Pptc7 is an essential phosphatase for promoting mammalian mitochondrial metabolism and biogenesis.

ABSTRACT: Mitochondrial proteins are replete with phosphorylation, yet its functional relevance remains largely unclear. The presence of multiple resident mitochondrial phosphatases, however, suggests that protein dephosphorylation may be broadly important for calibrating mitochondrial activities. To explore this, we deleted the poorly characterized matrix phosphatase Pptc7 from mice using CRISPR-Cas9 technology. Strikingly, Pptc7^-/- mice exhibit hypoketotic hypoglycemia, elevated acylcarnitines and serum lactate, and die soon after birth. Pptc7^-/- tissues have markedly diminished mitochondrial size and protein content despite normal transcript levels, and aberrantly elevated phosphorylation on select mitochondrial proteins. Among these, we identify the protein translocase comp

SUBMITTER: Niemi NM 

PROVIDER: S-EPMC6642090 | biostudies-literature | 2019 Jul

REPOSITORIES: biostudies-literature