Cytochrome-c Oxidase Deficiency [deficit cytochrom-c-oxidázy]

topical
27
Terms

cytochrom-c-oxidasa - nedostatek
deficit COX
deficit komplexu IV dýchacího řetězce
nedostatek cytochrom-c-oxidázy
nedostatek cytochromální oxidázy

 

Complex IV Deficiency
Cox Deficiency
Cytochrome C Oxidase Deficiency
Cytochrome Oxidase Deficiency
Deficiency, Cytochrome-c Oxidase
Mitochondrial Complex IV Deficiency

Persistent link   https://www.medvik.cz/link/D030401
Definition

A disease that results from a congenital defect in ELECTRON TRANSPORT COMPLEX IV. Defects in ELECTRON TRANSPORT COMPLEX IV can be caused by mutations in the SURF1, SCO2, COX10, or SCO1 genes. ELECTRON TRANSPORT COMPLEX IV deficiency caused by mutation in SURF1 manifests itself as LEIGH DISEASE; that caused by mutation in SCO2 as fatal infantile cardioencephalomyopathy; that caused by mutation in COX10 as tubulopathy and leukodystrophy; and that caused by mutation in SCO1 as early-onset hepatic failure and neurologic disorder. (from Online Mendelian Inheritance in Man, http://www.ncbi.nlm.nih.gov/Omim, MIM#220110, May 17, 2001)

DUI
D030401 MeSH Browser
CUI
M0335492
Previous indexing
Cytochrome-c Oxidase/deficiency (1976-2001); Leigh Disease (1997-2001)
History note
2002
Public note
2002

C Diseases
C16.320.565.176 Amyloidosis, Familial 16
C16.320.565.595 Lysosomal Storage Diseases 72
C16.320.565.663 Peroxisomal Disorders 20
C16.320.565.753 Progeria 27
C18.452 Metabolic Diseases 1 196
C18.452.660 Mitochondrial Diseases 159
C18.452.660.300 Friedreich Ataxia 54
C18.452.660.520 Leigh Disease 26
C18.452.660.560 Mitochondrial Myopathies 40