A cAMP-dependent protein kinase (PKA) is localized in mammalian mitochondria with the catalytic site at the matrix side of the membrane where it phosphorylates a number of proteins. One of these is the 18 kDa(IP) subunit of the mammalian complex I of the respiratory chain, encoded by the nuclear NDUFS4 gene. Mitochondria have a Ca2+-inhibited phosphatase, which dephosphorylates the 18 kDa phosphoprotein of complex I. In fibroblast and myoblast cultures cAMP-dependent phosphorylation of the 18 kDa protein is associated with stimulation of complex I and overall respiratory activity with NAD-linked substrates. Mutations in the human NDUFS4 gene have been found, which in the homozygous state are associated with deficiency of complex I and fatal neurological syndrome.
Complex I and the cAMP Cascade in Human Physiopathology
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S. Papa, S. Scacco, A.M. Sardanelli, V. Petruzzella, R. Vergari, A. Signorile, Z. Technikova-Dobrova; Complex I and the cAMP Cascade in Human Physiopathology. Biosci Rep 1 February 2002; 22 (1): 3–16. doi: https://doi.org/10.1023/A:1016004921277
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