki AE, Mole SE.(2012) |
Italy |
- |
1 |
Y Yang |
./. |
8 |
c.1064T>C |
r.(?) |
p.(Leu355Pro) |
VariO:0136 DNA substitution; VariO:0313 transition; VariO:0314 pyrimidine transition |
VariO:0021 amino acid substitution |
Unknown |
g.6637557A>G |
Sleat pers comm |
TPP1_000041 |
- |
? |
? |
NCL |
- |
- |
- |
1 |
Y Yang |
./. |
8i |
c.1075+2T>C |
r.spl? |
p.? |
VariO:0136 DNA substitution; VariO:0313 transition; VariO:0314 pyrimidine transition |
- |
Unknown |
g.6637544A>G |
Kousi M1, Lehesjoki AE, Mole SE.(2012) |
TPP1_000082 |
- |
? |
? |
NCL |
Kousi M1, Lehesjoki AE, Mole SE.(2012) |
The Netherlands |
- |
1 |
Y Yang |
./. |
8i |
c.1075+2T>G |
r.spl? |
p.? |
VariO:0136 DNA substitution; VariO:0316 transversion |
- |
Unknown |
g.6637544A>C |
Sleat et al. 1999 Am J Hum Genet 64:1511-1523 |
TPP1_000042 |
- |
? |
? |
NCL |
- |
- |
- |
1 |
Y Yang |
./. |
8i |
c.1076-18A>G |
r.(=) |
p.(=) |
VariO:0136 DNA substitution; VariO:0313 transition; VariO:0315 purine transition |
- |
Unknown |
g.6637323T>C |
- |
TPP1_000044 |
- |
? |
? |
NCL |
- |
- |
- |
1 |
Y Yang |
./. |
8i |
c.1076-2A>G |
r.spl? |
p.? |
VariO:0136 DNA substitution; VariO:0313 transition; VariO:0315 purine transition |
- |
Unknown |
g.6637307T>C |
Caillaud et al. 1999 Hum Genet 65 suppl:A232 |
TPP1_000043 |
- |
? |
? |
NCL |
- |
- |
- |
1 |
Y Yang |
./. |
9 |
c.1107T>C |
r.(=) |
p.(=) |
VariO:0136 DNA substitution; VariO:0313 transition; VariO:0314 pyrimidine transition |
- |
Unknown |
g.6637274A>G |
Sleat et al. 1997 Science 277:1802-1805;Sleat et al. 1999 Am J Hum Genet 64:1511-1523 |
TPP1_000045 |
- |
? |
? |
NCL |
- |
- |
- |
1 |
Y Yang |
./. |
9 |
c.1108G>A |
r.(?) |
p.(Gly370Arg) |
VariO:0136 DNA substitution; VariO:0313 transition; VariO:0315 purine transition |
VariO:0021 amino acid substitution |
Unknown |
g.6637273C>T |
Sleat et al. 1997 Science 277:1802-1805;Sleat et al. 1999 Am J Hum Genet 64:1511-1523;A. Schultz pers comm |
TPP1_000046 |
- |
? |
? |
NCL |
- |
- |
- |
1 |
Y Yang |
./. |
10 |
c.1144A>C |
r.(?) |
p.(Ser382Arg) |
VariO:0136 DNA substitution; VariO:0316 transversion |
VariO:0021 amino acid substitution |
Unknown |
g.6637237T>G |
Kousi M1, Lehesjoki AE, Mole SE.(2012) |
TPP1_000083 |
- |
? |
? |
NCL |
Kousi M1, Lehesjoki AE, Mole SE.(2012) |
Spain |
- |
1 |
Y Yang |
./. |
10 |
c.1154T>A |
r.(?) |
p.(Val385Asp) |
VariO:0136 DNA substitution; VariO:0316 transversion |
VariO:0021 amino acid substitution |
Unknown |
g.6636785A>T |
Sleat et al. 1999 Am J Hum Genet 64:1511-1523 |
TPP1_000047 |
- |
? |
? |
NCL |
- |
- |
- |
1 |
Y Yang |
./. |
10 |
c.1166G>A |
r.(?) |
p.(Gly389Glu) |
VariO:0136 DNA substitution; VariO:0313 transition; VariO:0315 purine transition |
VariO:0021 amino acid substitution |
Unknown |
g.6636773C>T |
Sleat et al. 1999 Am J Hum Genet 64:1511-1523;Lin and Lobel 2001 Hum Mutat 18:165 |
TPP1_000048 |
- |
? |
? |
NCL |
- |
- |
- |
1 |
Y Yang |
./. |
10 |
c.1204G>T |
r.(?) |
p.(Glu402*) |
VariO:0136 DNA substitution; VariO:0316 transversion |
VariO:0015 protein truncation |
Unknown |
g.6636735C>A |
Kousi et al 2009. Brain 132: 810-819 |
TPP1_000049 |
- |
? |
? |
NCL |
- |
- |
- |
1 |
Y Yang |
./. |
10 |
c.1266G>C |
r.(?) |
p.(Gln422His) |
VariO:0136 DNA substitution; VariO:0316 transversion |
VariO:0021 amino acid substitution |
Unknown |
g.6636673C>G |
Sleat et al. 1999 Am J Hum Genet 64:1511-1523;Steinfeld et al. 2002 Am J Med Genet 112:347-354;Taschner pers comm |
TPP1_000050 |
- |
? |
? |
NCL |
- |
- |
- |
1 |
Y Yang |
./. |
11 |
c.1284G>T |
r.(?) |
p.(Lys428Asn) |
VariO:0136 DNA substitution; VariO:0316 transversion |
VariO:0021 amino acid substitution |
Unknown |
g.6636543C>A |
Zhong et al. 2000 Genetics in Medicine 2: 312-318;Ju et al. 2002 J Med Genet 39:822-825 |
TPP1_000051 |
- |
? |
? |
NCL |
- |
- |
- |
1 |
Y Yang |
./. |
11 |
c.1340G>A |
r.(?) |
p.(Arg447His) |
VariO:0136 DNA substitution; VariO:0313 transition; VariO:0315 purine transition |
VariO:0021 amino acid substitution |
Unknown |
g.6636487C>T |
Zhong pers comm;Sleat et al. 1999 Am J Hum Genet 64:1511-1523;Lin and Lobel 2002 Hum Mutat 18:165 |
TPP1_000052 |
- |
? |
? |
NCL |
- |
- |
- |
1 |
Y Yang |
./. |
11 |
c.1343C>T |
r.(?) |
p.(Ala448Val) |
VariO:0136 DNA substitution; VariO:0313 transition; VariO:0314 pyrimidine transition |
VariO:0021 amino acid substitution |
Unknown |
g.6636484G>A |
Kousi M1, Lehesjoki AE, Mole SE.(2012) |
TPP1_000084 |
- |
? |
? |
NCL |
Kousi M1, Lehesjoki AE, Mole SE.(2012) |
Turkey |
- |
1 |
Y Yang |
./. |
11 |
c.1358C>T |
r.(?) |
p.(Ala453Val) |
VariO:0136 DNA substitution; VariO:0313 transition; VariO:0314 pyrimidine transition |
VariO:0021 amino acid substitution |
Unknown |
g.6636469G>A |
Kohan et al 2009 Clin Genet 76: 372-382 |
TPP1_000053 |
- |
? |
? |
NCL |
- |
- |
- |
1 |
Y Yang |
./. |
11 |
c.1361C>A |
r.(?) |
p.(Ala454Glu) |
VariO:0136 DNA substitution; VariO:0316 transversion |
VariO:0021 amino acid substitution |
Unknown |
g.6636466G>T |
Sleat et al. 1999 Am J Hum Genet 64:1511-1523 |
TPP1_000054 |
- |
? |
? |
NCL |
- |
- |
- |
1 |
Y Yang |
./. |
11 |
c.1380G>A |
r.(?) |
p.(Trp460*) |
VariO:0136 DNA substitution; VariO:0313 transition; VariO:0315 purine transition |
VariO:0015 protein truncation |
Unknown |
g.6636447C>T |
Zhong et al. 2000 Genetics in Medicine 2: 312-318 |
TPP1_000055 |
- |
? |
? |
NCL |
- |
- |
- |
1 |
Y Yang |
./. |
11 |
c.1417G>A |
r.(?) |
p.(Gly473Arg) |
VariO:0136 DNA substitution; VariO:0313 transition; VariO:0315 purine transition |
VariO:0021 amino acid substitution |
Unknown |
g.6636410C>T |
Zhong et al. 2000 Genetics in Medicine 2: 312-318;Lam et al 2001 Am J Med Genet 99:161-163 |
TPP1_000056 |
- |
? |
? |
NCL |
- |
- |
- |
1 |
Y Yang |
./. |
11 |
c.1424C>T |
r.(?) |
p.(Ser475Leu) |
VariO:0136 DNA substitution; VariO:0313 transition; VariO:0314 pyrimidine transition |
VariO:0021 amino acid substitution |
Unknown |
g.6636403G>A |
Sleat et al. 1999 Am J Hum Genet 64:1511-1523;L Johnson pers comm;M. Elleder pers comm |
TPP1_000057 |
- |
? |
? |
NCL |
- |
- |
- |
1 |
Y Yang |
./. |
11 |
c.1424delC |
r.(?) |
p.(Ser475Trpfs*13) |
VariO:0141 DNA deletion |
VariO:0023 amphigoric amino acid indel |
Unknown |
g.6636403delG |
Moore et al 2008 Clin Genet. 74:213-22 |
TPP1_000058 |
- |
? |
? |
NCL |
- |
- |
- |
1 |
Y Yang |
./. |
11i |
c.1425+1G>C |
r.spl? |
p.? |
VariO:0136 DNA substitution; VariO:0316 transversion |
- |
Unknown |
g.6636401C>G |
Kousi M1, Lehesjoki AE, Mole SE.(2012) |
TPP1_000085 |
- |
? |
? |
NCL |
Kousi M1, Lehesjoki AE, Mole SE.(2012) |
Belgium |
- |
1 |
Y Yang |
./. |
12 |
c.1439T>G |
r.(?) |
p.(Val480Gly) |
VariO:0136 DNA substitution; VariO:0316 transversion |
VariO:0021 amino acid substitution |
Unknown |
g.6636209A>C |
Elleder et al. 2008 Acta Neuropathologica 116:119-124;M. Elleder pers comm |
TPP1_000059 |
- |
? |
? |
NCL |
- |
- |
- |
1 |
Y Yang |
./. |
12 |
c.1442T>G |
r.(?) |
p.(Phe481Cys) |
VariO:0136 DNA substitution; VariO:0316 transversion |
VariO:0021 amino acid substitution |
Unknown |
g.6636206A>C |
Ju et al. 2002 J Med Genet 39:822-825 |
TPP1_000060 |
- |
? |
? |
NCL |
- |
- |
- |
1 |
Y Yang |
./. |
12 |
c.1444G>C |
r.(?) |
p.(Gly482Arg) |
VariO:0136 DNA substitution; VariO:0316 transversion |
VariO:0021 amino acid substitution |
Unknown |
g.6636204C>G |
Kousi et al 2009. Brain 132: 810-819 |
TPP1_000061 |
- |
? |
? |
NCL |
- |
- |
- |
1 |
Y Yang |
./. |
12 |
c.1497delT |
r.(?) |
p.(Gly501Alafs*18) |
VariO:0141 DNA deletion |
VariO:0023 amphigoric amino acid indel |
Unknown |
g.6636151delA |
Kousi M1, Lehesjoki AE, Mole SE.(2012) |
TPP1_000086 |
- |
? |
? |
NCL |
Kousi M1, Lehesjoki AE, Mole SE.(2012) |
Turkey |
- |
1 |
Y Yang |
./. |
12 |
c.1501G>T |
r.(?) |
p.(Gly501Cys) |
VariO:0136 DNA substitution; VariO:0316 transversion |
VariO:0021 amino acid substitution |
Unknown |
g.6636147C>A |
Kousi M1, Lehesjoki AE, Mole SE.(2012) |
TPP1_000087 |
- |
? |
? |
NCL |
Kousi M1, Lehesjoki AE, Mole SE.(2012) |
Turkey |
- |
1 |
Y Yang |
./. |
12 |
c.1510A>T |
r.(?) |
p.(Asn504Tyr) |
VariO:0136 DNA substitution; VariO:0316 transversion |
VariO:0021 amino acid substitution |
Unknown |
g.6636138T>A |
Kousi M1, Lehesjoki AE, Mole SE.(2012) |
TPP1_000088 |
- |
? |
? |
NCL |
Kousi M1, Lehesjoki AE, Mole SE.(2012) |
UK |
- |
1 |
Y Yang |
./. |
12 |
c.1547_1548del |
r.(?) |
p.(Phe516*) |
VariO:0141 DNA deletion |
VariO:0015 protein truncation |
Unknown |
g.6636100_6636101del |
Kousi M1, Lehesjoki AE, Mole SE.(2012) |
TPP1_000089 |
- |
? |
? |
NCL |
Kousi M1, Lehesjoki AE, Mole SE.(2012) |
Turkey |
- |
1 |
Y Yang |
./. |
12i |
c.1551+1G>A |
r.spl? |
p.? |
VariO:0136 DNA substitution; VariO:0313 transition; VariO:0315 purine transition |
- |
Unknown |
g.6636096C>T |
Wang et al 2010 Neurogenetics epub 7 Sept |
TPP1_000063 |
- |
? |
? |
NCL |
- |
- |
- |
1 |
Y Yang |
./. |
12i |
c.1551+1G>T |
r.(=) |
p.(=) |
- |
- |
Unknown |
g.6636096C>A |
Yu F et al. (2015) |
TPP1_000091 |
- |
? |
? |
NCL |
Yu F et al. (2015) |
- |
- |
1 |
XY Liu |
./. |
12i |
c.1551+5_1551+6delinsTA |
r.spl? |
p.? |
VariO:0143 DNA indel |
- |
Unknown |
g.6636091_6636092delinsTA |
F. Laranjeira, pers comm |
TPP1_000064 |
- |
? |
? |
NCL |
- |
- |
- |
1 |
Y Yang |
./. |
12i |
c.1551+5_1551+6delinsTA |
r.spl? |
p.? |
VariO:0143 DNA indel |
- |
Unknown |
g.6636091_6636092delinsTA |
Kousi M1, Lehesjoki AE, Mole SE.(2012) |
TPP1_000064 |
- |
? |
? |
NCL |
Kousi M1, Lehesjoki AE, Mole SE.(2012) |
Portugal |
- |
1 |
Y Yang |
./. |
12i |
c.1552-1G>C |
r.spl? |
p.? |
VariO:0136 DNA substitution; VariO:0316 transversion |
- |
Unknown |
g.6635918C>G |
Sleat et al. 1999 Am J Hum Genet 64:1511-1523 |
TPP1_000062 |
- |
? |
? |
NCL |
- |
- |
- |
1 |
Y Yang |
./. |
13 |
c.1611_1621delCTCTGGTCCTG |
r.(?) |
p.(Cys537Trpfs*67) |
VariO:0141 DNA deletion |
VariO:0023 amphigoric amino acid indel |
Unknown |
g.6635848_6635858delCAGGACCAGAG |
Caillaud et al. 1999 Hum Genet 65 suppl:A232 |
TPP1_000065 |
- |
? |
? |
NCL |
- |
- |
- |
1 |
Y Yang |
./. |
13 |
c.1613C>A |
r.(?) |
p.(Ser538Tyr) |
- |
- |
Unknown |
g.6635856G>T |
Yu F et al. (2015) |
TPP1_000092 |
- |
? |
? |
NCL |
Yu F et al. (2015) |
- |
- |
1 |
XY Liu |
./. |
13 |
c.1642T>C |
r.(?) |
p.(Trp548Arg) |
VariO:0136 DNA substitution; VariO:0313 transition; VariO:0314 pyrimidine transition |
VariO:0021 amino acid substitution |
Unknown |
g.6635827A>G |
Elleder pers comm |
TPP1_000067 |
- |
? |
? |
NCL |
- |
- |
- |
1 |
Y Yang |
./. |
13 |
c.1642T>C |
r.(?) |
p.(Trp548Arg) |
VariO:0136 DNA substitution; VariO:0313 transition; VariO:0314 pyrimidine transition |
VariO:0021 amino acid substitution |
Unknown |
g.6635827A>G |
Kousi M1, Lehesjoki AE, Mole SE.(2012) |
TPP1_000067 |
- |
? |
? |
NCL |
Kousi M1, Lehesjoki AE, Mole SE.(2012) |
Slovakia |
- |
1 |
Y Yang |
./. |
13 |
c.1644G>A |
r.(?) |
p.(Trp548*) |
VariO:0136 DNA substitution; VariO:0313 transition; VariO:0315 purine transition |
VariO:0015 protein truncation |
Unknown |
g.6635825C>T |
C. Caillaud pers comm 2000 |
TPP1_000066 |
- |
? |
? |
NCL |
- |
- |
- |
1 |
Y Yang |
./. |
13 |
c.1678_1679delCT |
r.(?) |
p.(Leu560Thrfs*47) |
VariO:0141 DNA deletion |
VariO:0023 amphigoric amino acid indel |
Unknown |
g.6635790_6635791delAG |
Sleat et al. 1999 Am J Hum Genet 64:1511-1523;A. Burkina pers comm |
TPP1_000068 |
- |
? |
? |
NCL |
- |
- |
- |
1 |
Y Yang |