English Journal

A novel heterozygous OPA3 mutation located in the mitochondrial target sequence results in altered steady-state levels and fragmented mitochondrial network.

Grau T, Burbulla LF, Engl G, Delettre C, Delprat B, Oexle K, Leo-Kottler B, Roscioli T, Krüger R, Rapaport D, Wissinger B, Schimpf-Linzenbold S.SourceMolecular Genetics Laboratory, Institute for Ophthalmic Research, Centre for Ophthalmology, University Clinics Tuebingen, Tuebingen, Germany.
Journal of medical genetics.J Med Genet.2013 Oct 17. doi: 10.1136/jmedgenet-2013-101774. [Epub ahead of print]
BACKGROUND: Mutations in OPA3 have been reported in patients with autosomal dominant optic atrophy plus cataract and Costeff syndrome. Here, we report the results of a comprehensive study on OPA3 mutations, including the mutation spectrum and its prevalence in a large cohort of OPA1-negative autosom
PMID 24136862

Organellar oligopeptidase (OOP) provides a complementary pathway for targeting peptide degradation in mitochondria and chloroplasts.

Kmiec B, Teixeira PF, Berntsson RP, Murcha MW, Branca RM, Radomiljac JD, Regberg J, Svensson LM, Bakali A, Langel U, Lehtiö J, Whelan J, Stenmark P, Glaser E.SourceDepartments of Biochemistry and Biophysics and Neurochemistry, Arrhenius Laboratories for Natural Sciences, Stockholm University, SE-106 91 Stockholm, Sweden.
Proceedings of the National Academy of Sciences of the United States of America.Proc Natl Acad Sci U S A.2013 Oct 1;110(40):E3761-9. doi: 10.1073/pnas.1307637110. Epub 2013 Sep 16.
Both mitochondria and chloroplasts contain distinct proteolytic systems for precursor protein processing catalyzed by the mitochondrial and stromal processing peptidases and for the degradation of targeting peptides catalyzed by presequence protease. Here, we have identified and characterized a comp
PMID 24043784

The plant mitochondrial protein import apparatus - The differences make it interesting.

Murcha MW, Wang Y, Narsai R, Whelan J.SourceARC Centre of Excellence in Plant Energy Biology, Bayliss Building M316, The University of Western Australia, 35 Stirling Hwy, Crawley, WA 6009, Australia. Electronic address: monika.murcha@uwa.edu.au.
Biochimica et biophysica acta.Biochim Biophys Acta.2013 Sep 28. pii: S0304-4165(13)00411-X. doi: 10.1016/j.bbagen.2013.09.026. [Epub ahead of print]
BACKGROUND: Mitochondria play essential roles in the life and death of almost all eukaryotic cells, ranging from single-celled to multi-cellular organisms that display tissue and developmental differentiation. As mitochondria only arose once in evolution, much can be learned from studying single cel
PMID 24080405

Japanese Journal

Isolation of mitochondrial and plastid ftsZ genes and analysis of the organelle targeting sequence in the diatom Chaetoceros neogracile (Diatoms, Bacillariophyceae)