Loss of mitochondrial peptidase Clpp leads to infertility, hearing loss plus growth retardation via accumulation of CLPX, mtDNA and inflammatory factors

Perrault Syndrome Is Caused by Recessive Mutations in CLPP, Encoding a Mitochondrial ATP-Dependent Chambered Protease

(2013) Emma M. Jenkinson et al.   AMERICAN JOURNAL OF HUMAN GENETICS

Human CLPP reverts the longevity phenotype of a fungal ClpP deletion strain

(2013) Fabian Fischer et al.   Nature Communications

A Mutation in PNPT1 , Encoding Mitochondrial-RNA-Import Protein PNPase, Causes Hereditary Hearing Loss

(2012) Simon von Ameln et al.   AMERICAN JOURNAL OF HUMAN GENETICS

Exercise early in life in rats born small does not normalize reductions in skeletal muscle PGC-1α in adulthood

(2012) Rhianna C. Laker et al.   AMERICAN JOURNAL OF PHYSIOLOGY-ENDOCRINOLOGY AND METABOLISM

Mitochondrial processing peptidase regulates PINK1 processing, import and Parkin recruitment

(2012) Andrew W Greene et al.   EMBO REPORTS

Maintenance of mitochondrial genome distribution by mitochondrial AAA+ protein ClpX

(2012) Katsumi Kasashima et al.   EXPERIMENTAL CELL RESEARCH

Opa3, a novel regulator of mitochondrial function, controls thermogenesis and abdominal fat mass in a mouse model for Costeff syndrome

(2012) T. Wells et al.   HUMAN MOLECULAR GENETICS

Proteomic and Metabolomic Analyses of Mitochondrial Complex I-deficient Mouse Model Generated by Spontaneous B2 Short Interspersed Nuclear Element (SINE) Insertion into NADH Dehydrogenase (Ubiquinone) Fe-S Protein 4 (Ndufs4) Gene

(2012) Dillon W. Leong et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Mitochondrial quality control: an integrated network of pathways

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

Mitochondrial peptidase IMMP2L mutation causes early onset of age-associated disorders and impairs adult stem cell self-renewal

(2011) Sunil K. George et al.   AGING CELL

AAA+ Proteases: ATP-Fueled Machines of Protein Destruction

(2011) Robert T. Sauer et al.   Annual Review of Biochemistry

The Mitochondrial Connection in Auditory Neuropathy

(2011) Anthony T. Cacace et al.   AUDIOLOGY AND NEURO-OTOLOGY

ClpXP, an ATP-powered unfolding and protein-degradation machine

(2011) Tania A. Baker et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH

Mitochondrial superoxide anion (O2·-) inducible "mev-1" animal models for aging research

(2011) Takamasa Ishii et al.   BMB Reports

SPICE: Exploration and analysis of post-cytometric complex multivariate datasets

(2011) Mario Roederer et al.   CYTOMETRY PART A

Mitochondria in innate immunity

(2011) Damien Arnoult et al.   EMBO REPORTS

Immune and non-immune functions of the immunoproteasome

(2011) Arkhjamil Angeles   Frontiers in Bioscience-Landmark

New Insights into Staphylococcus aureus Stress Tolerance and Virulence Regulation from an Analysis of the Role of the ClpP Protease in the Strains Newman, COL, and SA564

(2011) Dorte Frees et al.   JOURNAL OF PROTEOME RESEARCH

Mutations in mitochondrial histidyl tRNA synthetase HARS2 cause ovarian dysgenesis and sensorineural hearing loss of Perrault syndrome

(2011) S. B. Pierce et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Mitochondrial protein quality control during biogenesis and aging

(2011) Brooke M. Baker et al.   TRENDS IN BIOCHEMICAL SCIENCES

Whole-Exome Sequencing Identifies Homozygous AFG3L2 Mutations in a Spastic Ataxia-Neuropathy Syndrome Linked to Mitochondrial m-AAA Proteases

(2011) Tyler Mark Pierson et al.   PLoS Genetics

Mutations in the DBP-Deficiency Protein HSD17B4 Cause Ovarian Dysgenesis, Hearing Loss, and Ataxia of Perrault Syndrome

(2010) Sarah B. Pierce et al.   AMERICAN JOURNAL OF HUMAN GENETICS

Is the mitochondrial cloud the selection machinery for preferentially transmitting wild-type mtDNA between generations? Rewinding Müller’s ratchet efficiently

(2010) Rong Rong Zhou et al.   CURRENT GENETICS

The GRACILE mutation introduced into Bcs1l causes postnatal complex III deficiency: A viable mouse model for mitochondrial hepatopathy

(2010) Per Levéen et al.   HEPATOLOGY

Missense mutations in the AFG3L2 proteolytic domain account for ∼1.5% of European autosomal dominant cerebellar ataxias

(2010) Claudia Cagnoli et al.   HUMAN MUTATION

Neurologic Dysfunction and Male Infertility inDrosophila porinMutants

(2010) Brett H. Graham et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Mitochondrial membrane potential regulates PINK1 import and proteolytic destabilization by PARL

(2010) Seok Min Jin et al.   JOURNAL OF CELL BIOLOGY

Mitochondrial dysfunction and reduced prostaglandin synthesis in skeletal muscle of Group VIB Ca2+-independent phospholipase A2γ-deficient mice

(2010) Emiko Yoda et al.   JOURNAL OF LIPID RESEARCH

The role of mitochondria in the mammalian antiviral defense system

(2010) Iain Scott   MITOCHONDRION

The Matrix Peptide Exporter HAF-1 Signals a Mitochondrial UPR by Activating the Transcription Factor ZC376.7 in C. elegans

(2010) Cole M. Haynes et al.   MOLECULAR CELL

Circulating mitochondrial DAMPs cause inflammatory responses to injury

(2010) Qin Zhang et al.   NATURE

Mutations in the mitochondrial protease gene AFG3L2 cause dominant hereditary ataxia SCA28

(2010) Daniela Di Bella et al.   NATURE GENETICS

Autophagy proteins regulate innate immune responses by inhibiting the release of mitochondrial DNA mediated by the NALP3 inflammasome

(2010) Kiichi Nakahira et al.   NATURE IMMUNOLOGY

Mitochondrial DNA depletion syndromes – Many genes, common mechanisms

(2010) Anu Suomalainen et al.   NEUROMUSCULAR DISORDERS

The Mitochondrion — A Trojan Horse That Kicks Off Inflammation?

(2010) Angelo A. Manfredi et al.   NEW ENGLAND JOURNAL OF MEDICINE

Primary Follicular Dystrophy With Scarring Dermatitis in C57BL/6 Mouse Substrains Resembles Central Centrifugal Cicatricial Alopecia in Humans

(2010) J. P. Sundberg et al.   VETERINARY PATHOLOGY

Emerging roles of mitochondrial proteases in neurodegeneration

(2009) Paola Martinelli et al.   BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS

Mitochondrial glutathione peroxidase 4 disruption causes male infertility

(2009) Manuela Schneider et al.   FASEB JOURNAL

Frataxin deficiency causes upregulation of mitochondrial Lon and ClpP proteases and severe loss of mitochondrial Fe-S proteins

(2009) Blanche Guillon et al.   FEBS Journal

Short Form Glutathione Peroxidase 4 Is the Essential Isoform Required for Survival and Somatic Mitochondrial Functions

(2009) Hanyu Liang et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Depletion of Selenoprotein GPx4 in Spermatocytes Causes Male Infertility in Mice

(2009) Hirotaka Imai et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

SCID Dogs: Similar Transplant Potential but Distinct Intra-Uterine Growth Defects and Premature Replicative Senescence Compared with SCID Mice

(2009) K. Meek et al.   JOURNAL OF IMMUNOLOGY

Disruption of Supv3L1 damages the skin and causes sarcopenia, loss of fat, and death

(2009) Erin Paul et al.   MAMMALIAN GENOME

DNA extraction procedures meaningfully influence qPCR-based mtDNA copy number determination

(2009) Wen Guo et al.   MITOCHONDRION

Parkinson Phenotype in Aged PINK1-Deficient Mice Is Accompanied by Progressive Mitochondrial Dysfunction in Absence of Neurodegeneration

(2009) Suzana Gispert et al.   PLoS One

Mitochondrial protein homeostasis: the cooperative roles of chaperones and proteases

(2009) Wolfgang Voos   RESEARCH IN MICROBIOLOGY

Proteolysis of σS (RpoS) and the general stress response in Escherichia coli

(2009) Regine Hengge   RESEARCH IN MICROBIOLOGY

A Mitochondrial Protein Compendium Elucidates Complex I Disease Biology

(2008) David J. Pagliarini et al.   CELL

Quality control of mitochondria: protection against neurodegeneration and ageing

(2008) Takashi Tatsuta et al.   EMBO JOURNAL

Large-scale gene trapping in C57BL/6N mouse embryonic stem cells

(2008) G. M. Hansen et al.   GENOME RESEARCH

Progressive loss of mitochondrial DNA in thymidine kinase 2-deficient mice

(2008) X. Zhou et al.   HUMAN MOLECULAR GENETICS

The Hsp60-(p.V98I) Mutation Associated with Hereditary Spastic Paraplegia SPG13 Compromises Chaperonin Function Bothin Vitroandin Vivo

(2008) Peter Bross et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Mitochondrial dysfunction: An early event in Alzheimer pathology accumulates with age in AD transgenic mice

(2008) S. Hauptmann et al.   NEUROBIOLOGY OF AGING

Decreased expression of the mitochondrial matrix proteases Lon and ClpP in cells from a patient with hereditary spastic paraplegia (SPG13)

(2008) J. Hansen et al.   NEUROSCIENCE

A Mutation in the Inner Mitochondrial Membrane Peptidase 2-Like Gene (Immp2l) Affects Mitochondrial Function and Impairs Fertility in Mice1

(2007) Baisong Lu et al.   BIOLOGY OF REPRODUCTION

Mitochondrial stress signaling: a pathway unfolds

(2007) Sarah A. Broadley et al.   TRENDS IN CELL BIOLOGY