- Adipose transplant for inborn errors of branched chain amino acid metabolism in mice
Heather A Zimmerman
Department of Comparative Medicine, Penn State University College of Medicine, 500 University Dr, Hershey, PA 17033, USA
Mol Genet Metab 109:345-53. 2013
..for adipose tissue transplant to lower circulating BCAAs in two models of defective BCAA metabolism, BCATm and PP2Cm [branched chain keto acid dehydrogenase complex (BCKDC) phosphatase] knockout (KO) mice...
- PPM1l encodes an inositol requiring-protein 1 (IRE1) specific phosphatase that regulates the functional outcome of the ER stress response
Departments of Anesthesiology, Division of Molecular Medicine, David Geffen School of Medicine, UCLA, Los Angeles, CA, USA
Mol Metab 2:405-16. 2013
..Therefore, PPM1l mediated IRE1 regulation and downstream ER stress signaling is a plausible molecular basis for its role in metabolic regulation and disorder. ..
- Impaired adiponectin signaling contributes to disturbed catabolism of branched-chain amino acids in diabetic mice
Department of Cardiology, Xijing Hospital, Fourth Military Medical University, Xi an, China
Diabetes 64:49-59. 2015
..Here, we demonstrate that mitochondrial phosphatase 2C (PP2Cm), a newly identified BCKD phosphatase that increases BCKD activity, was significantly downregulated in ob/ob and ..
- IRE1 phosphatase PP2Ce regulates adaptive ER stress response in the postpartum mammary gland
Department of Anesthesiology, Division of Molecular Medicine, David Geffen School of Medicine, University of California Los Angeles, Los Angeles, CA, United States of America
PLoS ONE 9:e111606. 2014
..This study provides the first in vivo evidence that PP2Ce is an essential regulator of normal lactation, possibly involving IRE1 signaling and ER stress regulation in mammary epithelium. ..
- Tissue-specific and nutrient regulation of the branched-chain α-keto acid dehydrogenase phosphatase, protein phosphatase 2Cm (PP2Cm)
Department of Pathophysiology, Key Laboratory of Cell Differentiation and Apoptosis of Chinese Ministry of Education, Shanghai Jiao Tong University School of Medicine, Shanghai 200025, China
J Biol Chem 287:23397-406. 2012
..Our previous study has identified a mitochondria-targeted protein phosphatase, PP2Cm, as the BCKD phosphatase and thus serves as a key regulator for BCAA catabolism...
- A novel mitochondrial matrix serine/threonine protein phosphatase regulates the mitochondria permeability transition pore and is essential for cellular survival and development
Department of Anesthesiology, University of California at Los Angeles, Los Angeles, California 90095, USA
Genes Dev 21:784-96. 2007
..Here, we report the finding of a mitochondrial matrix-targeted protein phosphatase 2C family member (PP2Cm) that regulates mitochondrial membrane permeability transition pore (MPTP) opening and is essential for cell ..
- Protein phosphatase 2Cm is a critical regulator of branched-chain amino acid catabolism in mice and cultured cells
Division of Molecular Medicine, Department of Anesthesiology, David Geffen School of Medicine, UCLA, Los Angeles, California 90095, USA
J Clin Invest 119:1678-87. 2009
..Using unbiased, proteomic approaches, we have found that a mitochondrial-targeted phosphatase, PP2Cm, specifically binds the BCKD complex and induces dephosphorylation of Ser293 in the presence of BCKD substrates...