Mitochondrial respiratory chain super-complex I–III in physiology and pathology
نویسندگان
چکیده
منابع مشابه
Mitochondrial myopathies: deficiencies localized to complex I and complex III of the mitochondrial respiratory chain.
Deficiencies of the mitochondrial respiratory chain or the phosphorylation system are becoming increasingly recognized causes of myopathy and multisystem disease in man. In biochemical terms, the ones most clearly defined have been associated with impaired activities of NADH : ubiquinone reductase (Morgan-Hughes el al., 1979, 1984; Land et al., 1981; Moreadith etal., 1984), ubiquino1:cytochrome...
متن کاملMitochondrial respiratory-chain diseases.
s since January 1975, a full-text search capacity, and a personal archive for saving articles and search results of interest. All articles can be printed in a format that is virtually identical to that of the typeset pages. Beginning six months after publication the full text of all original articles and special articles is available free to nonsubscribers who have completed a brief registratio...
متن کاملThe mitochondrial respiratory chain.
In the present chapter, the structures and mechanisms of the major components of mammalian mitochondrial respiratory chains are reviewed. Particular emphasis is placed on the four protein complexes and their cofactors that catalyse the electron transfer pathway between oxidation of NADH and succinate and the reduction of oxygen to water. Current ideas are reviewed of how these electron transfer...
متن کاملNonylphenol ethoxylate plastic additives inhibit mitochondrial respiratory chain complex I.
The first and major entry point of electrons into the mitochondrial respiratory chain (MRC) 1 occurs through NADH-coenzyme Q reduc-tase (complex I). Decreased complex I activity is associated with a wide range of conditions, including inherited mitochondrial diseases and neu-rodegenerative conditions such as Parkinson disease (1–2). More than 60 natural and commercial compounds are reported to ...
متن کاملThe Assembly Pathway of Mitochondrial Respiratory Chain Complex I.
Mitochondrial complex I is the largest integral membrane enzyme of the respiratory chain and consists of 44 different subunits encoded in the mitochondrial and nuclear genome. Its biosynthesis is a highly complicated and multifaceted process involving at least 14 additional assembly factors. How these subunits assemble into a functional complex I and where the assembly factors come into play is...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
ژورنال
عنوان ژورنال: Biochimica et Biophysica Acta (BBA) - Bioenergetics
سال: 2010
ISSN: 0005-2728
DOI: 10.1016/j.bbabio.2010.01.025