Global Variome shared LOVD
POLE2 (polymerase (DNA directed), epsilon 2, access...)
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Phenotypes for disease #01943 (LGMDR2;LGMD2B (dystrophy, muscular, limb-girdle, autosomal recessive, type 2 (LGMD2B)), OMIM:253601)
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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
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Date
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Date
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Date
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Date
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Date
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Numeric
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Numeric
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Numeric
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33 entries on 1 page. Showing entries 1 - 33.
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How to query
Phenotype ID
Phenotype details
Diagnosis/Initial
Diagnosis/Definite
Inheritance
Age/Examination
Age/Diagnosis
Age/Onset
Phenotype/Onset
Protein
Owner
Individual ID
0000034253
muscular dystrophy (HP:0003560), elevated serum creatine phosphokinase (HP:0003236), lower limb muscle weakness (HP:0007340)
-
LGMD-2B
Familial, autosomal recessive
-
-
-
-
-
Csilla Lazar
00046458
0000052850
Difficulty to rise from floor (HP:0004302), no myositis (-HP:0100614), Elevated serum creatine phosphokinase (HP:0003236)
-
-
Familial, autosomal recessive
30y
-
18y
Difficulty to rise from floor (HP:0004302)
-
Pieter Klap
00073180
0000053397
no scapular winging (-HP:0003691), unable to stand on tiptoes (HP:0004302), unable to rise from floor (HP:0004302), Elevated serum creatine phosphokinase (HP:0003236), Absent muscle fiber dysferlin (HP:0030114)
-
-
Familial, autosomal recessive
24y
-
21y
-
-
Pieter Klap
00073661
0000053415
no scapular winging (-HP:0003691), unable to stand on tiptoes (HP:0004302), Difficulty walking (HP:0002355), Elevated serum creatine phosphokinase (HP:0003236), Absent muscle fiber dysferlin (HP:0030114),
-
-
Familial, autosomal recessive
41y
-
32y
-
-
Pieter Klap
00073669
0000053417
no scapular winging (-HP:0003691), unable to stand on tiptoes (HP:0004302), unable to rise from floor (HP:0004302), Elevated serum creatine phosphokinase (HP:0003236), Absent muscle fiber dysferlin (HP:0030114),
-
-
Familial, autosomal recessive
28y
-
19y
-
-
Pieter Klap
00073671
0000053418
no scapular winging (-HP:0003691), unable to stand on tiptoes (HP:0004302), Gait disturbance (HP:0001288), Elevated serum creatine phosphokinase (HP:0003236), Absent muscle fiber dysferlin (HP:0030114),
-
-
Familial, autosomal recessive
44y
-
38y
-
-
Pieter Klap
00073672
0000053419
Scapular winging (HP:0003691), unable to stand on tiptoes (HP:0004302), Difficulty climbing stairs (HP:0003551), Elevated serum creatine phosphokinase (HP:0003236), Absent muscle fiber dysferlin (HP:0030114),
-
-
Familial, autosomal recessive
29y
-
21y
-
-
Pieter Klap
00073673
0000053420
no scapular winging (-HP:0003691), unable to stand on tiptoes (HP:0004302), Muscle weakness (HP:0001324), Elevated serum creatine phosphokinase (HP:0003236), Absent muscle fiber dysferlin (HP:0030114),
-
-
Familial, autosomal recessive
27y
-
22y
-
-
Pieter Klap
00073674
0000053421
no scapular winging (-HP:0003691), unable to stand on tiptoes (HP:0004302), Difficulty walking (HP:0002355), Elevated serum creatine phosphokinase (HP:0003236), Absent muscle fiber dysferlin (HP:0030114),
-
-
Familial, autosomal recessive
49y
-
34y
-
-
Pieter Klap
00073675
0000053422
no scapular winging (-HP:0003691), unable to stand on tiptoes (HP:0004302), unable to rise from floor (HP:0004302), Elevated serum creatine phosphokinase (HP:0003236), Absent muscle fiber dysferlin (HP:0030114),
-
-
Familial, autosomal recessive
37y
-
37y
-
-
Pieter Klap
00073676
0000053423
no scapular winging (-HP:0003691), unable to stand on tiptoes (HP:0004302), Gowers sign (HP:0003391), Elevated serum creatine phosphokinase (HP:0003236), Absent muscle fiber dysferlin (HP:0030114),
-
-
Familial, autosomal recessive
>25y
-
>21y
-
-
Pieter Klap
00073677
0000053424
no scapular winging (-HP:0003691), unable to stand on tiptoes (HP:0004302), unable to rise from floor (HP:0004302), Elevated serum creatine phosphokinase (HP:0003236), Reduced muscle fiber dysferlin (HP:0030115)
-
-
Familial, autosomal recessive
20y
-
19y
-
-
Pieter Klap
00073678
0000053425
no scapular winging (-HP:0003691), unable to stand on tiptoes (HP:0004302), unable to rise from floor (HP:0004302), Elevated serum creatine phosphokinase (HP:0003236), Absent muscle fiber dysferlin (HP:0030114),
-
-
Familial, autosomal recessive
30y
-
25y
-
-
Pieter Klap
00073679
0000053426
no scapular winging (-HP:0003691), unable to stand on tiptoes (HP:0004302), unable to get up from a chair (HP:0004302), Elevated serum creatine phosphokinase (HP:0003236),
-
-
Familial, autosomal recessive
27y
-
17y
-
-
Pieter Klap
00073680
0000053565
Enlarged calf muscle (HP:00014300), Difficulty running (HP:0009046), Elevated serum creatine phosphokinase (HP:0003236), no arrhythmia (-HP:0011675), normal echocardiogram (-HP:0003116), Subcapsular cataract (HP:0000523), Reduced muscle fiber dysferlin (HP:0030115)
-
-
Familial, autosomal recessive
-
-
14y
Enlarged calf muscle (HP:00014300)
-
Pieter Klap
00073749
0000053567
Pelvic girdle muscle weakness (HP:0003749), Inability to walk (HP:0002540), Lower limb amyotrophy (HP:0007210), no calf muscle hypertrophy (-HP:0008981), Reduced muscle fiber dysferlin (HP:0030115),
-
-
Familial, autosomal recessive
-
52y
24y
Pelvic girdle muscle weakness (HP:0003749)
-
Pieter Klap
00073753
0000053568
Difficulty climbing stairs (HP:0003551), Waddling gait (HP:0002515), Pelvic girdle muscle weakness (HP:0003749), Muscular dystrophy (HP:0003560), Absent muscle fiber dysferlin (HP:0030114)
-
-
Familial, autosomal recessive
-
38y
32y
Difficulty climbing stairs (HP:0003551)
-
Pieter Klap
00073754
0000053571
Difficulty running (HP:0009046), Waddling gait (HP:0002515), Pelvic girdle muscle weakness (HP:0003749), Muscular dystrophy (HP:0003560), Absent muscle fiber dysferlin (HP:0030114)
-
-
Familial, autosomal recessive
-
-
25y
Difficulty running (HP:0009046)
-
Pieter Klap
00073755
0000201928
muscular dystrophy (HP:0003560), elevated serum creatine phosphokinase (HP:0003236), lower limb muscle weakness (HP:0007340)
-
LGMD-2B
Familial, autosomal recessive
-
-
-
-
-
Johan den Dunnen
00073166
0000228658
-
-
-
Familial, autosomal recessive
-
-
-
-
-
Helen Latsoudis
00301527
0000245265
HP:0009053, HP:0003236, HP:0003560, HP:0030113
-
-
Isolated (sporadic)
-
-
-
-
-
Svetlana Gorokhova
00326797
0000245270
Absent muscle fiber dysferlin HP:0030114, Elevated serum creatine kinase HP:0003236
-
-
Familial, autosomal recessive
-
-
-
-
-
Svetlana Gorokhova
00326804
0000245391
Absent muscle fiber dysferlin HP:0030114
-
-
Familial, autosomal recessive
-
-
-
-
-
Svetlana Gorokhova
00326928
0000245453
Absent muscle fiber dysferlin HP:0030114
-
-
Familial, autosomal recessive
-
-
-
-
-
Svetlana Gorokhova
00326988
0000245488
Muscular dystrophy HP:0003560, Abnormal muscle fiber dysferlin HP:0030113
-
-
Unknown
-
-
-
-
-
Svetlana Gorokhova
00327046
0000245489
Limb-girdle muscular dystrophy HP:0006785, Absent muscle fiber dysferlin HP:0030114
-
-
Unknown
-
-
-
-
-
Svetlana Gorokhova
00327048
0000245686
Limb-girdle muscular dystrophy HP:0006785, Absent muscle fiber dysferlin HP:0030114
-
-
Isolated (sporadic)
-
-
-
-
-
Svetlana Gorokhova
00327391
0000257314
Muscle weakness HP:0001324
CMT1A
LGMD2B
Familial, autosomal recessive
-
-
-
-
-
Ibrahim Sahin
00361916
0000281247
27-y male with progressive distal weakness and wasting of both upper and lower limbs
LGMD
LGMD2B
Familial, autosomal recessive
-
-
15y
LGMD
-
Sherifa Ahmed Hamed
00387684
0000295176
LGMDR2 Miyoushi type
LGMDR2-Dysferlin related
LGMDR2-Dysferlin related
Familial, autosomal recessive
30y
31y
23y
dystal myopathy
IHC no DYSF
JA Bevilacqua
00402413
0000295178
-
LGMDR2-dysferlin related
LGMDR2-dysferlin related
Familial, autosomal recessive
40y
41y
25y
distal myopathy
DYSF absent by IHC in muscle biopsy
JA Bevilacqua
00402414
0000295179
LGMD R2 dysfelin related
LGMDR2-dysferlin related
LGMDR2-dysferlin related
Familial, autosomal recessive
18y
18y
12y
LGMW
DYSF absentby IHC in muscle biopsy
JA Bevilacqua
00402415
0000342093
Exercise-induced myalgia (HP:0003738) Increased muscle fatiguability (HP:0003750) Unilateral ptosis (HP:0007687) Elevated circulating creatine kinase concentration (HP:0003236)
-
-
Familial, autosomal recessive
-
52y
-
-
-
Camille Verebi
00453430
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