Astrocyte‐specific deletion of the mitochondrial m ‐AAA protease reveals glial contribution to neurodegeneration

m-AAA proteases, mitochondrial calcium homeostasis and neurodegeneration

(2018) Maria Patron et al.   CELL RESEARCH

ZBP1: Innate Sensor Regulating Cell Death and Inflammation

(2018) Teneema Kuriakose et al.   TRENDS IN IMMUNOLOGY

Loss of mtDNA activates astrocytes and leads to spongiotic encephalopathy

(2018) Olesia Ignatenko et al.   Nature Communications

Neuronal Mitochondrial Dysfunction Activates the Integrated Stress Response to Induce Fibroblast Growth Factor 21

(2018) Lisa Michelle Restelli et al.   Cell Reports

Respiration-Deficient Astrocytes Survive As Glycolytic Cells In Vivo

(2017) Lotti M. Supplie et al.   JOURNAL OF NEUROSCIENCE

Neurotoxic reactive astrocytes are induced by activated microglia

(2017) Shane A. Liddelow et al.   NATURE

Mitochondrial diseases: the contribution of organelle stress responses to pathology

(2017) Anu Suomalainen et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

Recessive AFG3L2 Mutation Causes Progressive Microcephaly, Early Onset Seizures, Spasticity, and Basal Ganglia Involvement

(2017) Alaa Eskandrani et al.   PEDIATRIC NEUROLOGY

Downregulation of the Glial GLT1 Glutamate Transporter and Purkinje Cell Dysfunction in a Mouse Model of Myotonic Dystrophy

(2017) Géraldine Sicot et al.   Cell Reports

Transcriptomic and proteomic landscape of mitochondrial dysfunction reveals secondary coenzyme Q deficiency in mammals

(2017) Inge Kühl et al.   eLife

Transcriptome Analysis Reveals Neuroprotective aspects of Human Reactive Astrocytes induced by Interleukin 1β

(2017) Daniel Boon Loong Teh et al.   Scientific Reports

Mitochondrial DNA Replication Defects Disturb Cellular dNTP Pools and Remodel One-Carbon Metabolism

(2016) Joni Nikkanen et al.   Cell Metabolism

The m -AAA Protease Associated with Neurodegeneration Limits MCU Activity in Mitochondria

(2016) Tim König et al.   MOLECULAR CELL

RIPK1 counteracts ZBP1-mediated necroptosis to inhibit inflammation

(2016) Juan Lin et al.   NATURE

Functional diversity of astrocytes in neural circuit regulation

(2016) Lucile Ben Haim et al.   NATURE REVIEWS NEUROSCIENCE

RIPK1 mediates axonal degeneration by promoting inflammation and necroptosis in ALS

(2016) Yasushi Ito et al.   SCIENCE

The Mitochondrial m-AAA Protease Prevents Demyelination and Hair Greying

(2016) Shuaiyu Wang et al.   PLoS Genetics

Sex differences in glia reactivity after cortical brain injury

(2015) Estefania Acaz-Fonseca et al.   GLIA

Necroptosis and its role in inflammation

(2015) Manolis Pasparakis et al.   NATURE

Reappraisal of Bergmann glial cells as modulators of cerebellar circuit function

(2015) Chris I. De Zeeuw et al.   Frontiers in Cellular Neuroscience

Astrocytes Control Synapse Formation, Function, and Elimination

(2015) Won-Suk Chung et al.   Cold Spring Harbor Perspectives in Biology

Mitochondrial dynamics in astrocytes

(2014) Terri-Leigh Stephen et al.   BIOCHEMICAL SOCIETY TRANSACTIONS

Purkinje neuron Ca2+ influx reduction rescues ataxia in SCA28 model

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

An RNA-Sequencing Transcriptome and Splicing Database of Glia, Neurons, and Vascular Cells of the Cerebral Cortex

(2014) Y. Zhang et al.   JOURNAL OF NEUROSCIENCE

Partial deletion of AFG3L2 causing spinocerebellar ataxia type 28

(2014) K. Smets et al.   NEUROLOGY

Necroptosis Drives Motor Neuron Death in Models of Both Sporadic and Familial ALS

(2014) Diane B. Re et al.   NEURON

Mitochondrial quality control: a matter of life and death for neurons

(2012) Elena I Rugarli et al.   EMBO JOURNAL

Respiratory dysfunction by AFG3L2 deficiency causes decreased mitochondrial calcium uptake via organellar network fragmentation

(2012) F. Maltecca et al.   HUMAN MOLECULAR GENETICS

AFG3L2 supports mitochondrial protein synthesis and Purkinje cell survival

(2012) Eva R. Almajan et al.   JOURNAL OF CLINICAL INVESTIGATION

Genomic Analysis of Reactive Astrogliosis

(2012) J. L. Zamanian et al.   JOURNAL OF NEUROSCIENCE

Brain Energy Metabolism: Focus on Astrocyte-Neuron Metabolic Cooperation

(2011) Mireille Bélanger et al.   Cell Metabolism

Glial glutamate transporters: New actors in brain signaling

(2011) Esther López-Bayghen et al.   IUBMB LIFE

Impaired mitochondrial transport and Parkin-independent degeneration of respiratory chain-deficient dopamine neurons in vivo

(2011) F. H. Sterky et al.   PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA

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

Deranged Calcium Signaling in Purkinje Cells and Pathogenesis in Spinocerebellar Ataxia 2 (SCA2) and Other Ataxias

(2010) Adebimpe Kasumu et al.   CEREBELLUM

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

(2010) Daniela Di Bella et al.   NATURE GENETICS

Astrocytes: biology and pathology

(2009) Michael V. Sofroniew et al.   ACTA NEUROPATHOLOGICA

Genetic interaction between the m -AAA protease isoenzymes reveals novel roles in cerebellar degeneration

(2009) Paola Martinelli et al.   HUMAN MOLECULAR GENETICS

Haploinsufficiency of AFG3L2, the Gene Responsible for Spinocerebellar Ataxia Type 28, Causes Mitochondria-Mediated Purkinje Cell Dark Degeneration

(2009) F. Maltecca et al.   JOURNAL OF NEUROSCIENCE

Precursors with Glial Fibrillary Acidic Protein Promoter Activity Transiently Generate GABA Interneurons in the Postnatal Cerebellum

(2009) John Silbereis et al.   STEM CELLS

Gender-specific regulation of mitochondrial fusion and fission gene transcription and viability of cortical astrocytes by steroid hormones

(2008) Susanne Arnold et al.   JOURNAL OF MOLECULAR ENDOCRINOLOGY

The Mitochondrial Protease AFG3L2 Is Essential for Axonal Development

(2008) F. Maltecca et al.   JOURNAL OF NEUROSCIENCE

Inducible Cre recombinase activity in mouse mature astrocytes and adult neural precursor cells

(2008) Lionel M. L. Chow et al.   TRANSGENIC RESEARCH