Gene/Protein
Disease
Symptom
Drug
Enzyme
Compound
Pivot Concepts:
Gene/Protein
Disease
Symptom
Drug
Enzyme
Compound
Target Concepts:
Gene/Protein
Disease
Symptom
Drug
Enzyme
Compound
Query: UMLS:C0038187 (
starvation
)
24,951
document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)
Coenzyme Q
10
(CoQ
10
) is a redox molecule critical for the proper function of energy metabolism and antioxidant defenses. Despite its essential role in cellular metabolism, the regulation of CoQ
10
biosynthesis in humans remains mostly unknown. Herein, we determined that
PPTC7
is a regulatory protein of CoQ
10
biosynthesis required for human cell survival. We demonstrated by in vitro approaches that
PPTC7
is a bona fide protein phosphatase that dephosphorylates the human COQ7. Expression modulation experiments determined that human
PPTC7
dictates cellular CoQ
10
content. Using two different approaches (
PPTC7
over-expression and caloric restriction), we demonstrated that
PPTC7
facilitates and improves the human cell adaptation to respiratory conditions. Moreover, we determined that the physiological role of
PPTC7
takes place in the adaptation to
starvation
and pro-oxidant conditions, facilitating the induction of mitochondrial metabolism while preventing the accumulation of ROS. Here we unveil the first post-translational mechanism regulating CoQ
10
biosynthesis in humans and propose targeting the induction of
PPTC7
activity/expression for the treatment of CoQ
10
-related mitochondrial diseases.
...
PMID:The mitochondrial phosphatase PPTC7 orchestrates mitochondrial metabolism regulating coenzyme Q
10
biosynthesis. 3026 71
