PARL deficiency in mouse causes Complex III defects, coenzyme Q depletion, and Leigh-like syndrome

Multi-omic Mitoprotease Profiling Defines a Role for Oct1p in Coenzyme Q Production

(2017) Mike T. Veling et al.   MOLECULAR CELL

PARL mediates Smac proteolytic maturation in mitochondria to promote apoptosis

(2017) Shotaro Saita et al.   NATURE CELL BIOLOGY

Biochemistry of Mitochondrial Coenzyme Q Biosynthesis

(2017) Jonathan A. Stefely et al.   TRENDS IN BIOCHEMICAL SCIENCES

Coenzyme Q deficiency causes impairment of the sulfide oxidation pathway

(2016) Marcello Ziosi et al.   EMBO Molecular Medicine

PARL: The mitochondrial rhomboid protease

(2016) Marco Spinazzi et al.   SEMINARS IN CELL & DEVELOPMENTAL BIOLOGY

The CoQH2/CoQ Ratio Serves as a Sensor of Respiratory Chain Efficiency

(2016) Adela Guarás et al.   Cell Reports

Leigh syndrome: One disorder, more than 75 monogenic causes

(2015) Nicole J. Lake et al.   ANNALS OF NEUROLOGY

Intramembrane protease PARL defines a negative regulator of PINK1- and PARK2/Parkin-dependent mitophagy

(2015) Cathrin Meissner et al.   Autophagy

Mutations in TTC19: expanding the molecular, clinical and biochemical phenotype

(2015) Johannes Koch et al.   Orphanet Journal of Rare Diseases

Mitochondrial Ca2+ in neurodegenerative disorders

(2015) Rosella Abeti et al.   PHARMACOLOGICAL RESEARCH

Thei-AAA protease YME1L and OMA1 cleave OPA1 to balance mitochondrial fusion and fission

(2014) Ruchika Anand et al.   JOURNAL OF CELL BIOLOGY

Purkinje neuron Ca2+ influx reduction rescues ataxia in SCA28 model

(2014) Francesca Maltecca et al.   JOURNAL OF CLINICAL INVESTIGATION

Neural-Specific Deletion of Htra2 Causes Cerebellar Neurodegeneration and Defective Processing of Mitochondrial OPA1

(2014) Victoria L. Patterson et al.   PLoS One

Genetic deficiency of the mitochondrial protein PGAM5 causes a Parkinson’s-like movement disorder

(2014) Wei Lu et al.   Nature Communications

PINK1 is degraded through the N-end rule pathway

(2013) Koji Yamano et al.   Autophagy

The Mitochondrial Phosphatase PGAM5 Functions at the Convergence Point of Multiple Necrotic Death Pathways

(2012) Zhigao Wang et al.   CELL

Mitochondrial processing peptidase regulates PINK1 processing, import and Parkin recruitment

(2012) Andrew W Greene et al.   EMBO REPORTS

Molecular control over thymic involution: From cytokines and microRNAto aging and adipose tissue

(2012) James Dooley et al.   EUROPEAN JOURNAL OF IMMUNOLOGY

Rhomboid Protease PARL Mediates the Mitochondrial Membrane Potential Loss-induced Cleavage of PGAM5

(2012) Shiori Sekine et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Haploinsufficiency ofCOQ4causes coenzyme Q10deficiency

(2012) Leonardo Salviati et al.   JOURNAL OF MEDICAL GENETICS

Functional alteration of PARL contributes to mitochondrial dysregulation in Parkinson's disease

(2011) Guang Shi et al.   HUMAN MOLECULAR GENETICS

The mitochondrial intramembrane protease PARL cleaves human Pink1 to regulate Pink1 trafficking

(2011) Cathrin Meissner et al.   JOURNAL OF NEUROCHEMISTRY

Mutations in TTC19 cause mitochondrial complex III deficiency and neurological impairment in humans and flies

(2011) Daniele Ghezzi et al.   NATURE GENETICS

Regulation of Skeletal Muscle Oxidative Capacity and Insulin Signaling by the Mitochondrial Rhomboid Protease PARL

(2010) Anthony E. Civitarese et al.   Cell Metabolism

PINK1 cleavage at position A103 by the mitochondrial protease PARL

(2010) Emma Deas et al.   HUMAN MOLECULAR GENETICS

Mitochondrial membrane potential regulates PINK1 import and proteolytic destabilization by PARL

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

Complex I deficiency due to loss of Ndufs4 in the brain results in progressive encephalopathy resembling Leigh syndrome

(2010) A. Quintana et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Hax1 lacks BH modules and is peripherally associated to heavy membranes: implications for Omi/HtrA2 and PARL activity in the regulation of mitochondrial stress and apoptosis

(2009) D V Jeyaraju et al.   CELL DEATH AND DIFFERENTIATION

Thymic involution, a co-morbidity factor in amyotrophic lateral sclerosis

(2009) Akop Seksenyan et al.   JOURNAL OF CELLULAR AND MOLECULAR MEDICINE

Mice with Mitochondrial Complex I Deficiency Develop a Fatal Encephalomyopathy

(2008) Shane E. Kruse et al.   Cell Metabolism

Hax1-mediated processing of HtrA2 by Parl allows survival of lymphocytes and neurons

(2008) Jyh-Rong Chao et al.   NATURE