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Phenotypes for disease #02076 (SACS (ataxia, spastic, Charlevoix-Saguenay type (SACS)), OMIM:270550)
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.
Phenotype details
: additional information on the phenotype of the individual, preferably use HPO terms only (http://www.human-phenotype-ontology.org/)
Diagnosis/Initial
: initial diagnosis, before molecular testing
Diagnosis/Definite
: phenotype individual after molecular testing (OMIM abbreviation)
Inheritance
: Indicates the inheritance of the phenotype in the family; unknown, familial (autosomal/X-linked, dominant/ recessive), paternal (Y-linked), maternal (mitochondrial), isolated (sporadic) or complex
All options:
Unknown
Familial
Familial, autosomal dominant
Familial, autosomal recessive
Familial, X-linked
Familial, X-linked dominant
Familial, X-linked dominant, male sparing
Familial, X-linked recessive
Paternal, Y-linked
Maternal, mitochondrial
Isolated (sporadic)
Di-genic
Complex
- = Not applicable
Age/Examination
: age at which the individual was examined.
35y = 35 years
04y08m = 4 years and 8 months
00y00m01d12h = 1 day and 12 hours
18y? = around 18 years
30y-40y = between 30 and 40 years
>54y = older than 54
? = unknown
Age/Diagnosis
: age diagnosis was confirmed
35y = 35 years
04y08m = 4 years and 8 months
00y00m01d12h = 1 day and 12 hours
18y? = around 18 years
30y-40y = between 30 and 40 years
>54y = older than 54
? = unknown
Age/Onset
: Age first symptoms disease appeared in individual:
35y = 35 years
04y08m = 4 years and 8 months
00y00m01d12h = 1 day and 12 hours
18y? = around 18 years
30y-40y = between 30 and 40 years
>54y = older than 54
? = unknown
Phenotype/Onset
: individual's phenotype at Age/Onset described using HPO
Protein
: result from protein staining
How to query this table
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Text
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all entries containing 'Arg'
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Text
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|
Text
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!
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'
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Text
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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'
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99 entries on 1 page. Showing entries 1 - 99.
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Legend
How to query
Phenotype ID
Phenotype details
Diagnosis/Initial
Diagnosis/Definite
Inheritance
Age/Examination
Age/Diagnosis
Age/Onset
Phenotype/Onset
Protein
Owner
Individual ID
0000045742
ataxia, lower limb spasticity, muscle wasting, dysarthria, congnitive impairment, loss of vibration sensation
-
-
Familial, autosomal recessive
38y
-
11y
-
-
Mahmoud Koko
00059237
0000117419
-
ataxia, spastic, Charlevoix-Saguenay type (SACS)
-
Unknown
-
-
-
-
-
Bernard Brais
00144685
0000117422
-
ataxia, spastic, Charlevoix-Saguenay type (SACS)
-
Familial, autosomal recessive
-
-
-
-
-
Bernard Brais
00144688
0000117423
-
ataxia, spastic, Charlevoix-Saguenay type (SACS)
-
Familial
-
-
-
-
-
Bernard Brais
00144689
0000117424
-
ataxia, spastic, Charlevoix-Saguenay type, autosomal recessive (ARSACS)
-
Unknown
-
-
-
-
-
Bernard Brais
00144690
0000117425
-
ataxia, spastic, Charlevoix-Saguenay type, autosomal recessive (ARSACS)
-
Familial, autosomal recessive
-
-
-
-
-
Bernard Brais
00144691
0000117430
-
ataxia, spastic, Charlevoix-Saguenay type, autosomal recessive (ARSACS)
-
Isolated (sporadic)
-
-
-
-
-
Bernard Brais
00144696
0000117431
-
ataxia, spastic, Charlevoix-Saguenay type, autosomal recessive (ARSACS)
-
Isolated (sporadic)
-
-
-
-
-
Bernard Brais
00144697
0000117432
-
ataxia, spastic, Charlevoix-Saguenay type, autosomal recessive (ARSACS)
-
Unknown
-
-
-
-
-
Bernard Brais
00144698
0000117433
-
ataxia, spastic, Charlevoix-Saguenay type, autosomal recessive (ARSACS)
-
Unknown
-
-
-
-
-
Bernard Brais
00144699
0000117436
-
ataxia, spastic, Charlevoix-Saguenay type, autosomal recessive (ARSACS)
-
Isolated (sporadic)
-
-
-
-
-
Bernard Brais
00144702
0000117437
-
ataxia, spastic, Charlevoix-Saguenay type, autosomal recessive (ARSACS)
-
Unknown
-
-
-
-
-
Bernard Brais
00144703
0000117439
-
ataxia, spastic, Charlevoix-Saguenay type, autosomal recessive (ARSACS)
-
Isolated (sporadic)
-
-
-
-
-
Bernard Brais
00144705
0000117440
-
ataxia, spastic, Charlevoix-Saguenay type, autosomal recessive (ARSACS)
-
Unknown
-
-
-
-
-
Bernard Brais
00144706
0000117441
-
ataxia, spastic, Charlevoix-Saguenay type, autosomal recessive (ARSACS)
-
Unknown
-
-
-
-
-
Bernard Brais
00144707
0000117442
-
ataxia, spastic, Charlevoix-Saguenay type, autosomal recessive (ARSACS)
-
Unknown
-
-
-
-
-
Bernard Brais
00144708
0000117454
-
ataxia, spastic, Charlevoix-Saguenay type, autosomal recessive (ARSACS)
-
Isolated (sporadic)
-
-
-
-
-
Bernard Brais
00144720
0000117455
-
ataxia, spastic, Charlevoix-Saguenay type, autosomal recessive (ARSACS)
-
Isolated (sporadic)
-
-
-
-
-
Bernard Brais
00144721
0000117456
-
ataxia, spastic, Charlevoix-Saguenay type, autosomal recessive (ARSACS)
-
Isolated (sporadic)
-
-
-
-
-
Bernard Brais
00144722
0000117457
-
ataxia, spastic, Charlevoix-Saguenay type, autosomal recessive (ARSACS)
-
Isolated (sporadic)
-
-
-
-
-
Bernard Brais
00144723
0000117458
-
ataxia, spastic, Charlevoix-Saguenay type (SACS)
-
Isolated (sporadic)
-
-
-
-
-
Bernard Brais
00144724
0000117459
-
ataxia, spastic, Charlevoix-Saguenay type (SACS)
-
Unknown
-
-
-
-
-
Bernard Brais
00144725
0000117460
-
ataxia, spastic, Charlevoix-Saguenay type, autosomal recessive (ARSACS)
-
Unknown
-
-
-
-
-
Bernard Brais
00144726
0000117461
-
ataxia, spastic, Charlevoix-Saguenay type (SACS)
-
Isolated (sporadic)
-
-
-
-
-
Bernard Brais
00144727
0000117469
-
ataxia, spastic, Charlevoix-Saguenay type (SACS)
-
Familial, autosomal recessive
-
-
-
-
-
Bernard Brais
00144735
0000117474
-
ataxia, spastic, Charlevoix-Saguenay type (SACS)
-
Unknown
-
-
-
-
-
Bernard Brais
00144740
0000117477
-
ataxia, spastic, Charlevoix-Saguenay type, autosomal recessive (ARSACS)
-
Isolated (sporadic)
-
-
-
-
-
Bernard Brais
00144743
0000117481
-
ataxia, spastic, Charlevoix-Saguenay type (SACS)
-
Isolated (sporadic)
-
-
-
-
-
Bernard Brais
00144747
0000117482
-
ataxia, spastic, Charlevoix-Saguenay type, autosomal recessive (ARSACS)
-
Familial, autosomal recessive
-
-
-
-
-
Bernard Brais
00144748
0000117489
-
ataxia, spastic, Charlevoix-Saguenay type, autosomal recessive (ARSACS)
-
Familial, autosomal recessive
-
-
-
-
-
Bernard Brais
00144755
0000117492
-
ataxia, spastic, Charlevoix-Saguenay type (SACS)
-
Unknown
-
-
-
-
-
Bernard Brais
00144758
0000117494
-
ataxia, spastic, Charlevoix-Saguenay type, autosomal recessive (ARSACS)
-
Isolated (sporadic)
-
-
-
-
-
Bernard Brais
00144760
0000117495
-
ataxia, spastic, Charlevoix-Saguenay type (SACS)
-
Unknown
-
-
-
-
-
Bernard Brais
00144761
0000117496
-
ataxia, spastic, Charlevoix-Saguenay type (SACS)
-
Familial
-
-
-
-
-
Bernard Brais
00144762
0000117497
-
ataxia, spastic, Charlevoix-Saguenay type (SACS)
-
Unknown
-
-
-
-
-
Bernard Brais
00144763
0000117501
-
ataxia, spastic, Charlevoix-Saguenay type (SACS)
-
Unknown
-
-
-
-
-
Bernard Brais
00144767
0000117504
-
ataxia, spastic, Charlevoix-Saguenay type (SACS)
-
Unknown
-
-
-
-
-
Bernard Brais
00144770
0000117505
-
ataxia, spastic, Charlevoix-Saguenay type (SACS)
-
Unknown
-
-
-
-
-
Bernard Brais
00144771
0000117506
-
ataxia, spastic, Charlevoix-Saguenay type (SACS)
-
Unknown
-
-
-
-
-
Bernard Brais
00144772
0000117510
-
ataxia, spastic, Charlevoix-Saguenay type (SACS)
-
Isolated (sporadic)
-
-
-
-
-
Bernard Brais
00144776
0000117511
-
ataxia, spastic, Charlevoix-Saguenay type (SACS)
-
Unknown
-
-
-
-
-
Bernard Brais
00144777
0000117515
-
ataxia, spastic, Charlevoix-Saguenay type, autosomal recessive (ARSACS)
-
Unknown
-
-
-
-
-
Bernard Brais
00144781
0000117517
-
ataxia, spastic, Charlevoix-Saguenay type (SACS)
-
Isolated (sporadic)
-
-
-
-
-
Bernard Brais
00144783
0000117518
-
ataxia, spastic, Charlevoix-Saguenay type, autosomal recessive (ARSACS)
-
Isolated (sporadic)
-
-
-
-
-
Bernard Brais
00144784
0000117519
-
ataxia, spastic, Charlevoix-Saguenay type, autosomal recessive (ARSACS)
-
Unknown
-
-
-
-
-
Bernard Brais
00144785
0000117520
-
ataxia, spastic, Charlevoix-Saguenay type, autosomal recessive (ARSACS)
-
Unknown
-
-
-
-
-
Bernard Brais
00144786
0000117521
-
ataxia, spastic, Charlevoix-Saguenay type, autosomal recessive (ARSACS)
-
Unknown
-
-
-
-
-
Bernard Brais
00144787
0000117522
-
ataxia, spastic, Charlevoix-Saguenay type, autosomal recessive (ARSACS)
-
Unknown
-
-
-
-
-
Bernard Brais
00144788
0000117523
-
ataxia, spastic, Charlevoix-Saguenay type, autosomal recessive (ARSACS)
-
Unknown
-
-
-
-
-
Bernard Brais
00144789
0000117525
-
ataxia, spastic, Charlevoix-Saguenay type (SACS)
-
Isolated (sporadic)
-
-
-
-
-
Bernard Brais
00144791
0000117526
-
ataxia, spastic, Charlevoix-Saguenay type (SACS)
-
Unknown
-
-
-
-
-
Bernard Brais
00144792
0000117528
-
ataxia, spastic, Charlevoix-Saguenay type (SACS)
-
Isolated (sporadic)
-
-
-
-
-
Johan den Dunnen
00144794
0000117529
-
ataxia, spastic, Charlevoix-Saguenay type, autosomal recessive (ARSACS)
-
Isolated (sporadic)
-
-
-
-
-
Bernard Brais
00144795
0000117530
-
ataxia, spastic, Charlevoix-Saguenay type, autosomal recessive (ARSACS)
-
Isolated (sporadic)
-
-
-
-
-
Bernard Brais
00144796
0000117534
-
ataxia, spastic, Charlevoix-Saguenay type (SACS)
-
Unknown
-
-
-
-
-
Bernard Brais
00144800
0000117537
-
ataxia, spastic, Charlevoix-Saguenay type (SACS)
-
Unknown
-
-
-
-
-
Bernard Brais
00144803
0000117538
-
ataxia, spastic, Charlevoix-Saguenay type, autosomal recessive (ARSACS)
-
Unknown
-
-
-
-
-
Bernard Brais
00144804
0000117539
-
ataxia, spastic, Charlevoix-Saguenay type, autosomal recessive (ARSACS)
-
Unknown
-
-
-
-
-
Bernard Brais
00144805
0000117541
-
ataxia, spastic, Charlevoix-Saguenay type, autosomal recessive (ARSACS)
-
Isolated (sporadic)
-
-
-
-
-
Bernard Brais
00144807
0000117543
-
ataxia, spastic, Charlevoix-Saguenay type, autosomal recessive (ARSACS)
-
Unknown
-
-
-
-
-
Bernard Brais
00144809
0000117544
-
ataxia, spastic, Charlevoix-Saguenay type, autosomal recessive (ARSACS)
-
Isolated (sporadic)
-
-
-
-
-
Bernard Brais
00144810
0000117545
-
ataxia, spastic, Charlevoix-Saguenay type, autosomal recessive (ARSACS)
-
Isolated (sporadic)
-
-
-
-
-
Bernard Brais
00144811
0000117546
-
ataxia, spastic, Charlevoix-Saguenay type (SACS)
-
Unknown
-
-
-
-
-
Bernard Brais
00144812
0000117548
-
ataxia, spastic, Charlevoix-Saguenay type (SACS)
-
Unknown
-
-
-
-
-
Bernard Brais
00144814
0000117552
-
ataxia, spastic, Charlevoix-Saguenay type (SACS)
-
Unknown
-
-
-
-
-
Bernard Brais
00144818
0000117555
-
ataxia, spastic, Charlevoix-Saguenay type (SACS)
-
Unknown
-
-
-
-
-
Bernard Brais
00144821
0000117556
-
ataxia, spastic, Charlevoix-Saguenay type, autosomal recessive (ARSACS)
-
Isolated (sporadic)
-
-
-
-
-
Bernard Brais
00144822
0000117559
-
ataxia, spastic, Charlevoix-Saguenay type (SACS)
-
Unknown
-
-
-
-
-
Bernard Brais
00144825
0000117560
-
ataxia, spastic, Charlevoix-Saguenay type (SACS)
-
Unknown
-
-
-
-
-
Bernard Brais
00144826
0000117561
-
ataxia, spastic, Charlevoix-Saguenay type (SACS)
-
Unknown
-
-
-
-
-
Bernard Brais
00144827
0000117565
-
ataxia, spastic, Charlevoix-Saguenay type, autosomal recessive (ARSACS)
-
Isolated (sporadic)
-
-
-
-
-
Bernard Brais
00144831
0000117567
-
ataxia, spastic, Charlevoix-Saguenay type (SACS)
-
Unknown
-
-
-
-
-
Bernard Brais
00144833
0000117568
-
ataxia, spastic, Charlevoix-Saguenay type, autosomal recessive (ARSACS)
-
Unknown
-
-
-
-
-
Bernard Brais
00144834
0000117569
-
ataxia, spastic, Charlevoix-Saguenay type, autosomal recessive (ARSACS)
-
Unknown
-
-
-
-
-
Bernard Brais
00144835
0000117570
-
ataxia, spastic, Charlevoix-Saguenay type, autosomal recessive (ARSACS)
-
Unknown
-
-
-
-
-
Bernard Brais
00144836
0000117571
-
ataxia, spastic, Charlevoix-Saguenay type, autosomal recessive (ARSACS)
-
Familial, autosomal recessive
-
-
-
-
-
Bernard Brais
00144837
0000117575
-
ataxia, spastic, Charlevoix-Saguenay type (SACS)
-
Unknown
-
-
-
-
-
Bernard Brais
00144841
0000117577
-
ataxia, spastic, Charlevoix-Saguenay type, autosomal recessive (ARSACS)
-
Isolated (sporadic)
-
-
-
-
-
Bernard Brais
00144843
0000117578
-
ataxia, spastic, Charlevoix-Saguenay type (SACS)
-
Unknown
-
-
-
-
-
Bernard Brais
00144844
0000117579
-
ataxia, spastic, Charlevoix-Saguenay type (SACS)
-
Unknown
-
-
-
-
-
Bernard Brais
00144845
0000117580
-
ataxia, spastic, Charlevoix-Saguenay type, autosomal recessive (ARSACS)
-
Familial, autosomal recessive
-
-
-
-
-
Bernard Brais
00144846
0000117581
-
ataxia, spastic, Charlevoix-Saguenay type, autosomal recessive (ARSACS)
-
Isolated (sporadic)
-
-
-
-
-
Bernard Brais
00144847
0000117585
-
ataxia, spastic, Charlevoix-Saguenay type, autosomal recessive (ARSACS)
-
Unknown
-
-
-
-
-
Bernard Brais
00144851
0000117593
-
ataxia, spastic, Charlevoix-Saguenay type (SACS)
-
Unknown
-
-
-
-
-
Bernard Brais
00144859
0000117594
-
ataxia, spastic, Charlevoix-Saguenay type (SACS)
-
Unknown
-
-
-
-
-
Bernard Brais
00144860
0000117595
-
ataxia, spastic, Charlevoix-Saguenay type, autosomal recessive (ARSACS)
-
Isolated (sporadic)
-
-
-
-
-
Bernard Brais
00144861
0000117596
-
ataxia, spastic, Charlevoix-Saguenay type, autosomal recessive (ARSACS)
-
Isolated (sporadic)
-
-
-
-
-
Bernard Brais
00144862
0000117597
-
ataxia, spastic, Charlevoix-Saguenay type, autosomal recessive (ARSACS)
-
Isolated (sporadic)
-
-
-
-
-
Bernard Brais
00144863
0000117598
-
ataxia, spastic, Charlevoix-Saguenay type, autosomal recessive (ARSACS)
-
Isolated (sporadic)
-
-
-
-
-
Bernard Brais
00144864
0000117599
-
ataxia, spastic, Charlevoix-Saguenay type (SACS)
-
Unknown
-
-
-
-
-
Bernard Brais
00144865
0000117601
-
ataxia, spastic, Charlevoix-Saguenay type (SACS)
-
Isolated (sporadic)
-
-
-
-
-
Bernard Brais
00144867
0000117602
-
ataxia, spastic, Charlevoix-Saguenay type (SACS)
-
Isolated (sporadic)
-
-
-
-
-
Bernard Brais
00144868
0000117605
-
ataxia, spastic, Charlevoix-Saguenay type (SACS)
-
Unknown
-
-
-
-
-
Bernard Brais
00144871
0000117612
-
ataxia, spastic, Charlevoix-Saguenay type, autosomal recessive (ARSACS)
-
Isolated (sporadic)
-
-
-
-
-
Bernard Brais
00144878
0000117613
-
ataxia, spastic, Charlevoix-Saguenay type (SACS)
-
Unknown
-
-
-
-
-
Bernard Brais
00144879
0000117614
-
ataxia, spastic, Charlevoix-Saguenay type (SACS)
-
Unknown
-
-
-
-
-
Bernard Brais
00144880
0000117618
-
ataxia, spastic, Charlevoix-Saguenay type (SACS)
-
Unknown
-
-
-
-
-
Bernard Brais
00144884
0000296705
30-y male with progressive cerebellar ataxia, weakness and wasting of upper and lower limbs marked in hands and feet, stocking and glove hypesthesia, deep sensory loss, Babinski sign and bilateral pes cavus
spinocerebellar ataxia
SACs
Familial, autosomal recessive
27y
-
22y
-
-
Sherifa Ahmed Hamed
00404116
0000296710
18-y male with progressive ataxia, peripheral neuropathy, Babinski sign, pes cavus and mental retardation. He had delayed physical and mental development.
SCA
SACs
Familial, autosomal recessive
14y
-
08y
-
-
Sherifa Ahmed Hamed
00404121
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