Global Variome shared LOVD
ACTA2-AS1 (ACTA2 antisense RNA 1)
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Phenotypes for disease #02145 (Danon;GSD2B (Danon disease (glycogen storage disease, type IIb (GSD-2B))), OMIM:300257)
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
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88 entries on 1 page. Showing entries 1 - 88.
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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
0000043057
-
-
-
Unknown
-
-
-
-
-
Irene Vieitez
00056438
0000067591
see paper; ...
-
-
Familial, X-linked dominant
-
-
-
-
-
Johan den Dunnen
00088085
0000067592
-
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00088086
0000067593
-
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00088087
0000067594
-
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00088088
0000067595
-
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00088089
0000067596
-
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00088090
0000067597
-
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00088091
0000067598
-
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00088092
0000067599
see paper; ...
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00088093
0000067600
see paper;...
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00088094
0000067601
see paper; ...
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00088095
0000067602
see paper; progressive exertional dyspnea, fatigue, abdominal discomfort, motor/mental development normal, general health good, physical function allowed sports involvement, throbbing precordium, systolic murmur left hemithorax, hepatomegaly, ...; mother died at 46y (dilated cardiomyopathy)
-
-
Unknown
19y
-
-
-
-
Johan den Dunnen
00088096
0000067603
see paper; 14y- admitted to hospital for recurrent episodes of presyncope and severe chest pain on exertion, ...
-
-
Familial, X-linked
-
-
10y
chest pain, palpitations on exertion
-
Johan den Dunnen
00088097
0000067604
see paper; 13y-unexplained elevation of serum creatinine kinase, proximal weakness, Gower sign, no exertional dyspnea, no cardiac murmur, no hepatomegaly, no muscle atrophy, no hypotonia, normal muscle tone, ...
-
-
Isolated (sporadic)
-
-
12y
incidentally detected high level liver enzymes (ALT, AST)/total bilirubin
-
Johan den Dunnen
00088098
0000067605
see paper; skeletal myopathy, mental retardation, ophthalmic manifestations, massive hypertrophic obstructive cardiomyopathy necessitating heart transplantation, ...
-
-
Isolated (sporadic)
14y
-
<09y
-
-
Johan den Dunnen
00088099
0000067606
see paper; 21y-admitted to hospital with exertional dyspnea, ...
-
-
Unknown
-
-
14y
Danon's disease
-
Johan den Dunnen
00088100
0000067607
see paper; ...
-
-
Familial, X-linked dominant
-
-
-
-
-
Johan den Dunnen
00088101
0000067608
see paper; ...
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00088102
0000067609
see paper;, ..., skeletal muscle weakness, high creatine kinase level, ...
-
-
Isolated (sporadic)
-
-
12y
gastrointestinal
-
Johan den Dunnen
00088103
0000067610
-
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00088104
0000067611
-
-
-
Familial, X-linked
-
-
-
-
-
Johan den Dunnen
00088105
0000067612
-
-
-
Familial, X-linked
-
-
-
-
-
Johan den Dunnen
00088106
0000067613
-
-
-
Familial, X-linked
-
-
-
-
-
Johan den Dunnen
00088107
0000067614
-
-
-
Familial, X-linked
-
-
-
-
-
Johan den Dunnen
00088108
0000067615
-
-
-
Familial, X-linked
-
-
-
-
-
Johan den Dunnen
00088109
0000067616
-
-
-
Familial, X-linked
-
-
-
-
-
Johan den Dunnen
00088110
0000067617
-
-
-
Familial, X-linked
-
-
-
-
-
Johan den Dunnen
00088111
0000067618
-
-
-
Familial, X-linked
-
-
-
-
-
Johan den Dunnen
00088112
0000067619
see paper; ...
-
-
Familial, X-linked dominant
-
-
-
-
-
Johan den Dunnen
00088113
0000067624
see paper; ...
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00088151
0000067625
see paper; ..., easy fatigability, anorexia, abdominal pain, progressive diffuse muscle hypotrophy , Wolff-Parkinson-White (WPW) syndrome, heart failure, several syncopal episodes, elevated creatine kinase
-
-
Familial, X-linked dominant
-
-
-
-
-
Johan den Dunnen
00088152
0000067626
see paper; ..., 9y-scleral jaundice, abnormal hepatic laboratory tests, liver biopsy chronic hepatitis with normal serology, 18y-electrocardiography revealed WPW syndrome; 22y-exertion dyspnea, difficulty climbing stairs, several syncopal episodes; ...
-
-
Familial, X-linked dominant
-
-
09y
-
-
Johan den Dunnen
00088153
0000067627
see paper; ..., 12y-subclinical jaundice, viral hepatitis excluded (serology; 18y-easy fatigability after mild effort; 19y-ECG revealed bradycardia, WPW syndrome; 22y-very low aerobic resistance (tread-mill test); 27y-chest X-ray mild cardiomegaly; women have no raised CK
-
-
Familial, X-linked dominant
-
-
-
-
-
Johan den Dunnen
00088154
0000067628
see paper; ..., infancy mild hypotonia, global developmental delay, elevated serum CK levels, ...
-
-
Familial, X-linked
-
-
-
-
-
Johan den Dunnen
00088155
0000067629
see paper; ..., presented with HCM as teenager, progressed to dilated cardiomyopathy/heart failure, skeletal myopathy, Wolff-Parkinson-White syndrome; teenage carrier sister has HCM, no skeletal myopathy/WPW, ...
-
-
Familial, X-linked dominant
-
-
-
-
-
Johan den Dunnen
00088156
0000067630
see paper; ..., presented with HCM, Wolff-Parkinson-White syndrome, skeletal myopathy as teenager; carrier mother developed DCM during 40s, ...
-
-
Familial, X-linked dominant
-
-
-
-
-
Johan den Dunnen
00088157
0000067631
see paper; ...,
-
-
Familial, X-linked dominant
-
-
-
-
-
Johan den Dunnen
00088158
0000067632
see paper; ...
-
-
Familial, X-linked dominant
-
-
-
-
-
Johan den Dunnen
00088159
0000067633
see paper; ...
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00088160
0000067634
see paper; ...
-
-
Familial, X-linked dominant
-
-
-
-
-
Johan den Dunnen
00088161
0000067635
see paper; ..., severe skeletal muscular weakness, respiratory failure, history of two OHTs (1st severe HCM, 2nd allograft rejection, myopathy, ...
-
-
Unknown
-
-
01y
heart murmur
-
Johan den Dunnen
00088162
0000067636
see paper; ...,
-
-
Familial, X-linked dominant
-
-
-
-
-
Johan den Dunnen
00088163
0000067637
12y-HCM; 14y-acute episode heart failure, ECG concentric hypertrophy dilated left ventricle with moderate systolic dysfunction, neurological/EMG normal, increased levels serum creatine kinase (638 U/l); 15y orthotopic heart transplantation
-
-
Isolated (sporadic)
-
12y
-
-
-
Johan den Dunnen
00088164
0000067638
see paper; ...
-
-
Familial, X-linked dominant
-
-
-
-
-
Johan den Dunnen
00088165
0000067639
severe hypertrophic cardiomyopathy, Wolff-Parkinson-White syndrome, proximal muscle weakness, chronic painless diarrhea, muscle biopsy autophagic vacuolar myopathy, IHC LAMP2 absence
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00088166
0000067640
limb-girdle muscle weakness, mild left ventricular diastolic dysfunction, sub-clinical neuropathy, muscle biopsy autophagic vacuolar myopathy, IHC LAMP2 smear
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00088167
0000068360
glycogen storage disease, type IIb
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00088956
0000068361
variable clinical presentations, including cardiomyopathy, skeletal muscle pathology, hepatopathy, abnormal mitochondria/LAMP2 immunohistochemistry
-
-
Familial, autosomal dominant
-
-
-
-
-
Johan den Dunnen
00088957
0000068362
Danon disease, cone-rod dystrophy (CRD), visual acuity deteriorated progressively, color vision severely disturbed; ERG reduced photopic/scotopic responses, ...
-
-
Familial, autosomal dominant
-
-
-
-
-
Johan den Dunnen
00088958
0000068363
-
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00088959
0000068364
-
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00088960
0000068365
-
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00088961
0000068366
-
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00088962
0000068367
-
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00088963
0000068368
-
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00088964
0000068369
see paper; cardiomyopathy, mental retardation, myopathy, vacuolar myopathy without acid a-glucosidase deficiency, diffuse chorio-capillary ocular atrophy, successful heart transplantation, ...
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00088965
0000068370
-
-
-
Unknown
-
-
-
-
-
Johan den Dunnen
00088966
0000068373
see paper; ...
-
-
Isolated (sporadic)
-
-
-
-
-
Johan den Dunnen
00088969
0000068374
see paper; ...
-
-
Familial, X-linked
-
-
-
-
-
Johan den Dunnen
00088970
0000068386
Danon disease
-
-
Unknown
-
-
-
-
-
Peikuan Cong
00088982
0000068387
Danon disease
-
-
Unknown
-
-
-
-
-
Peikuan Cong
00088983
0000068388
Danon disease, hyperCKemia, hypertrophic cardiomyopathy, no muscle weakness, slight mental impairment; muscle biopsy autophagic vacuoles with sarcolemmal features and glycogen storage
-
-
Familial
-
-
-
-
-
Johan den Dunnen
00088984
0000068389
Danon disease
-
-
Unknown
-
-
-
-
-
Peikuan Cong
00088985
0000068390
Danon disease, see paper; progressive hypertrophic cardiomyopathy, death at 16y (before planned heart transplantation); mother developed severe dilated cardiomyopathy, 46y died of complications
-
-
Familial
-
-
02y06m
mild left ventricular hypertrophy, mild myopathy
-
Johan den Dunnen
00088986
0000068391
Danon disease
-
-
Unknown
-
-
-
-
-
Peikuan Cong
00088987
0000068392
Danon disease
-
-
Unknown
-
-
-
-
-
Peikuan Cong
00088988
0000068393
Danon disease
-
-
Familial
-
-
-
-
-
Peikuan Cong
00088989
0000068396
Danon disease
-
-
Unknown
-
-
-
-
-
Peikuan Cong
00088992
0000068397
Danon disease; cardiomyopathy, hypertrophic (HCM)
-
-
Familial
-
-
-
-
-
Peikuan Cong
00088993
0000068402
Danon disease
-
-
Familial
-
-
-
-
-
Peikuan Cong
00088998
0000068403
Danon disease
-
-
Unknown
-
-
-
-
-
Peikuan Cong
00088999
0000068404
Danon disease
-
-
Unknown
-
-
-
-
-
Peikuan Cong
00089000
0000068406
Danon disease
-
-
Unknown
-
-
-
-
-
Peikuan Cong
00089002
0000068409
Danon disease
-
-
Familial
-
-
-
-
-
Peikuan Cong
00089005
0000068411
Danon disease
-
-
Unknown
-
-
-
-
-
Peikuan Cong
00089007
0000068412
Danon disease
-
-
Familial
-
-
-
-
-
Peikuan Cong
00089008
0000068413
Danon disease
-
-
Familial
-
-
-
-
-
Peikuan Cong
00089009
0000068414
Danon disease
-
-
Unknown
-
-
-
-
-
Peikuan Cong
00089010
0000068415
Danon disease
-
-
Unknown
-
-
-
-
-
Peikuan Cong
00089011
0000068423
see paper; hypertrophic cardiomyopathy, ...
-
-
Familial, X-linked
-
-
-
-
-
Johan den Dunnen
00089021
0000159677
-
Danon disease
Danon disease
Familial, X-linked
-
-
-
-
-
Maria Iascone
00211195
0000334617
see paper; ..., visual impairment secondary to retinitis pigmentosa; uneventful spontaneous pregnancy/delivery; <14m-normal development; 18m-heart murmur, ECG asymptomatic WPW
Danon disease
Danon disease
Familial, X-linked dominant
03y
-
-
-
-
Johan den Dunnen
00445381
0000334619
see paper; ..., full-time job as storekeeper; 13y-heart palpitations, ECG Wolf–Parkinson–White pattern; 18y-increased echogenicity, borderline increase antero-septal wall thickness; 25y-progressive visual impairment with increased severity dark hours, ERG revealed rods/cones dystrophy; no psychiatric/gastrointestinal/pulmonary complaints; 27y-pacemaker; 42y-atrial fibrillation
Danon disease
Danon disease
Unknown
42y
-
-
-
-
Johan den Dunnen
00445382
0000343456
The ECG revealed premature atrial beats, premature ventricular beats, and ST-T changes. Echocardiography showed marked thickening of the septum and posterior wall of the left ventricle,reduced wall motion and diastolic dysfunction.
Danon disease
Danon disease
Familial, X-linked dominant
14y
14y
14y
precordial discomfort
-
Chunli Wang
00454846
0000343508
Cardiac enzymes were abnormal, ECG showed incomplete right bundle branch block, high voltage in the left ventricle, ST-T changes in extensive leads, and cardiac ultrasound showed a slight thickening of the left ventricle and septum, and uncoordinated ventricular wall motion.
myocarditis
Danon disease
Familial, X-linked
>07y
>07y
>07y
>7y
-
Chunli Wang
00454847
0000343510
shortness of breath
Danon disease
Danon disease
Familial, X-linked
00y04m
00y04m
00y04m
-
-
Chunli Wang
00454848
0000343511
heart murmur
Danon disease
Danon disease
Familial, X-linked
>10y
>10y
>10y
>10y
-
Chunli Wang
00454849
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