Global Variome shared LOVD
NBEA (neurobeachin)
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Phenotypes for disease #04285 (LGMDR1;LGMD2A (dystrophy, muscular, limb-girdle, recessive, type 1), OMIM:253600)
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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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12 entries on 1 page. Showing entries 1 - 12.
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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
0000060492
Muscular dystrophy, limb-girdle, type 2A (OMIM:253600)
-
-
Familial, autosomal recessive
-
-
-
-
-
Daniel Trujillano
00080923
0000128132
39y-shoulder pain, exercise intolerance; developed progressive weakness affecting proximal upper and lower limbs, scapular winging; 56y-wheelchair bound; elevated CK 4059 IU/L; EMG myopathic with fibrillation potentials; muscle biopsy dystrophic, large number lobulated fibres
LGMD
LGMD2A
Familial, autosomal recessive
68y
-
39y
-
-
Martine Tetreault
00155749
0000222945
-
-
LGMD2A
Unknown
-
-
-
-
WB CAPN3 reduced
Svetlana Gorokhova
00289314
0000222946
-
-
LGMD2A
Unknown
-
-
-
-
WB CAPN3 normal
Svetlana Gorokhova
00289315
0000222947
-
-
LGMD2A
Unknown
-
-
-
-
WB CAPN3 reduced
Svetlana Gorokhova
00289316
0000222949
-
-
LGMD2A
Unknown
-
-
-
-
WB CAPN3 absent
Svetlana Gorokhova
00289318
0000228653
-
-
-
Familial, autosomal recessive
-
-
-
-
-
Helen Latsoudis
00301522
0000257316
Muscle weakness HP:0001324 Difficulty walking HP:0002355
-
-
Familial, autosomal recessive
-
-
-
-
-
Ibrahim Sahin
00361918
0000257376
Muscle weakness HP:0001324
-
-
Familial, autosomal recessive
-
-
-
-
-
Ibrahim Sahin
00361984
0000295317
LGMDR1-Calpain-3 related
limb girdle muscle dystrophy
LGMDR1-Calpain 3-Related
Familial, autosomal recessive
18y
26y
08y
lower limbs weakness
-
JA Bevilacqua
00402534
0000295320
LGMDR1-Calpain 3 Related
limb girdle muscle dystrophy
LGMDR1-calpain-3 related
Familial, autosomal recessive
25y
16y
10y
lower limb weakness
-
JA Bevilacqua
00402557
0000305279
Proximal muscle weakness in lower limbs HP:0008994 Proximal muscle weakness in upper limbs HP:0008997 Achilles tendon contracture HP:0001771
muscular dystrophy
LGMDR1
Familial, autosomal recessive
18y
18y
16y
-
-
Alberto Hidalgo-Bravo
00413298
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