Global Variome shared LOVD
RAB7A (RAB7A, member RAS oncogene family)
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Phenotypes for disease #01740 (MC4DN (mitochondrial complex IV deficiency (MCDN4)))
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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Date
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Date
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17 entries on 1 page. Showing entries 1 - 17.
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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
0000017775
core clinical features: progressive neonatal hypertrophic, cardiomyopathy, lactic acidosis, muscular hypotonia: other features: pulomonary hypertension, persistent ductus arteriosus, dysmorphic features (small chin flat orbital bridges)
-
-
Unknown
-
-
-
-
-
Richard Rodenburg
00020015
0000083729
see paper; ..., mitochondrial complex IV deficiency (HP:0008347), neurodevelopmental regression (HP:0002376), medulla oblongata lesions (HP:0011441)
-
-
Familial, autosomal recessive
-
-
-
-
-
Herma Renkema
00095413
0000105551
-
-
-
Familial, autosomal recessive
-
-
-
-
-
Daniele Ghezzi
00132782
0000153418
Decreased activity of mitochondrial complex IV (HP:0008347) (fibroblasts)
-
-
Familial, autosomal recessive
-
-
00y00m45d?
-
-
Sze Chern Lim
00205226
0000153420
Decreased activity of mitochondrial complex IV (HP:0008347) (muscle, fibroblasts, liver)
-
-
Familial, autosomal recessive
-
-
-
-
-
Sze Chern Lim
00204778
0000154806
Decreased activity of mitochondrial complex IV (HP:0008347) (muscle, fibroblasts)
-
-
Familial, autosomal recessive
-
-
-
-
-
Sze Chern Lim
00207012
0000154808
Decreased activity of mitochondrial complex IV (HP:0008347) (fibroblasts)
-
-
Familial, autosomal recessive
-
-
-
-
-
Sze Chern Lim
00207013
0000154810
Decreased activity of mitochondrial complex IV (HP:0008347) (fibroblasts)
-
-
Familial, autosomal recessive
-
-
-
-
-
Sze Chern Lim
00207014
0000155192
Decreased activity of mitochondrial complex IV (HP:0008347) (fibroblasts)
-
-
Familial, autosomal recessive
-
-
-
-
-
Sze Chern Lim
00207418
0000155195
Decreased activity of mitochondrial complex IV (HP:0008347) (fibroblasts)
-
-
Familial, autosomal recessive
-
-
-
-
-
Sze Chern Lim
00207419
0000155197
Decreased activity of mitochondrial complex IV (HP:0008347) (fibroblasts)
-
-
Familial, autosomal recessive
-
-
00y06m
-
-
Sze Chern Lim
00207420
0000155199
Decreased activity of mitochondrial complex IV (HP:0008347) (fibroblasts, liver)
-
-
Familial, autosomal recessive
-
-
-
-
-
Sze Chern Lim
00207421
0000155201
Decreased activity of mitochondrial complex IV (HP:0008347) (fibroblasts, muscle and liver)
-
-
Familial, autosomal recessive
-
-
-
-
-
Sze Chern Lim
00207422
0000155202
Severe lactic acidosis(HP:0003128), Cranial ultrasound showed bilateral intraventricular cysts within the frontal horns and anterior portions of the lateral ventricles. Severely impaired liver function.
-
-
Familial, autosomal recessive
-
-
-
-
-
Sze Chern Lim
00207423
0000203566
age onset infancy; hypotonia, muscle weakness, facial weakness, high arched palate, no respiratory disorder; ECG normal; myopatholgy no ragged red fiber, uniformly decreased cytochrome C oxidase, lipid droplets
cytochrome C oxidase deficiency
-
Familial, autosomal recessive
09y
-
-
-
-
Johan den Dunnen
00265780
0000203567
hypotonia, muscle weakness, facial weakness, high arched palate, respiratory disorder; ECG cardiomyopathy; myopatholgy no ragged red fiber, uniformly decreased cytochrome C oxidase, lipid droplets
cytochrome C oxidase deficiency
-
Familial, autosomal recessive
-
-
00y00m00d
-
-
Johan den Dunnen
00265781
0000279730
see paper; ..., 8d-male died of hypertrophic cardiomyopathy left and right ventricles; 10d female died of hypertrophic restrictive cardiomyopathy
mitochondrial cardiomyopathy
MC4DN9
Familial, autosomal recessive
-
-
-
-
-
Johan den Dunnen
00385927
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