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Structural organization of mitochondrial human complex I: role of the ND4 and ND5 mitochondria-encoded subunits and interaction with prohibitin
- Source :
- The Biochemical journal. 383(Pt. 3)
- Publication Year :
- 2004
-
Abstract
- Mitochondria-encoded ND (NADH dehydrogenase) subunits, as components of the hydrophobic part of complex I, are essential for NADH:ubiquinone oxidoreductase activity. Mutations or lack of expression of these subunits have significant pathogenic consequences in humans. However, the way these events affect complex I assembly is poorly documented. To understand the effects of particular mutations in ND subunits on complex I assembly, we studied four human cell lines: ND4 non-expressing cells, ND5 non-expressing cells, and rho° cells that do not express any ND subunits, in comparison with normal complex I control cells. In control cells, all the seven analysed nuclear-encoded complex I subunits were found to be attached to the mitochondrial inner membrane, except for the 24 kDa subunit, which was nearly equally partitioned between the membranes and the matrix. Absence of a single ND subunit, or even all the seven ND subunits, caused no major changes in the nuclear-encoded complex I subunit content of mitochondria. However, in cells lacking ND4 or ND5, very low amounts of 24 kDa subunit were found associated with the membranes, whereas most of the other nuclear-encoded subunits remained attached. In contrast, membrane association of most of the nuclear subunits was significantly reduced in the absence of all seven ND proteins. Immunopurification detected several subcomplexes. One of these, containing the 23, 30 and 49 kDa subunits, also contained prohibitin. This is the first description of prohibitin interaction with complex I subunits and suggests that this protein might play a role in the assembly or degradation of mitochondrial complex I.
- Subjects :
- Genotype
Protein subunit
Bone Neoplasms
Mitochondrion
Biochemistry
Mass Spectrometry
Oxidative Phosphorylation
Mitochondrial Proteins
Oxidoreductase
Cell Line, Tumor
Prohibitins
Humans
Prohibitin
Inner mitochondrial membrane
Molecular Biology
chemistry.chemical_classification
Osteosarcoma
Electron Transport Complex I
biology
NADH dehydrogenase
NADH Dehydrogenase
Cell Biology
DNA, Neoplasm
Cell biology
Mitochondria
Repressor Proteins
Protein Subunits
chemistry
G12/G13 alpha subunits
Chaperone (protein)
biology.protein
Chromatography, Liquid
Research Article
Subjects
Details
- ISSN :
- 14708728
- Volume :
- 383
- Issue :
- Pt. 3
- Database :
- OpenAIRE
- Journal :
- The Biochemical journal
- Accession number :
- edsair.doi.dedup.....cb1d24a07ae58d8a12acac7f4dea265c