Complex I and the cAMP Cascade in Human Physiopathology
作者： S. Papa , S. Scacco , A.M. Sardanelli , V. Petruzzella , R. Vergari
关键词: Protein kinase A 、 PRKAR1A 、 Biology 、 Mitogen-activated protein kinase kinase 、 HSPA2 、 Biochemistry 、 HSPA4 、 Cyclin-dependent kinase complex 、 Respiratory chain 、 Protein subunit 、 Cell biology 、 Biophysics 、 Molecular biology
摘要: 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.
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