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CPS1 deficiency CPS1 deficiency is an autosomal-recessive disorder

Supplemental doses usually range from 0.5 to 2 g/day (133)

Nojiri, S

Unexpectedly high prevalence of the mild form of propionic acidemia in Japan: presence of a common mutation and possible clinical implications

In particular, these organelles are involved in the regulation of Ca 2+ homeostasis, different mechanisms of programmed cell death, autophagy, and reactive oxygen and nitrogen species (ROS and RNS) production

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