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PROTEIN INTERACTIONS: 2
1 OMIM reference -
25 associated genes
1 sign/symptom
Mitochondrial nonsyndromic sensorineural deafness with susceptibility to aminoglycoside exposure
Isolated NADH-CoQ reductase deficiency

MT-CO1 ACAD9
MT-ND4 FOXRED1
MT-RNR1 MT-ND2
MT-TS1 MTFMT
TRMU NDUFA1
NDUFA11
NDUFA2
NDUFA9
NDUFAF1
NDUFAF2
NDUFAF3
NDUFAF4
NDUFAF5
NDUFB3
NDUFB9
NDUFS1
NDUFS2
NDUFS3
NDUFS4
NDUFS6
NDUFS7
NDUFS8
NDUFV1
NDUFV2
NUBPL


INTERACTOME
ASSOCIATIONS

(click on a score value to see the evidence)
MT-CO1
MT-ND4
(0.58)
(0.58)
NDUFA9
NDUFA2



Citations in the biomedical literature:


Mitochondrial nonsyndromic sensorineural deafness with susceptibility to aminoglycoside exposure
MT-CO1 MT-ND4 MT-RNR1 MT-TS1 TRMU
Isolated NADH-CoQ reductase deficiency
ACAD9 FOXRED1 MT-ND2 MTFMT NDUFA1 NDUFA11
NDUFA2 NDUFA9 NDUFAF1 NDUFAF2 NDUFAF3 NDUFAF4
NDUFAF5 NDUFB3 NDUFB9 NDUFS1 NDUFS2 NDUFS3
NDUFS4 NDUFS6 NDUFS7 NDUFS8 NDUFV1 NDUFV2
NUBPL



Mitochondrial nonsyndromic sensorineural deafness with susceptibility to aminoglycoside exposure
Isolated NADH-CoQ reductase deficiency

Synonym(s):
- Mitochondrial isolated neurosensory deafness with susceptibility to aminoglycoside exposure
- Mitochondrial isolated neurosensory hearing loss with susceptibility to aminoglycoside exposure
- Mitochondrial isolated sensorineural deafness with susceptibility to aminoglycoside exposure
- Mitochondrial isolated sensorineural hearing loss with susceptibility to aminoglycoside exposure
- Mitochondrial nonsyndromic neurosensory deafness with susceptibility to aminoglycoside exposure
- Mitochondrial nonsyndromic neurosensory hearing loss with susceptibility to aminoglycoside exposure
- Mitochondrial nonsyndromic sensorineural hearing loss with susceptibility to aminoglycoside exposure

Synonym(s):
- Isolated NADH-coenzyme Q reductase deficiency
- Isolated NADH-ubiquinone reductase deficiency
- Isolated mitochondrial respiratory chain complex I deficiency

Classification (Orphanet):
- Inborn errors of metabolism
- Rare genetic disease
- Rare otorhinolaryngologic disease
Classification (Orphanet):
- Inborn errors of metabolism
- Rare genetic disease
- Rare neurologic disease

Classification (ICD10):
(no data available)
Classification (ICD10):
- Diseases of the nervous system -

Epidemiological data:
Class of prevalence: unknown
Average age onset: variable
Average age of death: -
Type of inheritance: mitochondrial inheritance
Epidemiological data:
(no data available)

External references:
1 OMIM reference -
No MeSH references
External references:
1 OMIM reference -
No MeSH references

Isolated NADH-CoQ reductase deficiency

Very frequent
- Organic acid metabolism anomalies



Mitochondrial nonsyndromic sensorineural deafness with susceptibility to aminoglycoside exposure

(no data available)