Mutant eIF2B leads to impaired mitochondrial oxidative phosphorylation in vanishing white matter disease

Astrocytes are central in the pathomechanisms of vanishing white matter

(2016) Stephanie Dooves et al.   JOURNAL OF CLINICAL INVESTIGATION

Mitonuclear communication in homeostasis and stress

(2016) Pedro M. Quirós et al.   NATURE REVIEWS MOLECULAR CELL BIOLOGY

A Comprehensive Genomic Analysis Reveals the Genetic Landscape of Mitochondrial Respiratory Chain Complex Deficiencies

(2016) Masakazu Kohda et al.   PLoS Genetics

Stoichiometry of the eIF2B complex is maintained by mutual stabilization of subunits

(2015) N. C. Wortham et al.   BIOCHEMICAL JOURNAL

Resistance of glia-like central and peripheral neural stem cells to genetically induced mitochondrial dysfunction--differential effects on neurogenesis

(2015) B. Diaz-Castro et al.   EMBO REPORTS

Proteomics-level analysis of myelin formation and regeneration in a mouse model for Vanishing White Matter disease

(2015) Irit Gat-Viks et al.   JOURNAL OF NEUROCHEMISTRY

MitoCarta2.0: an updated inventory of mammalian mitochondrial proteins

(2015) Sarah E. Calvo et al.   NUCLEIC ACIDS RESEARCH

Mitochondrial dysfunction in central nervous system white matter disorders

(2014) Laia Morató et al.   GLIA

Glycolytic Switch in Response to Betulinic Acid in Non-Cancer Cells

(2014) Elke H. Heiss et al.   PLoS One

Hyaluronan accumulation and arrested oligodendrocyte progenitor maturation in vanishing white matter disease

(2013) Marianna Bugiani et al.   BRAIN

Microscale oxygraphy reveals OXPHOS impairment in MRC mutant cells

(2012) F. Invernizzi et al.   MITOCHONDRION

Mitochondrial disorders as windows into an ancient organelle

(2012) Scott B. Vafai et al.   NATURE

Poor Cerebral Inflammatory Response in eIF2B Knock-In Mice: Implications for the Aetiology of Vanishing White Matter Disease

(2012) Yuval Cabilly et al.   PLoS One

Human diseases with impaired mitochondrial protein synthesis

(2011) Agnès Rötig   BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS

A mouse model for eukaryotic translation initiation factor 2B-leucodystrophy reveals abnormal development of brain white matter

(2011) Michal Geva et al.   BRAIN

Mitotic Modulation of Translation Elongation Factor 1 Leads to Hindered tRNA Delivery to Ribosomes

(2011) Gilad Sivan et al.   JOURNAL OF BIOLOGICAL CHEMISTRY

A Point Mutation in Translation Initiation Factor eIF2B Leads to Function- and Time-Specific Changes in Brain Gene Expression

(2011) Liraz Marom et al.   PLoS One

Leukoencephalopathy With Vanishing White Matter: A Review

(2010) Marianna Bugiani et al.   JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY

Defective Glial Maturation in Vanishing White Matter Disease

(2010) Marianna Bugiani et al.   JOURNAL OF NEUROPATHOLOGY AND EXPERIMENTAL NEUROLOGY

Structural and functional link between the mitochondrial network and the endoplasmic reticulum

(2009) Carlotta Giorgi et al.   INTERNATIONAL JOURNAL OF BIOCHEMISTRY & CELL BIOLOGY

Oligodendroglial differentiation induces mitochondrial genes and inhibition of mitochondrial function represses oligodendroglial differentiation

(2009) Robert Schoenfeld et al.   MITOCHONDRION

Inhibition of mitochondrial function induces an integrated stress response in oligodendroglia

(2009) Jillian M. Silva et al.   NEUROBIOLOGY OF DISEASE

A Point Mutation in Translation Initiation Factor 2B Leads to a Continuous Hyper Stress State in Oligodendroglial-Derived Cells

(2008) Liraz Kantor et al.   PLoS One