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2 OMIM references -
3 associated genes
No signs/symptoms info
COMMON GENES: 1
2 OMIM references -
14 associated genes
No signs/symptoms info
Familial infantile bilateral striatal necrosis
Maternally-inherited Leigh syndrome

ADAR MT-ATP6
MT-ATP6 MT-CO1
NUP62 MT-CO2
MT-CO3
MT-ND1
MT-ND2
MT-ND3
MT-ND4
MT-ND5
MT-ND6
MT-TK
MT-TL1
MT-TV
MT-TW


COMMON
GENES
MT-ATP6



Citations in the biomedical literature:


Familial infantile bilateral striatal necrosis
ADAR MT-ATP6 NUP62
Maternally-inherited Leigh syndrome
MT-CO1 MT-CO2 MT-CO3 MT-ND1 MT-ND2
MT-ND3 MT-ND4 MT-ND5 MT-ND6 MT-TK MT-TL1
MT-TV MT-TW



Familial infantile bilateral striatal necrosis
Maternally-inherited Leigh syndrome

Synonym(s):
- Familial IBSN
- Familial infantile striatonigral degeneration
- Familial infantile striatonigral necrosis

Synonym(s):
- MILS
- Maternally-inherited Leigh disease
- Maternally-inherited infantile subacute necrotizing encephalopathy

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

Classification (ICD10):
- Diseases of the nervous system -
Classification (ICD10):
- Diseases of the nervous system -

Epidemiological data:
Class of prevalence: <1 / 1 000 000
Average age onset: neonatal/infancy
Average age of death: -
Type of inheritance: autosomal dominant
Epidemiological data:
Class of prevalence: -
Average age onset: neonatal/infancy
Average age of death: -
Type of inheritance: mitochondrial inheritance

External references:
2 OMIM references -
No MeSH references
External references:
2 OMIM references -
No MeSH references

No signs/symptoms info available.