Neurodegeneration as a consequence of failed mitochondrial maintenance

Regulating mitochondrial outer membrane proteins by ubiquitination and proteasomal degradation

(2011) Mariusz Karbowski et al.   CURRENT OPINION IN CELL BIOLOGY

MiD49 and MiD51, new components of the mitochondrial fission machinery

(2011) Catherine S Palmer et al.   EMBO REPORTS

Bioenergetic dysfunction in Huntington's disease human cybrids

(2011) I. Luisa Ferreira et al.   EXPERIMENTAL NEUROLOGY

Genetic mouse models for Parkinson's disease display severe pathology in glial cell mitochondria

(2011) S. Schmidt et al.   HUMAN MOLECULAR GENETICS

Abnormal mitochondrial dynamics, mitochondrial loss and mutant huntingtin oligomers in Huntington's disease: implications for selective neuronal damage

(2011) U. Shirendeb et al.   HUMAN MOLECULAR GENETICS

Molecular mechanisms and physiologic functions of mitochondrial dynamics

(2011) H. Otera et al.   JOURNAL OF BIOCHEMISTRY

Mitofusin 2 Regulates STIM1 Migration from the Ca2+Store to the Plasma Membrane in Cells with Depolarized Mitochondria

(2011) Karthika Singaravelu et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Direct Membrane Association Drives Mitochondrial Fission by the Parkinson Disease-associated Protein α-Synuclein

(2011) Ken Nakamura et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

Regulation of mitochondrial morphology by APC/CCdh1-mediated control of Drp1 stability

(2011) Sarah R. Horn et al.   MOLECULAR BIOLOGY OF THE CELL

Milestones in ataxia

(2011) Thomas Klockgether et al.   MOVEMENT DISORDERS

Mutant huntingtin binds the mitochondrial fission GTPase dynamin-related protein-1 and increases its enzymatic activity

(2011) Wenjun Song et al.   NATURE MEDICINE

Mitochondria: The Next (Neurode)Generation

(2011) Eric A. Schon et al.   NEURON

Mitochondrial Oxidative Phosphorylation Compensation May Preserve Vision in Patients with OPA1-Linked Autosomal Dominant Optic Atrophy

(2011) Nicole J. Van Bergen et al.   PLoS One

Misfolded SOD1 Associated with Motor Neuron Mitochondria Alters Mitochondrial Shape and Distribution Prior to Clinical Onset

(2011) Christine Vande Velde et al.   PLoS One

Super-resolution microscopy reveals that mammalian mitochondrial nucleoids have a uniform size and frequently contain a single copy of mtDNA

(2011) C. Kukat et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Impairing the Mitochondrial Fission and Fusion Balance: A New Mechanism of Neurodegeneration

(2010) Andrew B. Knott et al.   Annals of the New York Academy of Sciences

The Role of Mitochondria in Reactive Oxygen Species Metabolism and Signaling

(2010) Anatoly A. Starkov   Annals of the New York Academy of Sciences

Genotype–phenotype correlations in Leber hereditary optic neuropathy

(2010) Katarzyna Tońska et al.   BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS

Ubiquitin–Proteasome System and mitochondria — Reciprocity

(2010) Nurit Livnat-Levanon et al.   Biochimica et Biophysica Acta-Gene Regulatory Mechanisms

DNA repair in organelles: Pathways, organization, regulation, relevance in disease and aging

(2010) Pierre Boesch et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH

Paraquat and Parkinson's disease

(2010) C Berry et al.   CELL DEATH AND DIFFERENTIATION

IBRDC2, an IBR-type E3 ubiquitin ligase, is a regulatory factor for Bax and apoptosis activation

(2010) Giovanni Benard et al.   EMBO JOURNAL

Mitochondrial fission and cristae disruption increase the response of cell models of Huntington's disease to apoptotic stimuli

(2010) Veronica Costa et al.   EMBO Molecular Medicine

Proteasome and p97 mediate mitophagy and degradation of mitofusins induced by Parkin

(2010) Atsushi Tanaka et al.   JOURNAL OF CELL BIOLOGY

Mff is an essential factor for mitochondrial recruitment of Drp1 during mitochondrial fission in mammalian cells

(2010) Hidenori Otera et al.   JOURNAL OF CELL BIOLOGY

Iron-dependent functions of mitochondria—relation to neurodegeneration

(2010) Gabriele Gille et al.   JOURNAL OF NEURAL TRANSMISSION

A mitochondrial ubiquitin ligase MITOL controls cell toxicity of polyglutamine-expanded protein

(2010) Ayumu Sugiura et al.   MITOCHONDRION

The AAA-ATPase p97 is essential for outer mitochondrial membrane protein turnover

(2010) Shan Xu et al.   MOLECULAR BIOLOGY OF THE CELL

The complexities of p97 function in health and disease

(2010) Eli Chapman et al.   Molecular BioSystems

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

(2010) Daniela Di Bella et al.   NATURE GENETICS

Misfolded Mutant SOD1 Directly Inhibits VDAC1 Conductance in a Mouse Model of Inherited ALS

(2010) Adrian Israelson et al.   NEURON

ALS-linked mutant superoxide dismutase 1 (SOD1) alters mitochondrial protein composition and decreases protein import

(2010) Q. Li et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Mitochondrial optic neuropathies – Disease mechanisms and therapeutic strategies

(2010) Patrick Yu-Wai-Man et al.   PROGRESS IN RETINAL AND EYE RESEARCH

The Role of Mitochondria in Apoptosis

(2009) Chunxin Wang et al.   Annual Review of Genetics

Retinal ganglion cell neurodegeneration in mitochondrial inherited disorders

(2009) Valerio Carelli et al.   BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS

Emerging roles of mitochondrial proteases in neurodegeneration

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

Gene–environment interactions in Leber hereditary optic neuropathy

(2009) Matthew Anthony Kirkman et al.   BRAIN

MAPL is a new mitochondrial SUMO E3 ligase that regulates mitochondrial fission

(2009) Emélie Braschi et al.   EMBO REPORTS

Regulation of OPA1 processing and mitochondrial fusion bym-AAA protease isoenzymes and OMA1

(2009) Sarah Ehses et al.   JOURNAL OF CELL BIOLOGY

Inducible proteolytic inactivation of OPA1 mediated by the OMA1 protease in mammalian cells

(2009) Brian Head et al.   JOURNAL OF CELL BIOLOGY

Controlling metabolism and cell death: At the heart of mitochondrial calcium signalling

(2009) Marta Murgia et al.   JOURNAL OF MOLECULAR AND CELLULAR CARDIOLOGY

Mitochondrial Ubiquitin Ligase MITOL Ubiquitinates Mutant SOD1 and Attenuates Mutant SOD1-induced Reactive Oxygen Species Generation

(2009) Ryo Yonashiro et al.   MOLECULAR BIOLOGY OF THE CELL

A Mutation Associated with CMT2A Neuropathy Causes Defects in Fzo1 GTP Hydrolysis, Ubiquitylation, and Protein Turnover

(2009) Elizabeth A. Amiott et al.   MOLECULAR BIOLOGY OF THE CELL

Increasing organismal healthspan by enhancing mitochondrial protein quality control

(2009) Karin Luce et al.   NATURE CELL BIOLOGY

Mitochondrial fission factor Drp1 is essential for embryonic development and synapse formation in mice

(2009) Naotada Ishihara et al.   NATURE CELL BIOLOGY

S-Nitrosylation of Drp1 Mediates β-Amyloid-Related Mitochondrial Fission and Neuronal Injury

(2009) Dong-Hyung Cho et al.   SCIENCE

Friedreich Ataxia

(2008) Massimo Pandolfo   ARCHIVES OF NEUROLOGY

Metalloprotease-mediated OPA1 processing is modulated by the mitochondrial membrane potential

(2008) Olwenn Guillery et al.   BIOLOGY OF THE CELL

A Mitochondrial Protein Compendium Elucidates Complex I Disease Biology

(2008) David J. Pagliarini et al.   CELL

GIDE is a mitochondrial E3 ubiquitin ligase that induces apoptosis and slows growth

(2008) Bicheng Zhang et al.   CELL RESEARCH

Cargo-Selected Transport from the Mitochondria to Peroxisomes Is Mediated by Vesicular Carriers

(2008) Margaret Neuspiel et al.   CURRENT BIOLOGY

Quality control of mitochondria: protection against neurodegeneration and ageing

(2008) Takashi Tatsuta et al.   EMBO JOURNAL

Fission and selective fusion govern mitochondrial segregation and elimination by autophagy

(2008) Gilad Twig et al.   EMBO JOURNAL

Mitochondrial fusion and function in Charcot–Marie–Tooth type 2A patient fibroblasts with mitofusin 2 mutations

(2008) Elizabeth A. Amiott et al.   EXPERIMENTAL NEUROLOGY

Effects of overexpression of Huntingtin proteins on mitochondrial integrity

(2008) Hongmin Wang et al.   HUMAN MOLECULAR GENETICS

Supercomplex organization of the oxidative phosphorylation enzymes in yeast mitochondria

(2008) Rosemary A. Stuart   JOURNAL OF BIOENERGETICS AND BIOMEMBRANES

Molecular Machinery of Mitochondrial Fusion and Fission

(2008) Benedikt Westermann   JOURNAL OF BIOLOGICAL CHEMISTRY

The Novel Tail-anchored Membrane Protein Mff Controls Mitochondrial and Peroxisomal Fission in Mammalian Cells

(2008) Shilpa Gandre-Babbe et al.   MOLECULAR BIOLOGY OF THE CELL

Mitofusin 2 tethers endoplasmic reticulum to mitochondria

(2008) Olga Martins de Brito et al.   NATURE

Dephosphorylation by calcineurin regulates translocation of Drp1 to mitochondria

(2008) G. M. Cereghetti et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

Amyloid-  overproduction causes abnormal mitochondrial dynamics via differential modulation of mitochondrial fission/fusion proteins

(2008) X. Wang et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

The mitochondrial impairment, oxidative stress and neurodegeneration connection: reality or just an attractive hypothesis?

(2008) Hirokazu Fukui et al.   TRENDS IN NEUROSCIENCES

The BCL-2 protein family: opposing activities that mediate cell death

(2007) Richard J. Youle et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY