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

 Phenotype ID
|

 Phenotype details
|

 Diagnosis/Initial
|

 Diagnosis/Definite
|

 Inheritance
|

 Age/Examination
|

 Age/Diagnosis
|

 Age/Onset
|

 Phenotype/Onset
|

 Protein
|

 Owner
|

 Individual ID
|
| 0000023250 |
see paper; pure form of cerebellar ataxia |
- |
- |
Familial, autosomal dominant |
- |
- |
- |
- |
- |
Johan den Dunnen |
00027179 |
| 0000023251 |
see paper |
- |
- |
Familial, autosomal dominant |
- |
- |
- |
- |
- |
Johan den Dunnen |
00027180 |
| 0000023252 |
see paper |
- |
- |
Familial, autosomal dominant |
- |
- |
- |
- |
- |
Johan den Dunnen |
00027181 |
| 0000023253 |
see paper |
- |
- |
Familial, autosomal dominant |
- |
- |
- |
- |
- |
Johan den Dunnen |
00027182 |
| 0000023254 |
- |
- |
- |
Unknown |
- |
- |
- |
- |
- |
Johan den Dunnen |
00027183 |
| 0000087311 |
possibly autosomal recessive |
- |
- |
Isolated (sporadic) |
- |
- |
- |
- |
- |
Johan den Dunnen |
00111198 |
| 0000120915 |
- |
- |
- |
Familial, autosomal dominant |
- |
- |
- |
- |
- |
Johan den Dunnen |
00148466 |
| 0000120916 |
- |
- |
- |
Familial, autosomal dominant |
- |
- |
- |
- |
- |
Johan den Dunnen |
00148467 |
| 0000120968 |
- |
- |
- |
Isolated (sporadic) |
- |
- |
- |
- |
- |
Johan den Dunnen |
00148524 |
| 0000120969 |
- |
- |
- |
Familial, autosomal dominant |
- |
- |
- |
- |
- |
Johan den Dunnen |
00148525 |
| 0000120970 |
- |
- |
- |
Familial, autosomal dominant |
- |
- |
- |
- |
- |
Johan den Dunnen |
00148526 |
| 0000120971 |
- |
SCA |
SCA-6 |
Familial, autosomal dominant |
- |
- |
37y |
- |
- |
Johan den Dunnen |
00148528 |
| 0000120972 |
- |
SCA |
SCA-6 |
Familial, autosomal dominant |
- |
- |
40y |
- |
- |
Johan den Dunnen |
00148529 |
| 0000120973 |
- |
- |
- |
Familial, autosomal dominant |
- |
- |
- |
- |
- |
Johan den Dunnen |
00148545 |
| 0000130012 |
dysarthria (HP:0001260), ataxia (HP:0001251 ) |
ataxia, spinocerebellar |
SCA-37 |
Familial, autosomal dominant |
64y |
- |
30y |
dysarthria |
- |
Johan den Dunnen |
00165115 |
| 0000157151 |
- |
SCA |
SCA-6 |
Familial, autosomal dominant |
- |
- |
- |
32y |
- |
Johan den Dunnen |
00148527 |
| 0000157152 |
- |
SCA |
SCA-6 |
Familial, autosomal dominant |
- |
- |
58y |
- |
- |
Johan den Dunnen |
00208463 |
| 0000157153 |
- |
SCA |
SCA-6 |
Familial, autosomal dominant |
- |
- |
33y |
- |
- |
Johan den Dunnen |
00208464 |
| 0000157154 |
- |
SCA |
SCA-6 |
Familial, autosomal dominant |
- |
- |
32y |
- |
- |
Johan den Dunnen |
00208465 |
| 0000157155 |
- |
SCA |
SCA-6 |
Familial, autosomal dominant |
- |
- |
24y |
- |
- |
Johan den Dunnen |
00208466 |
| 0000157156 |
- |
SCA |
SCA-6 |
Familial, autosomal dominant |
- |
- |
25y |
- |
- |
Johan den Dunnen |
00208467 |
| 0000157157 |
- |
SCA |
SCA-6 |
Familial, autosomal dominant |
- |
- |
45y |
- |
- |
Johan den Dunnen |
00208468 |
| 0000157158 |
- |
SCA |
SCA-6 |
Familial, autosomal dominant |
- |
- |
33y |
- |
- |
Johan den Dunnen |
00208469 |
| 0000157159 |
- |
SCA |
SCA-6 |
Familial, autosomal dominant |
- |
- |
35y |
- |
- |
Johan den Dunnen |
00208470 |
| 0000157160 |
- |
SCA |
SCA-6 |
Familial, autosomal dominant |
- |
- |
37y |
- |
- |
Johan den Dunnen |
00208471 |
| 0000157161 |
- |
SCA |
SCA-6 |
Familial, autosomal dominant |
- |
- |
38y |
- |
- |
Johan den Dunnen |
00208472 |
| 0000157162 |
- |
SCA |
SCA-6 |
Familial, autosomal dominant |
- |
- |
39y |
- |
- |
Johan den Dunnen |
00208473 |
| 0000157163 |
- |
SCA |
SCA-6 |
Familial, autosomal dominant |
- |
- |
40y |
- |
- |
Johan den Dunnen |
00208474 |
| 0000157164 |
- |
SCA |
SCA-6 |
Familial, autosomal dominant |
- |
- |
43y |
- |
- |
Johan den Dunnen |
00208475 |
| 0000157165 |
- |
SCA |
SCA-6 |
Familial, autosomal dominant |
- |
- |
44y |
- |
- |
Johan den Dunnen |
00208476 |
| 0000157166 |
- |
SCA |
SCA-6 |
Familial, autosomal dominant |
- |
- |
45y |
- |
- |
Johan den Dunnen |
00208477 |
| 0000157167 |
- |
SCA |
SCA-6 |
Familial, autosomal dominant |
- |
- |
50y |
- |
- |
Johan den Dunnen |
00208478 |
| 0000157168 |
- |
SCA |
SCA-6 |
Familial, autosomal dominant |
- |
- |
56y |
- |
- |
Johan den Dunnen |
00208479 |
| 0000157169 |
- |
SCA |
SCA-6 |
Familial, autosomal dominant |
- |
- |
59y |
- |
- |
Johan den Dunnen |
00208480 |
| 0000157170 |
- |
SCA |
SCA-6 |
Familial, autosomal dominant |
- |
- |
38y |
- |
- |
Johan den Dunnen |
00208481 |
| 0000157171 |
- |
SCA |
SCA-6 |
Familial, autosomal dominant |
- |
- |
40y |
- |
- |
Johan den Dunnen |
00208482 |
| 0000157172 |
- |
SCA |
SCA-6 |
Familial, autosomal dominant |
- |
- |
43y |
- |
- |
Johan den Dunnen |
00208483 |
| 0000157173 |
- |
SCA |
SCA-6 |
Familial, autosomal dominant |
- |
- |
47y |
- |
- |
Johan den Dunnen |
00208484 |
| 0000157174 |
- |
SCA |
SCA-6 |
Familial, autosomal dominant |
- |
- |
48y |
- |
- |
Johan den Dunnen |
00208485 |
| 0000157175 |
- |
SCA |
SCA-6 |
Familial, autosomal dominant |
- |
- |
50y |
- |
- |
Johan den Dunnen |
00208486 |
| 0000157176 |
- |
SCA |
SCA-6 |
Familial, autosomal dominant |
- |
- |
52y |
- |
- |
Johan den Dunnen |
00208487 |
| 0000157177 |
- |
SCA |
SCA-6 |
Familial, autosomal dominant |
- |
- |
55y |
- |
- |
Johan den Dunnen |
00208488 |
| 0000157178 |
- |
SCA |
SCA-6 |
Familial, autosomal dominant |
- |
- |
56y |
- |
- |
Johan den Dunnen |
00208489 |
| 0000157179 |
- |
SCA |
SCA-6 |
Familial, autosomal dominant |
- |
- |
60y |
- |
- |
Johan den Dunnen |
00208490 |
| 0000157180 |
- |
SCA |
SCA-6 |
Familial, autosomal dominant |
- |
- |
40y |
- |
- |
Johan den Dunnen |
00208491 |
| 0000157181 |
- |
SCA |
SCA-6 |
Familial, autosomal dominant |
- |
- |
42y |
- |
- |
Johan den Dunnen |
00208492 |
| 0000157182 |
- |
SCA |
SCA-6 |
Familial, autosomal dominant |
- |
- |
43y |
- |
- |
Johan den Dunnen |
00208493 |
| 0000157183 |
- |
SCA |
SCA-6 |
Familial, autosomal dominant |
- |
- |
46y |
- |
- |
Johan den Dunnen |
00208494 |
| 0000157184 |
- |
SCA |
SCA-6 |
Familial, autosomal dominant |
- |
- |
48y |
- |
- |
Johan den Dunnen |
00208495 |
| 0000157185 |
- |
SCA |
SCA-6 |
Familial, autosomal dominant |
- |
- |
52y |
- |
- |
Johan den Dunnen |
00208496 |
| 0000157186 |
- |
SCA |
SCA-6 |
Familial, autosomal dominant |
- |
- |
53y |
- |
- |
Johan den Dunnen |
00208497 |
| 0000157187 |
- |
SCA |
SCA-6 |
Familial, autosomal dominant |
- |
- |
55y |
- |
- |
Johan den Dunnen |
00208498 |
| 0000157188 |
- |
SCA |
SCA-6 |
Familial, autosomal dominant |
- |
- |
57y |
- |
- |
Johan den Dunnen |
00208499 |
| 0000157189 |
- |
SCA |
SCA-6 |
Familial, autosomal dominant |
- |
- |
58y |
- |
- |
Johan den Dunnen |
00208500 |
| 0000157190 |
- |
SCA |
SCA-6 |
Familial, autosomal dominant |
- |
- |
59y |
- |
- |
Johan den Dunnen |
00208501 |
| 0000157191 |
- |
SCA |
SCA-6 |
Familial, autosomal dominant |
- |
- |
60y |
- |
- |
Johan den Dunnen |
00208502 |
| 0000157192 |
- |
SCA |
SCA-6 |
Familial, autosomal dominant |
- |
- |
67y |
- |
- |
Johan den Dunnen |
00208503 |
| 0000157193 |
- |
SCA |
SCA-6 |
Familial, autosomal dominant |
- |
- |
64y |
- |
- |
Johan den Dunnen |
00208504 |
| 0000157194 |
- |
SCA |
SCA-6 |
Familial, autosomal dominant |
- |
- |
63y |
- |
- |
Johan den Dunnen |
00208505 |
| 0000157195 |
- |
SCA |
SCA-6 |
Familial, autosomal dominant |
- |
- |
61y |
- |
- |
Johan den Dunnen |
00208506 |
| 0000157196 |
- |
SCA |
SCA-6 |
Familial, autosomal dominant |
- |
- |
61y |
- |
- |
Johan den Dunnen |
00208507 |
| 0000157197 |
- |
SCA |
SCA-6 |
Familial, autosomal dominant |
- |
- |
56y |
- |
- |
Johan den Dunnen |
00208508 |
| 0000157198 |
- |
SCA |
SCA-6 |
Familial, autosomal dominant |
- |
- |
39y |
- |
- |
Johan den Dunnen |
00208509 |
| 0000157235 |
severe ataxia, mild dysarthria, mild down-beat nystagmus, diabetes mellitus |
spinocerebellar ataxia |
SCA-6 |
Familial, autosomal dominant |
83y |
- |
58y |
- |
- |
Johan den Dunnen |
00208604 |
| 0000157236 |
moderate ataxia, mild dysarthria, moderate down-beat nystagmus, diabetes mellitus; phenotype not more severe compared to heterozygous cases |
spinocerebellar ataxia |
SCA-6 |
Familial, autosomal dominant |
63y |
- |
60y |
- |
- |
Johan den Dunnen |
00208606 |
| 0000157237 |
severe ataxia, mild dysarthria, mild down-beat nystagmus, diabetes mellitus |
spinocerebellar ataxia |
SCA-6 |
Familial, autosomal dominant |
80y |
- |
54y |
- |
- |
Johan den Dunnen |
00208607 |
| 0000157238 |
mild ataxia, no dysarthria, noe down-beat nystagmus |
spinocerebellar ataxia |
SCA-6 |
Familial, autosomal dominant |
75y |
- |
75y |
- |
- |
Johan den Dunnen |
00208608 |
| 0000157239 |
mild ataxia, no dysarthria, mild down-beat nystagmus; phenotype not more severe compared to heterozygous cases |
spinocerebellar ataxia |
SCA-6 |
Familial, autosomal dominant |
52y |
- |
>51y |
- |
- |
Johan den Dunnen |
00208609 |
| 0000174473 |
see paper; ... |
cerebellar ataxia with retinal degeneration (ADCA type II) |
SCA-7 |
Familial, autosomal dominant |
- |
- |
- |
- |
- |
Johan den Dunnen |
00234051 |
| 0000188617 |
- |
Machado-Joseph disease |
MJD |
Familial, autosomal dominant |
- |
- |
- |
- |
- |
Johan den Dunnen |
00249748 |
| 0000188618 |
- |
Machado-Joseph disease |
MJD |
Familial, autosomal dominant |
- |
- |
- |
- |
- |
Johan den Dunnen |
00249749 |
| 0000188619 |
- |
Machado-Joseph disease |
MJD |
Familial, autosomal dominant |
- |
- |
- |
- |
- |
Johan den Dunnen |
00249750 |
| 0000188620 |
- |
Machado-Joseph disease |
MJD |
Familial, autosomal dominant |
- |
- |
- |
- |
- |
Johan den Dunnen |
00249751 |
| 0000188621 |
- |
Machado-Joseph disease |
MJD |
Familial, autosomal dominant |
- |
- |
- |
- |
- |
Johan den Dunnen |
00249752 |
| 0000188622 |
- |
Machado-Joseph disease |
MJD |
Familial, autosomal dominant |
- |
- |
- |
- |
- |
Johan den Dunnen |
00249753 |
| 0000188623 |
- |
Machado-Joseph disease |
MJD |
Familial, autosomal dominant |
- |
- |
- |
- |
- |
Johan den Dunnen |
00249754 |
| 0000188624 |
- |
Machado-Joseph disease |
MJD |
Familial, autosomal dominant |
- |
- |
- |
- |
- |
Johan den Dunnen |
00249755 |
| 0000188625 |
- |
Machado-Joseph disease |
MJD |
Familial, autosomal dominant |
- |
- |
- |
- |
- |
Johan den Dunnen |
00249756 |
| 0000188626 |
- |
Machado-Joseph disease |
MJD |
Familial, autosomal dominant |
- |
- |
- |
- |
- |
Johan den Dunnen |
00249757 |
| 0000188627 |
- |
Machado-Joseph disease |
MJD |
Familial, autosomal dominant |
- |
- |
- |
- |
- |
Johan den Dunnen |
00249758 |
| 0000188628 |
- |
Machado-Joseph disease |
MJD |
Familial, autosomal dominant |
- |
- |
- |
- |
- |
Johan den Dunnen |
00249759 |
| 0000200615 |
- |
cerebellar ataxia |
SCA-7 |
Familial, autosomal dominant |
- |
- |
60y |
- |
- |
Johan den Dunnen |
00262139 |
| 0000200616 |
- |
cerebellar ataxia |
SCA-7 |
Familial, autosomal dominant |
- |
- |
56y |
- |
- |
Johan den Dunnen |
00262140 |
| 0000200617 |
- |
cerebellar ataxia |
SCA-7 |
Familial, autosomal dominant |
- |
- |
45y |
- |
- |
Johan den Dunnen |
00262141 |
| 0000200618 |
- |
cerebellar ataxia |
SCA-7 |
Familial, autosomal dominant |
- |
- |
36y |
- |
- |
Johan den Dunnen |
00262142 |
| 0000200619 |
- |
cerebellar ataxia |
SCA-7 |
Familial, autosomal dominant |
- |
- |
35y |
- |
- |
Johan den Dunnen |
00262143 |
| 0000200620 |
- |
cerebellar ataxia |
SCA-7 |
Familial, autosomal dominant |
- |
- |
31y |
- |
- |
Johan den Dunnen |
00262144 |
| 0000200621 |
- |
cerebellar ataxia |
SCA-7 |
Familial, autosomal dominant |
- |
- |
28y |
- |
- |
Johan den Dunnen |
00262145 |
| 0000200622 |
- |
cerebellar ataxia |
SCA-7 |
Familial, autosomal dominant |
- |
- |
26y |
- |
- |
Johan den Dunnen |
00262146 |
| 0000200623 |
- |
cerebellar ataxia |
SCA-7 |
Familial, autosomal dominant |
- |
- |
25y |
- |
- |
Johan den Dunnen |
00262147 |
| 0000200624 |
- |
cerebellar ataxia |
SCA-7 |
Familial, autosomal dominant |
- |
- |
23y |
- |
- |
Johan den Dunnen |
00262148 |
| 0000200625 |
- |
cerebellar ataxia |
SCA-7 |
Familial, autosomal dominant |
- |
- |
21y |
- |
- |
Johan den Dunnen |
00262149 |
| 0000200626 |
- |
cerebellar ataxia |
SCA-7 |
Familial, autosomal dominant |
- |
- |
19y |
- |
- |
Johan den Dunnen |
00262150 |
| 0000200627 |
- |
cerebellar ataxia |
SCA-7 |
Familial, autosomal dominant |
- |
- |
13y |
- |
- |
Johan den Dunnen |
00262151 |
| 0000200628 |
- |
cerebellar ataxia |
SCA-7 |
Familial, autosomal dominant |
- |
- |
9y |
- |
- |
Johan den Dunnen |
00262152 |
| 0000200629 |
- |
cerebellar ataxia |
SCA-7 |
Familial, autosomal dominant |
- |
- |
8y |
- |
- |
Johan den Dunnen |
00262153 |
| 0000200630 |
- |
cerebellar ataxia |
SCA-7 |
Familial, autosomal dominant |
- |
- |
6y |
- |
- |
Johan den Dunnen |
00262154 |
| 0000200631 |
- |
cerebellar ataxia |
SCA-7 |
Familial, autosomal dominant |
- |
- |
2y |
- |
- |
Johan den Dunnen |
00262155 |
| 0000249903 |
Gait ataxia at age 50y, negative family history regarding ataxia |
50y |
- |
Unknown |
- |
- |
- |
- |
- |
Andreas Laner |
00331711 |
| 0000296711 |
20-y male with progressive ataxia, abnormal ocular eye movement, cognitive impairment distal lower limb weakness and peripheral neuropathy. |
SCA |
AOA2 |
Familial, autosomal recessive |
17y |
- |
13y |
- |
- |
Sherifa Ahmed Hamed |
00404122 |