NDDVD - Neurodegenerative Diseases Variation Database
OPA1 (optic atrophy 1 (autosomal dominant))
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Curator:
Y Yang
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All variants in the OPA1 gene
The variants shown are described using the NM_015560.2 transcript reference sequence.
Legend
Please note that a short description of a certain column can be displayed when you move your mouse cursor over the column's header and hold it still. Below, a more detailed description is shown per column.
Effect
: The variant's effect on the function of the gene/protein, displayed in the format 'R/C'. R is the value reported by the source (publication, submitter) and this classification may vary between records. C is the value concluded by the curator. Note that in some database the curator uses Summary records to give details on the classification of the variant.Values used: '+' indicating the variant affects function, '+?' probably affects function, '-' does not affect function, '-?' probably does not affect function, '?' effect unknown, '.' effect was not classified.
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).
How to query this table
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Operator
Column type
Example
Matches
Text
Arg
all entries containing 'Arg'
space
Text
Arg Ser
all entries containing 'Arg' and 'Ser'
|
Text
Arg|Ser
all entries containing 'Arg' or 'Ser'
!
Text
!fs
all entries not containing 'fs'
^
Text
^p.(Arg
all entries beginning with 'p.(Arg'
$
Text
Ser)$
all entries ending with 'Ser)'
=""
Text
=""
all entries with this field empty
=""
Text
="p.0"
all entries exactly matching 'p.0'
!=""
Text
!=""
all entries with this field not empty
!=""
Text
!="p.0"
all entries not exactly matching 'p.0?'
combination
Text
*|Ter !fs
all entries containing '*' or 'Ter' but not containing 'fs'
Date
2020
all entries matching the year 2020
|
Date
2020-03|2020-04
all entries matching March or April, 2020
!
Date
!2020-03
all entries not matching March, 2020
<
Date
<2020
all entries before the year 2020
<=
Date
<=2020-06
all entries in or before June, 2020
>
Date
>2020-06
all entries after June, 2020
>=
Date
>=2020-06-15
all entries on or after June 15th, 2020
combination
Date
2019|2020 <2020-03
all entries in 2019 or 2020, and before March, 2020
Numeric
23
all entries exactly matching 23
|
Numeric
23|24
all entries exactly matching 23 or 24
!
Numeric
!23
all entries not exactly matching 23
<
Numeric
<23
all entries lower than 23
<=
Numeric
<=23
all entries lower than, or equal to, 23
>
Numeric
>23
all entries higher than 23
>=
Numeric
>=23
all entries higher than, or equal to, 23
combination
Numeric
>=20 <30 !23
all entries with values from 20 to 29, but not equal to 23
Some more advanced examples:
Example
Matches
Asian
all entries containing 'Asian', 'asian', including 'Caucasian', 'caucasian', etc.
Asian !Caucasian
all entries containing 'Asian' but not containing 'Caucasian'
Asian|African !Caucasian
all entries containing 'Asian' or 'African', but not containing 'Caucasian'
"South Asian"
all entries containing 'South Asian', but not containing 'South East Asian'
To sort on a certain column, click on the column header or on the arrows. If that column is already selected to sort on, the sort order will be swapped. The column currently sorted on has a darker blue background color than the other columns. The up and down arrows next to the column name indicate the current sorting direction. When sorting on any field other than the default, LOVD will sort secondarily on the default sort column.
291 entries on 3 pages. Showing entries 1 - 100.
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Effect
Exon
DNA change (cDNA)
RNA change
Protein
Variant/VariO/DNA
Variant/VariO/protein
DNA change (genomic) (hg19)
Reference
DB-ID
Frequency
Owner
./.
1
c.5G>A
r.(?)
p.(Trp2*)
VariO:0136 DNA substitution; VariO:0313 transition; VariO:0315 purine transition
VariO:0015 protein truncation
g.193311171G>A
Pesch UE1, Leo-Kottler B, Mayer S, Jurklies B, Kellner U, Apfelstedt-Sylla E, Zrenner E, Alexander C, Wissinger B.(2001)
OPA1_000121
-
Y Yang
./.
1
c.22G>T
r.(?)
p.(Ala8Ser)
VariO:0136 DNA substitution; VariO:0316 transversion
VariO:0021 amino acid substitution
g.193311188G>T
Han J1, Thompson-Lowrey AJ, Reiss A, Mayorov V, Jia H, Biousse V, Newman NJ, Brown MD.(2006)
OPA1_000120
-
Y Yang
./.
2
c.112C>T
r.(?)
p.(Arg38*)
VariO:0136 DNA substitution; VariO:0313 transition; VariO:0314 pyrimidine transition
VariO:0015 protein truncation
g.193332591C>T
Thiselton DL1, Alexander C, Taanman JW, Brooks S, Rosenberg T, Eiberg H, Andreasson S, Van Regemorter N, Munier FL, Moore AT, Bhattacharya SS, Votruba M. (2002)
OPA1_000122
-
Y Yang
./.
2
c.112C>T
r.(?)
p.(Arg38*)
VariO:0136 DNA substitution; VariO:0313 transition; VariO:0314 pyrimidine transition
VariO:0015 protein truncation
g.193332591C>T
Nakamura M1, Lin J, Ueno S, Asaoka R, Hirai T, Hotta Y, Miyake Y, Terasaki H.(2006)
OPA1_000122
-
Y Yang
./.
2
c.112_129del18
r.(?)
p.(Arg38_Ser43del)
VariO:0141 DNA deletion
VariO:0016 sequence retaining amino acid deletion
g.193332591_193332608del18
Thiselton DL1, Alexander C, Taanman JW, Brooks S, Rosenberg T, Eiberg H, Andreasson S, Van Regemorter N, Munier FL, Moore AT, Bhattacharya SS, Votruba M. (2002)
OPA1_000114
-
Y Yang
./.
2
c.113_130del
r.(?)
p.(Arg38_Ser43del)
VariO:0141 DNA deletion
VariO:0016 sequence retaining amino acid deletion
g.193332592_193332609del
Barboni P1, Savini G2, Cascavilla ML3, Caporali L4, Milesi J3, Borrelli E3, La Morgia C5, Valentino ML5, Triolo G3, Lembo A6, Carta A7, De Negri A8, Sadun F9, Rizzo G5, Parisi V2(2014)
OPA1_000226
-
Y Yang
./.
2
c.154C>T
r.(?)
p.(Arg52*)
VariO:0136 DNA substitution; VariO:0313 transition; VariO:0314 pyrimidine transition
VariO:0015 protein truncation
g.193332633C>T
Ban Y, Yoshida Y, Kawasaki S, Mochida C.(2007)
OPA1_000123
-
Y Yang
./.
2
c.154C>T
r.(?)
p.(Arg52*)
-
-
g.193332633C>T
Rocca MA et al. (2015)
OPA1_000123
-
XY Liu
./.
2
c.181C>T
r.(?)
p.(Gln61*)
VariO:0136 DNA substitution; VariO:0313 transition; VariO:0314 pyrimidine transition
VariO:0015 protein truncation
g.193332660C>T
Nakamura M1, Lin J, Ueno S, Asaoka R, Hirai T, Hotta Y, Miyake Y, Terasaki H.(2006)
OPA1_000124
-
Y Yang
./.
2
c.190delT
r.(?)
p.(Ser64Leufs*2)
VariO:0141 DNA deletion
VariO:0023 amphigoric amino acid indel
g.193332669delT
Ferré M1, Bonneau D, Milea D, Chevrollier A, Verny C, Dollfus H, Ayuso C, Defoort S, Vignal C, Zanlonghi X, Charlin JF, Kaplan J, Odent S, Hamel CP, Procaccio V, Reynier P, Amati-Bonneau P.(2009)
OPA1_000067
-
Y Yang
./.
2
c.239A>G
r.(?)
p.(Tyr80Cys)
VariO:0136 DNA substitution; VariO:0313 transition; VariO:0315 purine transition
VariO:0021 amino acid substitution
g.193332718A>G
Yu-Wai-Man P1, Shankar SP, Biousse V, Miller NR, Bean LJ, Coffee B, Hegde M, Newman NJ.(2011)
OPA1_000208
-
Y Yang
./.
2
c.284C>T
r.(?)
p.(Thr95Met)
VariO:0136 DNA substitution; VariO:0313 transition; VariO:0314 pyrimidine transition
VariO:0021 amino acid substitution
g.193332763C>T
Ferré M1, Bonneau D, Milea D, Chevrollier A, Verny C, Dollfus H, Ayuso C, Defoort S, Vignal C, Zanlonghi X, Charlin JF, Kaplan J, Odent S, Hamel CP, Procaccio V, Reynier P, Amati-Bonneau P.(2009)
OPA1_000126
-
Y Yang
./.
2
c.305A>G
r.(?)
p.(Tyr102Cys)
VariO:0136 DNA substitution; VariO:0313 transition; VariO:0315 purine transition
VariO:0021 amino acid substitution
g.193332784A>G
Ferré M1, Bonneau D, Milea D, Chevrollier A, Verny C, Dollfus H, Ayuso C, Defoort S, Vignal C, Zanlonghi X, Charlin JF, Kaplan J, Odent S, Hamel CP, Procaccio V, Reynier P, Amati-Bonneau P.(2009)
OPA1_000127
-
Y Yang
./.
2
c.312A>G
r.(?)
p.(Ile104Met)
-
-
g.193332791A>G
Kazamel M et al. (2014)
OPA1_000242
-
XY Liu
./.
2
c.344C>T
r.(?)
p.(Ala115Val)
VariO:0136 DNA substitution; VariO:0313 transition; VariO:0314 pyrimidine transition
VariO:0021 amino acid substitution
g.193332823C>T
Yu-Wai-Man P1, Griffiths PG, Gorman GS, Lourenco CM, Wright AF, Auer-Grumbach M, Toscano A, Musumeci O, Valentino ML, Caporali L, Lamperti C,(2010)
OPA1_000005
-
Y Yang
./.
3
c.361C>T
r.(?)
p.(Gln121*)
VariO:0136 DNA substitution; VariO:0313 transition; VariO:0314 pyrimidine transition
VariO:0015 protein truncation
g.193333472C>T
Ferré M1, Bonneau D, Milea D, Chevrollier A, Verny C, Dollfus H, Ayuso C, Defoort S, Vignal C, Zanlonghi X, Charlin JF, Kaplan J, Odent S, Hamel CP, Procaccio V, Reynier P, Amati-Bonneau P.(2009)
OPA1_000128
-
Y Yang
./.
2
c.439A>G
r.(?)
p.(Ile147Val)
VariO:0136 DNA substitution; VariO:0313 transition; VariO:0315 purine transition
VariO:0021 amino acid substitution
g.193333550A>G
Han J1, Thompson-Lowrey AJ, Reiss A, Mayorov V, Jia H, Biousse V, Newman NJ, Brown MD.(2006)
OPA1_000125
-
Y Yang
./.
3i
c.448+1G>C
r.spl?
p.?
VariO:0136 DNA substitution; VariO:0316 transversion
-
g.193333560G>C
Thiselton DL1, Alexander C, Taanman JW, Brooks S, Rosenberg T, Eiberg H, Andreasson S, Van Regemorter N, Munier FL, Moore AT, Bhattacharya SS, Votruba M.(2002)
OPA1_000030
-
Y Yang
./.
3i
c.448+2T>G
r.spl?
p.?
VariO:0136 DNA substitution; VariO:0316 transversion
-
g.193333561T>G
Ferré M1, Bonneau D, Milea D, Chevrollier A, Verny C, Dollfus H, Ayuso C, Defoort S, Vignal C, Zanlonghi X, Charlin JF, Kaplan J, Odent S, Hamel CP, Procaccio V, Reynier P, Amati-Bonneau P.(2009)
OPA1_000029
-
Y Yang
./.
6
c.629C>A
r.(?)
p.(Ser210*)
VariO:0136 DNA substitution; VariO:0316 transversion
VariO:0015 protein truncation
g.193349405C>A
Pesch UE1, Leo-Kottler B, Mayer S, Jurklies B, Kellner U, Apfelstedt-Sylla E, Zrenner E, Alexander C, Wissinger B.(2001)
OPA1_000129
-
Y Yang
./.
6
c.631_634delGACA
r.(?)
p.(Asp211Lysfs*16)
VariO:0141 DNA deletion
VariO:0023 amphigoric amino acid indel
g.193349407_193349410delGACA
Cohn AC1, Toomes C, Potter C, Towns KV, Hewitt AW, Inglehearn CF, Craig JE, Mackey DA.(2007)
OPA1_000028
-
Y Yang
./.
6
c.635del
r.(?)
p.(Glu213Argfs*15)
VariO:0141 DNA deletion
VariO:0023 amphigoric amino acid indel
g.193349411del
Yu-Wai-Man P1, Griffiths PG, Gorman GS, Lourenco CM, Wright AF, Auer-Grumbach M, Toscano A, Musumeci O, Valentino ML, Caporali L, Lamperti C,(2010)
OPA1_000202
-
Y Yang
./.
6
c.635_636del2
r.(?)
p.(Lys212Argfs*4)
VariO:0141 DNA deletion
VariO:0023 amphigoric amino acid indel
g.193349411_193349412del2
Toomes C1, Marchbank NJ, Mackey DA, Craig JE, Newbury-Ecob RA, Bennett CP, Vize CJ, Desai SP, Black GC, Patel N, Teimory M, Markham AF, Inglehearn CF, Churchill AJ.(2001)
OPA1_000027
-
Y Yang
./.
6
c.635_638del4
r.(?)
p.(Lys212Argfs*15)
VariO:0141 DNA deletion
VariO:0023 amphigoric amino acid indel
g.193349411_193349414del4
Baris O1, Delettre C, Amati-Bonneau P, Surget MO, Charlin JF, Catier A, Derieux L, Guyomard JL, Dollfus H, Jonveaux P, Ayuso C, Maumenee I, Lorenz B, Mohammed S, Tourmen Y, Bonneau D, Malthièry Y, Hamel C, Reynier P.(2003)
OPA1_000026
-
Y Yang
./.
6
c.638_639del2
r.(?)
p.(Lys214Asnfs*2)
VariO:0141 DNA deletion
VariO:0023 amphigoric amino acid indel
g.193349414_193349415del2
Yu-Wai-Man P1, Griffiths PG, Burke A, Sellar PW, Clarke MP, Gnanaraj L, Ah-Kine D, Hudson G, Czermin B, Taylor RW, Horvath R, Chinnery PF.(2010)
OPA1_000004
-
Y Yang
./.
6
c.665T>C
r.(?)
p.(Leu222Pro)
VariO:0136 DNA substitution; VariO:0313 transition; VariO:0314 pyrimidine transition
VariO:0021 amino acid substitution
g.193349441T>C
Ferré M1, Bonneau D, Milea D, Chevrollier A, Verny C, Dollfus H, Ayuso C, Defoort S, Vignal C, Zanlonghi X, Charlin JF, Kaplan J, Odent S, Hamel CP, Procaccio V, Reynier P, Amati-Bonneau P.(2009)
OPA1_000130
-
Y Yang
./.
7
c.703C>T
r.(?)
p.(Arg235*)
VariO:0136 DNA substitution; VariO:0313 transition; VariO:0314 pyrimidine transition
VariO:0015 protein truncation
g.193353231C>T
Puomila A1, Huoponen K, M?ntyj?rvi M, H?m?l?inen P, Paananen R, Sankila EM, Savontaus ML, Somer M, Nikoskelainen E.(2005)
OPA1_000132
-
Y Yang
./.
7
c.703C>T
r.(?)
p.(Arg235*)
VariO:0136 DNA substitution; VariO:0313 transition; VariO:0314 pyrimidine transition
VariO:0015 protein truncation
g.193353231C>T
Barboni P1, Savini G2, Cascavilla ML3, Caporali L4, Milesi J3, Borrelli E3, La Morgia C5, Valentino ML5, Triolo G3, Lembo A6, Carta A7, De Negri A8, Sadun F9, Rizzo G5, Parisi V2(2014)
OPA1_000132
-
Y Yang
./.
7
c.724G>T
r.(?)
p.(Glu242*)
VariO:0136 DNA substitution; VariO:0316 transversion
VariO:0015 protein truncation
g.193353252G>T
Puomila A1, Huoponen K, M?ntyj?rvi M, H?m?l?inen P, Paananen R, Sankila EM, Savontaus ML, Somer M, Nikoskelainen E.(2005)
OPA1_000133
-
Y Yang
./.
7
c.728T>A
r.(?)
p.(Leu243*)
VariO:0136 DNA substitution; VariO:0316 transversion
VariO:0015 protein truncation
g.193353256T>A
Ferré M1, Bonneau D, Milea D, Chevrollier A, Verny C, Dollfus H, Ayuso C, Defoort S, Vignal C, Zanlonghi X, Charlin JF, Kaplan J, Odent S, Hamel CP, Procaccio V, Reynier P, Amati-Bonneau P.(2009)
OPA1_000131
-
Y Yang
./.
7
c.733A>T
r.(?)
p.(Lys245*)
VariO:0136 DNA substitution; VariO:0316 transversion
VariO:0015 protein truncation
g.193353261A>T
Thiselton DL1, Alexander C, Taanman JW, Brooks S, Rosenberg T, Eiberg H, Andreasson S, Van Regemorter N, Munier FL, Moore AT, Bhattacharya SS, Votruba M. (2002)
OPA1_000134
-
Y Yang
./.
7i
c.784-1G>A
r.spl?
p.?
VariO:0136 DNA substitution; VariO:0313 transition; VariO:0315 purine transition
-
g.193354983G>A
Ferré M1, Bonneau D, Milea D, Chevrollier A, Verny C, Dollfus H, Ayuso C, Defoort S, Vignal C, Zanlonghi X, Charlin JF, Kaplan J, Odent S, Hamel CP, Procaccio V, Reynier P, Amati-Bonneau P.(2009)
OPA1_000025
-
Y Yang
./.
7i
c.784-1G>T
r.spl?
p.?
VariO:0136 DNA substitution; VariO:0316 transversion
-
g.193354983G>T
Kamakari S1, Koutsodontis G1, Tsilimbaris M2, Fitsios A3, Chrousos G3.(2014)
OPA1_000220
-
Y Yang
./.
8
c.794_797del4
r.(?)
p.(Ile265Thrfs*42)
VariO:0141 DNA deletion
VariO:0023 amphigoric amino acid indel
g.193354994_193354997del4
Toomes C1, Marchbank NJ, Mackey DA, Craig JE, Newbury-Ecob RA, Bennett CP, Vize CJ, Desai SP, Black GC, Patel N, Teimory M, Markham AF, Inglehearn CF, Churchill AJ.(2001)
OPA1_000024
-
Y Yang
./.
8
c.803A>G
r.(?)
p.(Tyr268Cys)
VariO:0136 DNA substitution; VariO:0313 transition; VariO:0315 purine transition
VariO:0021 amino acid substitution
g.193355003A>G
-
OPA1_000003
-
Y Yang
./.
8
c.806C>T
r.(?)
p.(Ser269Phe)
VariO:0136 DNA substitution; VariO:0313 transition; VariO:0314 pyrimidine transition
VariO:0021 amino acid substitution
g.193355006C>T
-
OPA1_000002
-
Y Yang
./.
8
c.808G>A
r.(?)
p.(Glu270Lys)
VariO:0136 DNA substitution; VariO:0313 transition; VariO:0315 purine transition
VariO:0021 amino acid substitution
g.193355008G>A
Pesch UE1, Leo-Kottler B, Mayer S, Jurklies B, Kellner U, Apfelstedt-Sylla E, Zrenner E, Alexander C, Wissinger B.(2001)
OPA1_000135
-
Y Yang
./.
8
c.815T>C
r.(?)
p.(Leu272Pro)
VariO:0136 DNA substitution; VariO:0313 transition; VariO:0314 pyrimidine transition
VariO:0021 amino acid substitution
g.193355015T>C
Baris O1, Delettre C, Amati-Bonneau P, Surget MO, Charlin JF, Catier A, Derieux L, Guyomard JL, Dollfus H, Jonveaux P, Ayuso C, Maumenee I, Lorenz B, Mohammed S, Tourmen Y, Bonneau D, Malthièry Y, Hamel C, Reynier P.(2003)
OPA1_000136
-
Y Yang
./.
8
c.818A>C
r.(?)
p.(Asp273Ala)
VariO:0136 DNA substitution; VariO:0316 transversion
VariO:0021 amino acid substitution
g.193355018A>C
Pesch UE1, Leo-Kottler B, Mayer S, Jurklies B, Kellner U, Apfelstedt-Sylla E, Zrenner E, Alexander C, Wissinger B.(2001)
OPA1_000137
-
Y Yang
./.
8
c.868C>T
r.(?)
p.(Arg290Trp)
VariO:0136 DNA substitution; VariO:0313 transition; VariO:0314 pyrimidine transition
VariO:0021 amino acid substitution
g.193355068C>T
Pesch UE1, Leo-Kottler B, Mayer S, Jurklies B, Kellner U, Apfelstedt-Sylla E, Zrenner E, Alexander C, Wissinger B.(2001)
OPA1_000138
-
Y Yang
./.
8
c.869G>A
r.(?)
p.(Arg290Gln)
VariO:0136 DNA substitution; VariO:0313 transition; VariO:0315 purine transition
VariO:0021 amino acid substitution
g.193355069G>A
Alexander C1, Votruba M, Pesch UE, Thiselton DL, Mayer S, Moore A, Rodriguez M, Kellner U, Leo-Kottler B, Auburger G, Bhattacharya SS, Wissinger B.(2000)
OPA1_000139
-
Y Yang
./.
8
c.869G>A
r.(?)
p.(Arg290Gln)
VariO:0136 DNA substitution; VariO:0313 transition; VariO:0315 purine transition
VariO:0021 amino acid substitution
g.193355069G>A
Gallus GN1, Cardaioli E, Rufa A, Collura M, Da Pozzo P, Pretegiani E, Tumino M, Pavone L, Federico A.(2012)
OPA1_000139
-
Y Yang
./.
8
c.869G>A
r.(?)
p.(Arg290Gln)
VariO:0136 DNA substitution; VariO:0313 transition; VariO:0315 purine transition
VariO:0021 amino acid substitution
g.193355069G>A
Russo A1, Delcassi L, Marchina E, Semeraro F.(2013)
OPA1_000139
-
Y Yang
./.
8
c.869G>A
r.(?)
p.(Arg290Gln)
VariO:0136 DNA substitution; VariO:0313 transition; VariO:0315 purine transition
VariO:0021 amino acid substitution
g.193355069G>A
Barboni P1, Savini G2, Cascavilla ML3, Caporali L4, Milesi J3, Borrelli E3, La Morgia C5, Valentino ML5, Triolo G3, Lembo A6, Carta A7, De Negri A8, Sadun F9, Rizzo G5, Parisi V2(2014)
OPA1_000139
-
Y Yang
./.
8
c.869G>A
r.(?)
p.(Arg290Gln)
-
-
g.193355069G>A
Loo JL et al. (2017)
OPA1_000139
-
XY Liu
./.
8i
c.870+1G>T
r.spl?
p.?
VariO:0136 DNA substitution; VariO:0316 transversion
-
g.193355071G>T
Ferré M1, Bonneau D, Milea D, Chevrollier A, Verny C, Dollfus H, Ayuso C, Defoort S, Vignal C, Zanlonghi X, Charlin JF, Kaplan J, Odent S, Hamel CP, Procaccio V, Reynier P, Amati-Bonneau P.(2009)
OPA1_000023
-
Y Yang
./.
8i
c.870+5G>A
r.spl?
p.?
VariO:0136 DNA substitution; VariO:0313 transition; VariO:0315 purine transition
-
g.193355075G>A
Toomes C1, Marchbank NJ, Mackey DA, Craig JE, Newbury-Ecob RA, Bennett CP, Vize CJ, Desai SP, Black GC, Patel N, Teimory M, Markham AF, Inglehearn CF, Churchill AJ.(2001)
OPA1_000022
-
Y Yang
./.
8i
c.870+5G>A
r.spl?
p.?
VariO:0136 DNA substitution; VariO:0313 transition; VariO:0315 purine transition
-
g.193355075G>A
Barboni P1, Savini G2, Cascavilla ML3, Caporali L4, Milesi J3, Borrelli E3, La Morgia C5, Valentino ML5, Triolo G3, Lembo A6, Carta A7, De Negri A8, Sadun F9, Rizzo G5, Parisi V2(2014)
OPA1_000022
-
Y Yang
./.
8i
c.870+5G>A
r.spl?
p.?
-
-
g.193355075G>A
Rocca MA et al. (2015)
OPA1_000022
-
XY Liu
./.
8i
c.871-1G>A
r.spl?
p.?
VariO:0136 DNA substitution; VariO:0313 transition; VariO:0315 purine transition
-
g.193355740G>A
Ferré M1, Bonneau D, Milea D, Chevrollier A, Verny C, Dollfus H, Ayuso C, Defoort S, Vignal C, Zanlonghi X, Charlin JF, Kaplan J, Odent S, Hamel CP, Procaccio V, Reynier P, Amati-Bonneau P.(2009)
OPA1_000021
-
Y Yang
./.
8i
c.871-1G>A
r.spl?
p.?
-
-
g.193355740G>A
Loo JL et al. (2017)
OPA1_000021
-
XY Liu
./.
9
c.876_878delTGT
r.(?)
p.(Val294del)
VariO:0141 DNA deletion
VariO:0016 sequence retaining amino acid deletion
g.193355746_193355748delTGT
Yu-Wai-Man P1, Griffiths PG, Burke A, Sellar PW, Clarke MP, Gnanaraj L, Ah-Kine D, Hudson G, Czermin B, Taylor RW, Horvath R, Chinnery PF.(2010)
OPA1_000001
-
Y Yang
./.
9
c.876_878delTGT
r.(?)
p.(Val294del)
VariO:0141 DNA deletion
VariO:0016 sequence retaining amino acid deletion
g.193355746_193355748delTGT
Gallus GN1, Cardaioli E, Rufa A, Collura M, Da Pozzo P, Pretegiani E, Tumino M, Pavone L, Federico A.(2012)
OPA1_000209
ADMIN
Y Yang
./.
9
c.876_878delTGT
r.(?)
p.(Val294del)
VariO:0141 DNA deletion
VariO:0016 sequence retaining amino acid deletion
g.193355746_193355748delTGT
Kamakari S1, Koutsodontis G1, Tsilimbaris M2, Fitsios A3, Chrousos G3.(2014)
OPA1_000001
-
Y Yang
./.
9
c.877_882delGTGGTT
r.(?)
p.(Val293_Val294del)
VariO:0141 DNA deletion
VariO:0016 sequence retaining amino acid deletion
g.193355747_193355752delGTGGTT
Ferré M1, Bonneau D, Milea D, Chevrollier A, Verny C, Dollfus H, Ayuso C, Defoort S, Vignal C, Zanlonghi X, Charlin JF, Kaplan J, Odent S, Hamel CP, Procaccio V, Reynier P, Amati-Bonneau P.(2009)
OPA1_000020
-
Y Yang
./.
9
c.889C>T
r.(?)
p.(Gln297*)
VariO:0136 DNA substitution; VariO:0313 transition; VariO:0314 pyrimidine transition
VariO:0015 protein truncation
g.193355759C>T
Barboni P1, Savini G2, Cascavilla ML3, Caporali L4, Milesi J3, Borrelli E3, La Morgia C5, Valentino ML5, Triolo G3, Lembo A6, Carta A7, De Negri A8, Sadun F9, Rizzo G5, Parisi V2(2014)
OPA1_000234
-
Y Yang
./.
9
c.892A>G
r.(?)
p.(Ser298Gly)
-
-
g.193355762A>G
Loo JL et al. (2017)
OPA1_000250
-
XY Liu
./.
9
c.899G>A
r.(?)
p.(Gly300Glu)
VariO:0136 DNA substitution; VariO:0313 transition; VariO:0315 purine transition
VariO:0021 amino acid substitution
g.193355769G>A
Delettre C1, Lenaers G, Griffoin JM, Gigarel N, Lorenzo C, Belenguer P, Pelloquin L, Grosgeorge J, Turc-Carel C, Perret E, Astarie-Dequeker C, Lasquellec L, Arnaud B, Ducommun B, Kaplan J, Hamel CP.(2000)
OPA1_000140
-
Y Yang
./.
9
c.929A>G
r.(?)
p.(Gln310Arg)
VariO:0136 DNA substitution; VariO:0313 transition; VariO:0315 purine transition
VariO:0021 amino acid substitution
g.193355799A>G
Ferré M1, Bonneau D, Milea D, Chevrollier A, Verny C, Dollfus H, Ayuso C, Defoort S, Vignal C, Zanlonghi X, Charlin JF, Kaplan J, Odent S, Hamel CP, Procaccio V, Reynier P, Amati-Bonneau P.(2009)
OPA1_000141
-
Y Yang
./.
9
c.932delC
r.(?)
p.(Ala311Valfs*11)
VariO:0141 DNA deletion
VariO:0023 amphigoric amino acid indel
g.193355802delC
Pesch UE1, Leo-Kottler B, Mayer S, Jurklies B, Kellner U, Apfelstedt-Sylla E, Zrenner E, Alexander C, Wissinger B.(2001)
OPA1_000019
-
Y Yang
./.
9
c.934C>T
r.(?)
p.(Arg312*)
VariO:0136 DNA substitution; VariO:0313 transition; VariO:0314 pyrimidine transition
VariO:0015 protein truncation
g.193355804C>T
Cardaioli E, Gallus GN, Da Pozzo P, Rufa A, Franceschini R, Motolese E, Caporossi A, Dotti MT, Federico A.(2006)
OPA1_000142
-
Y Yang
./.
9
c.934C>T
r.(?)
p.(Arg312*)
-
-
g.193355804C>T
Formichi P et al. (2015)
OPA1_000142
-
XY Liu
./.
9
c.937A>G
r.(?)
p.(Ile313Val)
VariO:0136 DNA substitution; VariO:0313 transition; VariO:0315 purine transition
VariO:0021 amino acid substitution
g.193355807A>G
Cohn AC1, Toomes C, Potter C, Towns KV, Hewitt AW, Inglehearn CF, Craig JE, Mackey DA.(2007)
OPA1_000204
-
Y Yang
./.
9
c.946A>T
r.(?)
p.(Arg316*)
VariO:0136 DNA substitution; VariO:0316 transversion
VariO:0015 protein truncation
g.193355816A>T
Nakamura M1, Lin J, Ueno S, Asaoka R, Hirai T, Hotta Y, Miyake Y, Terasaki H.(2006)
OPA1_000143
-
Y Yang
./.
9
c.970_978delCGTTCTCCA
r.(?)
p.(Arg324_Pro326del)
VariO:0141 DNA deletion
VariO:0016 sequence retaining amino acid deletion
g.193355840_193355848delCGTTCTCCA
Puomila A1, Huoponen K, M?ntyj?rvi M, H?m?l?inen P, Paananen R, Sankila EM, Savontaus ML, Somer M, Nikoskelainen E.(2005)
OPA1_000018
-
Y Yang
./.
9
c.983A>G
r.(?)
p.(Lys328Arg)
VariO:0136 DNA substitution; VariO:0313 transition; VariO:0315 purine transition
VariO:0021 amino acid substitution
g.193355853A>G
Baris O1, Delettre C, Amati-Bonneau P, Surget MO, Charlin JF, Catier A, Derieux L, Guyomard JL, Dollfus H, Jonveaux P, Ayuso C, Maumenee I, Lorenz B, Mohammed S, Tourmen Y, Bonneau D, Malthièry Y, Hamel C, Reynier P.(2003)
OPA1_000206
-
Y Yang
./.
9
c.983A>G
r.(?)
p.(Lys328Arg)
VariO:0136 DNA substitution; VariO:0313 transition; VariO:0315 purine transition
VariO:0021 amino acid substitution
g.193355853A>G
Almind GJ1, Ek J, Rosenberg T, Eiberg H, Larsen M, Lucamp L, Br?ndum-Nielsen K, Gr?nskov K.(2012)
OPA1_000206
-
Y Yang
./.
9
c.983A>G
r.(?)
p.(Lys328Arg)
-
-
g.193355853A>G
Nissen C et al. (2015)
OPA1_000206
-
XY Liu
./.
9
c.983A>G
r.(?)
p.(Lys328Arg)
-
-
g.193355853A>G
Rönnbäck C et al. (2015)
OPA1_000206
-
XY Liu
./.
9
c.984G>A
r.(=)
p.(=)
VariO:0136 DNA substitution; VariO:0313 transition; VariO:0315 purine transition
-
g.193355854G>A
Delettre C1, Griffoin JM, Kaplan J, Dollfus H, Lorenz B, Faivre L, Lenaers G, Belenguer P, Hamel CP.(2001)
OPA1_000205
-
Y Yang
./.
9i
c.984+1G>A
r.spl?
p.?
VariO:0136 DNA substitution; VariO:0313 transition; VariO:0315 purine transition
-
g.193355855G>A
Fül?p L1, Rajki A2, Maka E3, Molnár MJ4, Sp?t A5.(2015)
OPA1_000241
-
XY Liu
./.
9i
c.984+1_984+4del4
r.spl?
p.?
VariO:0141 DNA deletion
-
g.193355855_193355858del4
Thiselton DL1, Alexander C, Taanman JW, Brooks S, Rosenberg T, Eiberg H, Andreasson S, Van Regemorter N, Munier FL, Moore AT, Bhattacharya SS, Votruba M.(2002)
OPA1_000017
-
Y Yang
./.
9i
c.984+3A>T
r.spl?
p.?
VariO:0136 DNA substitution; VariO:0316 transversion
-
g.193355857A>T
Thiselton DL1, Alexander C, Taanman JW, Brooks S, Rosenberg T, Eiberg H, Andreasson S, Van Regemorter N, Munier FL, Moore AT, Bhattacharya SS, Votruba M.(2002)
OPA1_000016
-
Y Yang
./.
9i
c.984+3delA
r.spl?
p.?
VariO:0141 DNA deletion
-
g.193355857delA
Ferré M1, Bonneau D, Milea D, Chevrollier A, Verny C, Dollfus H, Ayuso C, Defoort S, Vignal C, Zanlonghi X, Charlin JF, Kaplan J, Odent S, Hamel CP, Procaccio V, Reynier P, Amati-Bonneau P.(2009)
OPA1_000201
-
Y Yang
./.
9i
c.985-1G>A
r.spl?
p.?
VariO:0136 DNA substitution; VariO:0313 transition; VariO:0315 purine transition
-
g.193360553G>A
Delettre C1, Lenaers G, Griffoin JM, Gigarel N, Lorenzo C, Belenguer P, Pelloquin L, Grosgeorge J, Turc-Carel C, Perret E, Astarie-Dequeker C, Lasquellec L, Arnaud B, Ducommun B, Kaplan J, Hamel CP.(2000)
OPA1_000015
-
Y Yang
./.
10
c.985G>T
r.(?)
p.Val329_Asp355del
-
-
g.193360554G>T
Iida K et al. (2016)
OPA1_000249
-
XY Liu
./.
10
c.988A>T
r.(?)
p.(Thr330Ser)
VariO:0136 DNA substitution; VariO:0316 transversion
VariO:0021 amino acid substitution
g.193360557A>T
Chen Y1, Jia X, Wang P, Xiao X, Li S, Guo X, Zhang Q.(2013)
OPA1_000217
-
Y Yang
./.
10
c.1016delC
r.(?)
p.(Leu340Tyrfs*20)
VariO:0141 DNA deletion
VariO:0023 amphigoric amino acid indel
g.193360585delC
Alexander C1, Votruba M, Pesch UE, Thiselton DL, Mayer S, Moore A, Rodriguez M, Kellner U, Leo-Kottler B, Auburger G, Bhattacharya SS, Wissinger B.(2000)
OPA1_000119
-
Y Yang
./.
10
c.1039G>T
r.(?)
p.(Glu347*)
VariO:0136 DNA substitution; VariO:0316 transversion
VariO:0015 protein truncation
g.193360608G>T
Yamada T1, Hayasaka S, Matsumoto M, Budu, Esa T, Hayasaka Y, Endo M, Nagaki Y, Fujiki K, Murakami A, Kanai A.(2003)
OPA1_000144
-
Y Yang
./.
10i
c.1065+2T>C
r.spl?
p.?
VariO:0136 DNA substitution; VariO:0313 transition; VariO:0314 pyrimidine transition
-
g.193360636T>C
Puomila A1, Huoponen K, M?ntyj?rvi M, H?m?l?inen P, Paananen R, Sankila EM, Savontaus ML, Somer M, Nikoskelainen E.(2005)
OPA1_000118
-
Y Yang
./.
10i
c.1065+2T>G
r.spl?
p.?
VariO:0136 DNA substitution; VariO:0316 transversion
-
g.193360636T>G
Kim JY1, Hwang JM, Ko HS, Seong MW, Park BJ, Park SS.(2005)
OPA1_000117
-
Y Yang
./.
10i
c.1065+3A>C
r.spl?
p.?
VariO:0136 DNA substitution; VariO:0316 transversion
-
g.193360637A>C
Pesch UE1, Leo-Kottler B, Mayer S, Jurklies B, Kellner U, Apfelstedt-Sylla E, Zrenner E, Alexander C, Wissinger B.(2001)
OPA1_000116
-
Y Yang
./.
11
c.1069G>A
r.(?)
p.(Ala357Thr)
VariO:0136 DNA substitution; VariO:0313 transition; VariO:0315 purine transition
VariO:0021 amino acid substitution
g.193360767G>A
Ferré M1, Bonneau D, Milea D, Chevrollier A, Verny C, Dollfus H, Ayuso C, Defoort S, Vignal C, Zanlonghi X, Charlin JF, Kaplan J, Odent S, Hamel CP, Procaccio V, Reynier P, Amati-Bonneau P.(2009)
OPA1_000145
-
Y Yang
./.
11
c.1072_1093del22
r.(?)
p.(Ala358Phefs*3)
VariO:0141 DNA deletion
VariO:0023 amphigoric amino acid indel
g.193360770_193360791del22
Pesch UE1, Leo-Kottler B, Mayer S, Jurklies B, Kellner U, Apfelstedt-Sylla E, Zrenner E, Alexander C, Wissinger B.(2001)
OPA1_000115
-
Y Yang
./.
11
c.1074A>G
r.(=)
p.(=)
VariO:0136 DNA substitution; VariO:0313 transition; VariO:0315 purine transition
-
g.193360772A>G
Yu-Wai-Man P1, Griffiths PG, Gorman GS, Lourenco CM, Wright AF, Auer-Grumbach M, Toscano A, Musumeci O, Valentino ML, Caporali L, Lamperti C,(2010)
OPA1_000014
-
Y Yang
./.
11
c.1084G>T
r.(?)
p.(Glu362*)
VariO:0136 DNA substitution; VariO:0316 transversion
VariO:0015 protein truncation
g.193360782G>T
Hamahata T1, Fujimaki T, Fujiki K, Miyazaki A, Mizota A, Murakami A.(2012)
OPA1_000211
-
Y Yang
./.
11
c.1096C>T
r.(?)
p.(Arg366*)
VariO:0136 DNA substitution; VariO:0313 transition; VariO:0314 pyrimidine transition
VariO:0015 protein truncation
g.193360794C>T
Alexander C1, Votruba M, Pesch UE, Thiselton DL, Mayer S, Moore A, Rodriguez M, Kellner U, Leo-Kottler B, Auburger G, Bhattacharya SS, Wissinger B.(2000)
OPA1_000146
-
Y Yang
./.
11
c.1126A>G
r.(?)
p.(Thr376Ala)
VariO:0136 DNA substitution; VariO:0313 transition; VariO:0315 purine transition
VariO:0021 amino acid substitution
g.193360824A>G
Cohn AC1, Toomes C, Potter C, Towns KV, Hewitt AW, Inglehearn CF, Craig JE, Mackey DA.(2007)
OPA1_000113
-
Y Yang
./.
11
c.1129G>A
r.(?)
p.(Val377Ile)
VariO:0136 DNA substitution; VariO:0313 transition; VariO:0315 purine transition
VariO:0021 amino acid substitution
g.193360827G>A
Chen Y1, Jia X, Wang P, Xiao X, Li S, Guo X, Zhang Q.(2013)
OPA1_000218
-
Y Yang
./.
11
c.1140G>A
r.(=)
p.(=)
-
-
g.193360838G>A
Loo JL et al. (2017)
OPA1_000251
-
XY Liu
./.
11i
c.1140+1G>A
r.spl?
p.?
VariO:0136 DNA substitution; VariO:0313 transition; VariO:0315 purine transition
-
g.193360839G>A
Baris O1, Delettre C, Amati-Bonneau P, Surget MO, Charlin JF, Catier A, Derieux L, Guyomard JL, Dollfus H, Jonveaux P, Ayuso C, Maumenee I, Lorenz B, Mohammed S, Tourmen Y, Bonneau D, Malthièry Y, Hamel C, Reynier P.(2003)
OPA1_000112
-
Y Yang
./.
11i
c.1140+5G>C
r.spl?
p.?
VariO:0136 DNA substitution; VariO:0316 transversion
-
g.193360843G>C
Ferré M1, Bonneau D, Milea D, Chevrollier A, Verny C, Dollfus H, Ayuso C, Defoort S, Vignal C, Zanlonghi X, Charlin JF, Kaplan J, Odent S, Hamel CP, Procaccio V, Reynier P, Amati-Bonneau P.(2009)
OPA1_000111
-
Y Yang
./.
11i
c.1140+5G>C
r.spl?
p.?
VariO:0136 DNA substitution; VariO:0316 transversion
-
g.193360843G>C
Kamakari S1, Koutsodontis G1, Tsilimbaris M2, Fitsios A3, Chrousos G3.(2014)
OPA1_000221
-
Y Yang
./.
12
c.1146A>G
r.(?)
p.(Ile382Met)
VariO:0136 DNA substitution; VariO:0313 transition; VariO:0315 purine transition
VariO:0021 amino acid substitution
g.193361167A>G
Ferré M1, Bonneau D, Milea D, Chevrollier A, Verny C, Dollfus H, Ayuso C, Defoort S, Vignal C, Zanlonghi X, Charlin JF, Kaplan J, Odent S, Hamel CP, Procaccio V, Reynier P, Amati-Bonneau P.(2009)
OPA1_000147
-
Y Yang
./.
12
c.1146A>G
r.(?)
p.(Ile382Met)
VariO:0136 DNA substitution; VariO:0313 transition; VariO:0315 purine transition
VariO:0021 amino acid substitution
g.193361167A>G
Schaaf CP1, Blazo M, Lewis RA, Tonini RE, Takei H, Wang J, Wong LJ, Scaglia F.(2011)
OPA1_000147
-
Y Yang
./.
12
c.1146A>G
r.(?)
p.(Ile382Met)
VariO:0136 DNA substitution; VariO:0313 transition; VariO:0315 purine transition
VariO:0021 amino acid substitution
g.193361167A>G
Bonifert T1, Karle KN2, Tonagel F3, Batra M4, Wilhelm C5, Theurer Y2, Schoenfeld C2, Kluba T6, Kamenisch Y7, Carelli V8, Wolf J2, Gonzalez MA9, Speziani F9, Schüle R10, Züchner S9, Sch?ls L2, Wissinger B11, Synofzik M2.(2014)
OPA1_000147
-
Y Yang
./.
12
c.1146A>G
r.(?)
p.(Ile382Met)
-
-
g.193361167A>G
Kane MS et al. (2017)
OPA1_000147
-
XY Liu
./.
12
c.1152A>T
r.(?)
p.(Leu384Phe)
VariO:0136 DNA substitution; VariO:0316 transversion
VariO:0021 amino acid substitution
g.193361173A>T
Toomes C1, Marchbank NJ, Mackey DA, Craig JE, Newbury-Ecob RA, Bennett CP, Vize CJ, Desai SP, Black GC, Patel N, Teimory M, Markham AF, Inglehearn CF, Churchill AJ.(2001)
OPA1_000148
-
Y Yang
./.
12
c.1152_1153del2
r.(?)
p.(Asn385Cysfs*13)
VariO:0141 DNA deletion
VariO:0023 amphigoric amino acid indel
g.193361173_193361174del2
Pesch UE1, Leo-Kottler B, Mayer S, Jurklies B, Kellner U, Apfelstedt-Sylla E, Zrenner E, Alexander C, Wissinger B.(2001)
OPA1_000110
-
Y Yang
./.
12
c.1187T>C
r.(?)
p.(Leu396Pro)
VariO:0136 DNA substitution; VariO:0313 transition; VariO:0314 pyrimidine transition
VariO:0021 amino acid substitution
g.193361208T>C
Ferré M1, Bonneau D, Milea D, Chevrollier A, Verny C, Dollfus H, Ayuso C, Defoort S, Vignal C, Zanlonghi X, Charlin JF, Kaplan J, Odent S, Hamel CP, Procaccio V, Reynier P, Amati-Bonneau P.(2009)
OPA1_000149
-
Y Yang
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