FAT IMPROVES MITOCHONDRIAL PERFORMANCE IN A RESPIRATORY CHAIN DISORDER(COMPLEX I DEFICIENCY). † 848
نویسندگان
چکیده
منابع مشابه
Recurrent de novo mitochondrial DNA mutations in respiratory chain deficiency.
Starting from a cohort of 50 NADH-oxidoreductase (complex I) deficient patients, we carried out the systematic sequence analysis of all mitochondrially encoded complex I subunits (ND1 to ND6 and ND4L) in affected tissues. This approach yielded the unexpectedly high rate of 20% mutation identification in our series. Recurrent heteroplasmic mutations included two hitherto unreported (T10158C and ...
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...
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ژورنال
عنوان ژورنال: Pediatric Research
سال: 1996
ISSN: 0031-3998,1530-0447
DOI: 10.1203/00006450-199604001-00870