Mutation or knock-down of 17β-hydroxysteroid dehydrogenase type 10 cause loss of MRPP1 and impaired processing of mitochondrial heavy strand transcripts

ELAC2 Mutations Cause a Mitochondrial RNA Processing Defect Associated with Hypertrophic Cardiomyopathy

(2013) Tobias B. Haack et al.   AMERICAN JOURNAL OF HUMAN GENETICS

GRSF1 Regulates RNA Processing in Mitochondrial RNA Granules

(2013) Alexis A. Jourdain et al.   Cell Metabolism

The Mitochondrial RNA-Binding Protein GRSF1 Localizes to RNA Granules and Is Required for Posttranscriptional Mitochondrial Gene Expression

(2013) Hana Antonicka et al.   Cell Metabolism

Human RNase H1 Is Associated with Protein P32 and Is Involved in Mitochondrial Pre-rRNA Processing

(2013) Hongjiang Wu et al.   PLoS One

Moonlighting is mainstream: Paradigm adjustment required

(2012) Shelley D. Copley   BIOESSAYS

A subcomplex of human mitochondrial RNase P is a bifunctional methyltransferase—extensive moonlighting in mitochondrial tRNA biogenesis

(2012) Elisa Vilardo et al.   NUCLEIC ACIDS RESEARCH

Mitochondrial quality control: an integrated network of pathways

(2012) Fabian Fischer et al.   TRENDS IN BIOCHEMICAL SCIENCES

Human Mitochondrial tRNAs: Biogenesis, Function, Structural Aspects, and Diseases

(2011) Tsutomu Suzuki et al.   Annual Review of Genetics

RNA processing in human mitochondria

(2011) Maria I.G. Lopez Sanchez et al.   CELL CYCLE

Maternally Inherited Essential Hypertension Is Associated With the Novel 4263A>G Mutation in the Mitochondrial tRNA Ile Gene in a Large Han Chinese Family

(2011) Shiwen Wang et al.   CIRCULATION RESEARCH

HSD10 disease: clinical consequences of mutations in the HSD17B10 gene

(2011) Johannes Zschocke   JOURNAL OF INHERITED METABOLIC DISEASE

Structure of MyTH4-FERM Domains in Myosin VIIa Tail Bound to Cargo

(2011) L. Wu et al.   SCIENCE

Mechanism of neurodegeneration of neurons with mitochondrial DNA mutations

(2010) A. Y. Abramov et al.   BRAIN

A non-enzymatic function of 17β-hydroxysteroid dehydrogenase type 10 is required for mitochondrial integrity and cell survival

(2010) Katharina Rauschenberger et al.   EMBO Molecular Medicine

Structures of the Human GTPase MMAA and Vitamin B12-dependent Methylmalonyl-CoA Mutase and Insight into Their Complex Formation

(2010) D. Sean Froese et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

A single Arabidopsis organellar protein has RNase P activity

(2010) Anthony Gobert et al.   NATURE STRUCTURAL & MOLECULAR BIOLOGY

SEDLIN Forms Homodimers: Characterisation of SEDLIN Mutations and Their Interactions with Transcription Factors MBP1, PITX1 and SF1

(2010) Jeshmi Jeyabalan et al.   PLoS One

tRNA recognition, processing, and disease: Hypotheses around an unorthodox type of RNase P in human mitochondria

(2009) Johann Holzmann et al.   MITOCHONDRION

RNase P without RNA: Identification and Functional Reconstitution of the Human Mitochondrial tRNA Processing Enzyme

(2008) Johann Holzmann et al.   CELL

Study of patients and carriers with 2-methyl-3-hydroxybutyryl-CoA dehydrogenase (MHBD) deficiency: Difficulties in the diagnosis

(2008) Judit García-Villoria et al.   CLINICAL BIOCHEMISTRY