Global Variome shared LOVD
NDUFA1 (NADH dehydrogenase (ubiquinone) 1 alpha sub...)
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Phenotypes for disease #00611 (MC1DN (mitochondrial complex I deficiency, nuclear (MC1DN)), OMIM:252010)
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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29 entries on 1 page. Showing entries 1 - 29.
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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
0000050847
-
-
-
Familial, autosomal recessive
-
-
-
-
-
NeuroMeGen
00063250
0000060636
m complex I deficiency (OMIM:252010)
-
-
Familial, autosomal recessive
-
-
-
-
-
Daniel Trujillano
00081067
0000085575
see paper; ...
-
-
Familial, autosomal recessive
-
-
-
-
-
Johan den Dunnen
00107834
0000172888
normal skeletal muscle; normal skin; MRI-brain normal; no epilepsy, EEG-mild background activity depression; no developmental delay/intellectual disability; no abnormalities eyes; normal growth; temporary normocytic anemia; lactic acidosis (serum lactate 2-20 mmol/l); hypertrophic cardiomyopathy
-
-
Isolated (sporadic)
03y06m
-
>00y00m01d
-
-
Sander Pajusalu
00180279
0000172889
normal skeletal muscle; normal skin; MRI-brain mild cerebral atrophy; no epilepsy; developmental delay/intellectual disability; optic atrophy; short stature; persistent leukopenia and microcytic anemia; lactic acidosis (CSF lactate 3.1 mmol/l (normal <2.1 mmol/l)); hypertrophic cardiomyopathy; joint contractures, non-progressive mild hearing impairment
-
-
Isolated (sporadic)
15y
-
>00y00m01d
03y-04y
-
Sander Pajusalu
00180280
0000172971
histiocytoid cardiomyopathy
MC1DN
-
Isolated (sporadic)
-
-
-
-
-
Johan den Dunnen
00229764
0000172972
axial hypotonia; linear skin defect; lacrimalduct atresia; failure to thrive; no anemia; no lactic acidosis; histiocytoid cardiomyopathy; died at age 6m, autopsy revealed thyroid abnormalities (large and multiple sites of oncocytic metaplasia)
MC1DN
-
Isolated (sporadic)
6m
-
1d
-
-
Johan den Dunnen
00229765
0000172973
mild to moderate bulbar palsy; normal skin; MRI-brain focal histiocytoid change in choroid plexus brain; no epilepsy; intermittent squint; no anemia; histiocytoid cardiomyopathy; focal histiocytoid change in the thyroid and lungs
MC1DN
-
Isolated (sporadic)
13m
-
<0d
-
-
Johan den Dunnen
00229766
0000172974
normal skeletal muscle; normal skin; MRI-brain normal; no epilepsy; no developmental delay/intellectual disability; normal growth; congenital sideroblastic anemia; lactic acidosis (4.2-8.2 mmol/l); fetal tachycardia and hypertrophy ventricular walls with moderate trabeculature right ventricle; dysmorphic features: hypertelorism, saddle nose, low set ears; hepatosplenomegaly and hydrocele
MC1DN
-
Isolated (sporadic)
9y
-
<0d
-
-
Johan den Dunnen
00229767
0000172975
normal skin; no epilepsy; no developmental delay/intellectual disability; no abnormalities eyes; short stature; congenital sideroblastic anemia; no; no cardiac abnormality
MC1DN
-
Isolated (sporadic)
2y
-
>0d
-
-
Johan den Dunnen
00229768
0000172976
myopathy; normal skin; no epilepsy; no developmental delay/intellectual disability; no abnormalities eyes; normal growth; congenital sideroblastic anemia; lactic acidosis; no cardiac abnormality
MC1DN
-
Familial
23y
-
-
-
-
Johan den Dunnen
00229769
0000172977
normal skin; no epilepsy; developmental delay/intellectual disability; congenital optic atrophy; short stature; congenital sideroblastic anemia; no lactic acidosis; no cardiac abnormality
MC1DN
-
Familial, X-linked recessive
20y
-
<0d
-
-
Johan den Dunnen
00229770
0000172978
normal skin; no epilepsy; developmental delay/intellectual disability; congenital optic atrophy; short stature; congenital sideroblastic anemia; no lactic acidosis; no cardiac abnormality
MC1DN
-
Familial, X-linked recessive
16y
-
<0d
-
-
Johan den Dunnen
00229771
0000172979
myopathy; normal skin; epilepsy; no developmental delay/intellectual disability; no abnormalities eyes; normal growth; congenital sideroblastic anemia; no lactic acidosis; no cardiac abnormality; single kidney, pulmonary stenosis, congenial inguinal hernia
MC1DN
-
Unknown
8y
-
<0d
-
-
Johan den Dunnen
00229772
0000172980
histiocytoid cardiomyopathy
MC1DN
-
Isolated (sporadic)
-
-
-
-
-
Johan den Dunnen
00229773
0000172981
redundant skin; intrauterine growth restriction; heart failure; died at age 55h
MC1DN
-
Isolated (sporadic)
3d
-
<0d
-
-
Johan den Dunnen
00229774
0000172982
linear skin defect; MRI-brain corpus callosum agenesis, dilated lateral ventricles; epilepsy; severe global developmental delay; myopia, nystagmus, strabismus; failure to thrive; no anemia; no lactic acidosis; dilated cardiomyopathy
MC1DN
-
Familial, X-linked recessive
4y6m
-
-
-
-
Johan den Dunnen
00229775
0000172983
MRI-brain corpus callosum dysgenesis, connected lateral ventricles, small cerebellum and cavum septum pellucidum; intrauterine growth restriction; thickened myocardium and pericardial effusion
MC1DN
-
Familial, X-linked recessive
<0d
-
<0d
-
-
Johan den Dunnen
00229776
0000257296
Neuroregression following viral illness, hypotonia, normal deep tendon reflexes
-
-
Familial, autosomal recessive
-
-
-
-
-
Anju Shukla
00361901
0000268949
see paper; ...
mitochondrial encephalomyopathy
MC1DN21
Familial, autosomal recessive
-
-
-
-
-
Johan den Dunnen
00373724
0000268950
see paper; ...
Leigh syndrome
MC1DN19
Familial, autosomal recessive
-
-
-
-
-
Johan den Dunnen
00373726
0000268951
see paper; ...
encephalomyopathy
MC1DN19
Familial, autosomal recessive
-
-
-
-
-
Johan den Dunnen
00373727
0000268954
see paper; ...
mitochondrial complex I deficiency
MC1DN21
Familial, autosomal recessive
-
-
-
-
-
Johan den Dunnen
00373730
0000268955
see paper; ...
mitochondrial complex I deficiency
MC1DN21
Familial, autosomal recessive
-
-
-
-
-
Johan den Dunnen
00373731
0000268956
see paper; ...
mitochondrial complex I deficiency
MC1DN21
Familial, autosomal recessive
-
-
-
-
-
Johan den Dunnen
00373732
0000268957
see paper; ...
mitochondrial complex I deficiency
MC1DN21
Familial, autosomal recessive
-
-
-
-
-
Johan den Dunnen
00373733
0000268958
see paper; ...
mitochondrial complex I deficiency
MC1DN21
Familial, autosomal recessive
-
-
-
-
-
Johan den Dunnen
00373734
0000268959
see paper; ...
mitochondrial complex I deficiency
MC1DN21
Familial, autosomal recessive
-
-
-
-
-
Johan den Dunnen
00373735
0000268960
see paper; ...
complex leukoencephalopathy with thalamic involvement
MC1DN21
Familial, autosomal recessive
-
-
-
-
-
Johan den Dunnen
00373736
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