5EKZ

Crystal structure of mouse Taco1

Summary for 5EKZ

• Entry DOI: 10.2210/pdb5ekz/pdb
• Descriptor: Translational activator of cytochrome c oxidase 1 (2 entities in total)
• Functional Keywords: oxidoreductase, mitochondria, rna binding
• Biological source: Mus musculus (House Mouse)
• Cellular location: Mitochondrion : Q8K0Z7
• Total number of polymer chains: 1
• Total formula weight: 32364.19

Authors

Spahr, H.,Richman, T.R.,Larsson, N.G.,Rackham, O.,Filipovska, A. (deposition date: 2015-11-04, release date: 2016-06-29, Last modification date: 2024-05-08)

Primary citation

Richman, T.R.,Spahr, H.,Ermer, J.A.,Davies, S.M.,Viola, H.M.,Bates, K.A.,Papadimitriou, J.,Hool, L.C.,Rodger, J.,Larsson, N.G.,Rackham, O.,Filipovska, A.
Loss of the RNA-binding protein TACO1 causes late-onset mitochondrial dysfunction in mice.
Nat Commun, 7:11884-11884, 2016

PubMed Abstract: The recognition and translation of mammalian mitochondrial mRNAs are poorly understood. To gain further insights into these processes in vivo, we characterized mice with a missense mutation that causes loss of the translational activator of cytochrome oxidase subunit I (TACO1). We report that TACO1 is not required for embryonic survival, although the mutant mice have substantially reduced COXI protein, causing an isolated complex IV deficiency. We show that TACO1 specifically binds the mt-Co1 mRNA and is required for translation of COXI through its association with the mitochondrial ribosome. We determined the atomic structure of TACO1, revealing three domains in the shape of a hook with a tunnel between domains 1 and 3. Mutations in the positively charged domain 1 reduce RNA binding by TACO1. The Taco1 mutant mice develop a late-onset visual impairment, motor dysfunction and cardiac hypertrophy and thus provide a useful model for future treatment trials for mitochondrial disease.

PubMed: 27319982
DOI: 10.1038/ncomms11884

Experimental method

X-RAY DIFFRACTION (2 Å)