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4da1

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Template:STRUCTURE 4da1

Contents

Crystal structure of branched-chain alpha-ketoacid dehydrogenase phosphatase with Mg (II) ions at the active site

Template:ABSTRACT PUBMED 22291014

Disease

[PPM1K_HUMAN] Intermediate maple syrup urine disease.

Function

[PPM1K_HUMAN] Regulates the mitochondrial permeability transition pore and is essential for cellular survival and development.[1]

About this Structure

4da1 is a 1 chain structure with sequence from Homo sapiens. Full crystallographic information is available from OCA.

Reference

  • Wynn RM, Li J, Brautigam CA, Chuang JL, Chuang DT. Structural and biochemical characterization of human mitochondrial branched-chain alpha-ketoacid dehydrogenase phosphatase. J Biol Chem. 2012 Mar 16;287(12):9178-92. Epub 2012 Jan 30. PMID:22291014 doi:10.1074/jbc.M111.314963
  1. Lu G, Ren S, Korge P, Choi J, Dong Y, Weiss J, Koehler C, Chen JN, Wang Y. A novel mitochondrial matrix serine/threonine protein phosphatase regulates the mitochondria permeability transition pore and is essential for cellular survival and development. Genes Dev. 2007 Apr 1;21(7):784-96. Epub 2007 Mar 20. PMID:17374715 doi:http://dx.doi.org/10.1101/gad.1499107

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