Mitochondrial matrix proteostasis is linked to hereditary paraganglioma: LON-mediated turnover of the human flavinylation factor SDH5 is regulated by its interaction with SDHA

Solution NMR Structure of Yeast Succinate Dehydrogenase Flavinylation Factor Sdh5 Reveals a Putative Sdh1 Binding Site

(2012) Alexander Eletsky et al.   BIOCHEMISTRY

The role of complex II in disease

(2012) Attje S. Hoekstra et al.   BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS

Substrate recognition and processing by a Walker B mutant of the human mitochondrial AAA+ protein CLPX

(2012) Bradley R. Lowth et al.   JOURNAL OF STRUCTURAL BIOLOGY

Phosphorylation of Human TFAM in Mitochondria Impairs DNA Binding and Promotes Degradation by the AAA+ Lon Protease

(2012) Bin Lu et al.   MOLECULAR CELL

Toxin-Antitoxin Systems in Bacteria and Archaea

(2011) Yoshihiro Yamaguchi et al.   Annual Review of Genetics

Processing of mitochondrial presequences

(2011) Dirk Mossmann et al.   Biochimica et Biophysica Acta-Gene Regulatory Mechanisms

Lon Peptidase 1 (LONP1)-dependent Breakdown of Mitochondrial 5-Aminolevulinic Acid Synthase Protein by Heme in Human Liver Cells

(2011) Qing Tian et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Diverse functions of mitochondrial AAA+ proteins: protein activation, disaggregation, and degradationThis paper is one of a selection of papers published in this special issue entitled 8th International Conference on AAA Proteins and has undergone the Journal's usual peer review process.

(2010) Kaye N. Truscott et al.   Biochemistry and Cell Biology

SDHA is a tumor suppressor gene causing paraganglioma

(2010) Nelly Burnichon et al.   HUMAN MOLECULAR GENETICS

Succinate dehydrogenase – Assembly, regulation and role in human disease

(2010) Jared Rutter et al.   MITOCHONDRION

Global Analysis of the Mitochondrial N-Proteome Identifies a Processing Peptidase Critical for Protein Stability

(2009) F.-Nora Vögtle et al.   CELL

Importing Mitochondrial Proteins: Machineries and Mechanisms

(2009) Agnieszka Chacinska et al.   CELL

Modification of PATase by L/F-transferase generates a ClpS-dependent N-end rule substrate in Escherichia coli

(2009) Robert L Ninnis et al.   EMBO JOURNAL

SDH5, a Gene Required for Flavination of Succinate Dehydrogenase, Is Mutated in Paraganglioma

(2009) H.-X. Hao et al.   SCIENCE