White matter disturbances in phenylketonuria: Possible underlying mechanisms

Transcriptional control of myelination and remyelination

(2019) Elisabeth Sock et al.   GLIA

Axoglial interactions in myelin plasticity: Evaluating the relationship between neuronal activity and oligodendrocyte dynamics

(2019) Antoinette Y. Foster et al.   GLIA

A Microtubule-Myelination Connection

(2019) Antonina Roll-Mecak   CELL

Oligodendrocytes express synaptic proteins that modulate myelin sheath formation

(2019) Alexandria N. Hughes et al.   Nature Communications

Metabolomic Markers of Essential Fatty Acids, Carnitine, and Cholesterol Metabolism in Adults and Adolescents with Phenylketonuria

(2018) Bridget M Stroup et al.   JOURNAL OF NUTRITION

Brain bioenergetics in rats with acute hyperphenylalaninemia

(2018) Nádia Weber Dimer et al.   NEUROCHEMISTRY INTERNATIONAL

Protective effects of a radical scavenger edaravone on oligodendrocyte precursor cells against oxidative stress

(2018) Hajime Takase et al.   NEUROSCIENCE LETTERS

Status of nutrients important in brain function in phenylketonuria: a systematic review and meta-analysis

(2018) Gina A. Montoya Parra et al.   Orphanet Journal of Rare Diseases

Regulation of Central Nervous System Myelination in Higher Brain Functions

(2018) Mara Nickel et al.   NEURAL PLASTICITY

Polysialylation at Early Stages of Oligodendrocyte Differentiation Promotes Myelin Repair

(2017) Sebastian Werneburg et al.   JOURNAL OF NEUROSCIENCE

Ca2+ activity signatures of myelin sheath formation and growth in vivo

(2017) Marion Baraban et al.   NATURE NEUROSCIENCE

Regulation of developing myelin sheath elongation by oligodendrocyte calcium transients in vivo

(2017) Anna M. Krasnow et al.   NATURE NEUROSCIENCE

Single-cell analysis of experience-dependent transcriptomic states in the mouse visual cortex

(2017) Sinisa Hrvatin et al.   NATURE NEUROSCIENCE

Oxygen impairs oligodendroglial development via oxidative stress and reduced expression of HIF-1α

(2017) Christina Brill et al.   Scientific Reports

Intrinsic property of phenylalanine to trigger protein aggregation and hemolysis has a direct relevance to phenylketonuria

(2017) Bibin G. Anand et al.   Scientific Reports

Expression of Kir4.1 and Kir5.1 inwardly rectifying potassium channels in oligodendrocytes, the myelinating cells of the CNS

(2016) C. Brasko et al.   Brain Structure & Function

The role of growth factors as a therapeutic approach to demyelinating disease

(2016) Yangyang Huang et al.   EXPERIMENTAL NEUROLOGY

Origin of oligodendrocytes in mammalian forebrains: a revised perspective

(2016) Masae Naruse et al.   Journal of Physiological Sciences

DNA methylation in the pathophysiology of hyperphenylalaninemia in the PAH enu2 mouse model of phenylketonuria

(2016) S.F. Dobrowolski et al.   MOLECULAR GENETICS AND METABOLISM

Morphometric analysis of gray matter integrity in individuals with early-treated phenylketonuria

(2016) Shawn E. Christ et al.   MOLECULAR GENETICS AND METABOLISM

Adult-onset phenylketonuria with rapidly progressive dementia and parkinsonism

(2016) Zeynep Tufekcioglu et al.   NEUROCASE

Brain-Derived Neurotrophic Factor Deficiency Restricts Proliferation of Oligodendrocyte Progenitors Following Cuprizone-Induced Demyelination

(2015) Vladislav Tsiperson et al.   ASN Neuro

Metabolism and functions of lipids in myelin

(2015) Sebastian Schmitt et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR AND CELL BIOLOGY OF LIPIDS

Cholesterol in myelin biogenesis and hypomyelinating disorders

(2015) Gesine Saher et al.   BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR AND CELL BIOLOGY OF LIPIDS

CNS Myelin Wrapping Is Driven by Actin Disassembly

(2015) J. Bradley Zuchero et al.   DEVELOPMENTAL CELL

Urinary biomarkers of oxidative stress and plasmatic inflammatory profile in phenylketonuric treated patients

(2015) Marion Deon et al.   INTERNATIONAL JOURNAL OF DEVELOPMENTAL NEUROSCIENCE

Altered DNA methylation in PAH deficient phenylketonuria

(2015) Steven F. Dobrowolski et al.   MOLECULAR GENETICS AND METABOLISM

Neurocognitive and neuroimaging outcome of early treated young adult PKU patients: A longitudinal study

(2015) Francesca Nardecchia et al.   MOLECULAR GENETICS AND METABOLISM

Exploring the origins of grey matter damage in multiple sclerosis

(2015) Massimiliano Calabrese et al.   NATURE REVIEWS NEUROSCIENCE

Plasma cholesterol in adults with phenylketonuria

(2015) Robin A. Williams et al.   PATHOLOGY

Myelin, myelin-related disorders, and psychosis

(2015) Michelle I. Mighdoll et al.   SCHIZOPHRENIA RESEARCH

Oligodendrocytes: Myelination and Axonal Support

(2015) Mikael Simons et al.   Cold Spring Harbor Perspectives in Biology

Phenylketonuria Pathophysiology: on the Role of Metabolic Alterations

(2015) Patrícia Fernanda Schuck et al.   Aging and Disease

Dynamics of Oligodendrocyte Generation and Myelination in the Human Brain

(2014) Maggie S.Y. Yeung et al.   CELL

Phenylketonuria: Brain Phenylalanine Concentrations Relate Inversely to Cerebral Protein Synthesis

(2014) Martijn J de Groot et al.   JOURNAL OF CEREBRAL BLOOD FLOW AND METABOLISM

Altered brain protein expression profiles are associated with molecular neurological dysfunction in the PKU mouse model

(2014) Esther Imperlini et al.   JOURNAL OF NEUROCHEMISTRY

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

Myelin vs Axon Abnormalities in White Matter in Bipolar Disorder

(2014) Kathryn E Lewandowski et al.   NEUROPSYCHOPHARMACOLOGY

Myelination and Oligodendrocyte Functions in Psychiatric Diseases

(2014) Klaus-Armin Nave et al.   JAMA Psychiatry

Oligodendrogenesis in the normal and pathological central nervous system

(2014) Bilal El Waly et al.   Frontiers in Neuroscience

DNA damage induced by phenylalanine and its analogue p-chlorophenylalanine in blood and brain of rats subjected to a model of hyperphenylalaninemia

(2013) Kellen R. Simon et al.   Biochemistry and Cell Biology

Intrinsic and extrinsic control of oligodendrocyte development

(2013) J Bradley Zuchero et al.   CURRENT OPINION IN NEUROBIOLOGY

Role of CNPase in the oligodendrocytic extracellular 2′,3′-cAMP-adenosine pathway

(2013) Jonathan D. Verrier et al.   GLIA

Phenylalanine hydroxylase: Function, structure, and regulation

(2013) Marte I. Flydal et al.   IUBMB LIFE

Tract-based evaluation of white matter damage in individuals with early-treated phenylketonuria

(2013) Huiling Peng et al.   JOURNAL OF INHERITED METABOLIC DISEASE

White matter integrity and executive abilities in individuals with phenylketonuria

(2013) Jo Ann V. Antenor-Dorsey et al.   MOLECULAR GENETICS AND METABOLISM

Optimal serum phenylalanine for adult patients with phenylketonuria

(2013) Yoshiyuki Okano et al.   MOLECULAR GENETICS AND METABOLISM

Normal Myelination

(2013) Helen M. Branson   NEUROIMAGING CLINICS OF NORTH AMERICA

Mechanisms regulating the development of oligodendrocytes and central nervous system myelin

(2013) S. Mitew et al.   NEUROSCIENCE

Phenylketonuria: reduced tyrosine brain influx relates to reduced cerebral protein synthesis

(2013) Martijn J de Groot et al.   Orphanet Journal of Rare Diseases

Oligodendroglia: metabolic supporters of axons

(2013) Brett M. Morrison et al.   TRENDS IN CELL BIOLOGY

Free asymmetric dimethylarginine (ADMA) is low in children and adolescents with classical phenylketonuria (PKU)

(2012) M. Huemer et al.   JOURNAL OF INHERITED METABOLIC DISEASE

The brain in vivo expresses the 2′,3′-cAMP-adenosine pathway

(2012) Jonathan D. Verrier et al.   JOURNAL OF NEUROCHEMISTRY

Specification and maintenance of oligodendrocyte precursor cells from neural progenitor cells: involvement of microRNA-7a

(2012) Xianghui Zhao et al.   MOLECULAR BIOLOGY OF THE CELL

A volumetric study of basal ganglia structures in individuals with early-treated phenylketonuria

(2012) Kimberly E. Bodner et al.   MOLECULAR GENETICS AND METABOLISM

Phenylalanine assembly into toxic fibrils suggests amyloid etiology in phenylketonuria

(2012) Lihi Adler-Abramovich et al.   Nature Chemical Biology

Biogenesis and Significance of Central Nervous System Myelin

(2012) M. Soldán et al.   SEMINARS IN NEUROLOGY

Phenylalanine hydroxylase deficiency

(2011) John J. Mitchell et al.   GENETICS IN MEDICINE

An Alzheimer's disease-relevant presenilin-1 mutation augments amyloid-beta-induced oligodendrocyte dysfunction

(2011) Maya K. Desai et al.   GLIA

PDGF-AA Mediates B104CM-Induced Oligodendrocyte Precursor Cell Differentiation of Embryonic Neural Stem Cells Through Erk, PI3K, and p38 Signaling

(2011) Jian-Guo Hu et al.   JOURNAL OF MOLECULAR NEUROSCIENCE

Genetically Induced Adult Oligodendrocyte Cell Death Is Associated with Poor Myelin Clearance, Reduced Remyelination, and Axonal Damage

(2011) H. B. F. Pohl et al.   JOURNAL OF NEUROSCIENCE

Mapping Infant Brain Myelination with Magnetic Resonance Imaging

(2011) S. C. L. Deoni et al.   JOURNAL OF NEUROSCIENCE

Pyruvate and creatine prevent oxidative stress and behavioral alterations caused by phenylalanine administration into hippocampus of rats

(2011) Simone Luisa Berti et al.   METABOLIC BRAIN DISEASE

Regular exercise prevents oxidative stress in the brain of hyperphenylalaninemic rats

(2011) Priscila Nicolao Mazzola et al.   METABOLIC BRAIN DISEASE

Mechanisms regulating superoxide generation in experimental models of phenylketonuria: An essential role of NADPH oxidase

(2011) Lihua Lu et al.   MOLECULAR GENETICS AND METABOLISM

Targeted Ablation of Oligodendrocytes Triggers Axonal Damage

(2011) Aniket Ghosh et al.   PLoS One

Central nervous system myelin: structure, synthesis and assembly

(2011) Shweta Aggarwal et al.   TRENDS IN CELL BIOLOGY

Neurological outcome in 22 treated adolescents with hyperphenylalaninemia

(2010) A. C. Ludolph et al.   ACTA NEUROLOGICA SCANDINAVICA

Evidence that l-Carnitine and Selenium Supplementation Reduces Oxidative Stress in Phenylketonuric Patients

(2010) A. Sitta et al.   CELLULAR AND MOLECULAR NEUROBIOLOGY

Progressive Neural Muscular Atrophy in à Case of Phenylketonuria

(2010) C. Meier et al.   DEVELOPMENTAL MEDICINE AND CHILD NEUROLOGY

Oligodendrocyte development and myelinogenesis are not impaired by high concentrations of phenylalanine or its metabolites

(2010) Renaud Schoemans et al.   JOURNAL OF INHERITED METABOLIC DISEASE

Effects of phenylalanine on the survival and neurite outgrowth of rat cortical neurons in primary cultures: possible involvement of brain-derived neurotrophic factor

(2010) Duan Li et al.   MOLECULAR AND CELLULAR BIOCHEMISTRY

Myelination and support of axonal integrity by glia

(2010) Klaus-Armin Nave   NATURE

Regulation of Oligodendrocyte Differentiation and Myelination

(2010) B. Emery   SCIENCE

Experimental Evidence that Phenylalanine Provokes Oxidative Stress in Hippocampus and Cerebral Cortex of Developing Rats

(2009) Carolina G. Fernandes et al.   CELLULAR AND MOLECULAR NEUROBIOLOGY

Oligodendroglial impact on axonal function and survival – a hypothesis

(2009) Celia Michèle Kassmann et al.   CURRENT OPINION IN NEUROLOGY

Neuropathology of a 4-month-old infant born to a woman with phenylketonuria

(2009) Richard Koch et al.   DEVELOPMENTAL MEDICINE AND CHILD NEUROLOGY

Early ultrastructural defects of axons and axon-glia junctions in mice lacking expression ofCnp1

(2009) Julia M. Edgar et al.   GLIA

Neural cell adhesion molecule stimulates survival of premyelinating oligodendrocytes via the fibroblast growth factor receptor

(2009) Anne L. Palser et al.   JOURNAL OF NEUROSCIENCE RESEARCH

Phenylketonuria: High plasma phenylalanine decreases cerebral protein synthesis

(2009) Marieke Hoeksma et al.   MOLECULAR GENETICS AND METABOLISM

White matter pathology in phenylketonuria☆

(2009) Peter J. Anderson et al.   MOLECULAR GENETICS AND METABOLISM

Evidence that DNA damage is associated to phenylalanine blood levels in leukocytes from phenylketonuric patients

(2009) Angela Sitta et al.   MUTATION RESEARCH-GENETIC TOXICOLOGY AND ENVIRONMENTAL MUTAGENESIS

Brain-Derived Neurotrophic Factor Inhibits Phenylalanine-Induced Neuronal Apoptosis by Preventing RhoA Pathway Activation

(2009) Yongjun Zhang et al.   NEUROCHEMICAL RESEARCH

Classification of Childhood White Matter Disorders Using Proton MR Spectroscopic Imaging

(2008) A. Bizzi et al.   AMERICAN JOURNAL OF NEURORADIOLOGY

Neuropsychological speed tests and blood phenylalanine levels in patients with phenylketonuria: A meta-analysis

(2008) Julia Albrecht et al.   NEUROSCIENCE AND BIOBEHAVIORAL REVIEWS

Platelet-derived growth factor-AA mediates oligodendrocyte lineage differentiation through activation of extracellular signal-regulated kinase signaling pathway

(2007) J.-G. Hu et al.   NEUROSCIENCE

Specification of CNS glia from neural stem cells in the embryonic neuroepithelium

(2007) N. Kessaris et al.   PHILOSOPHICAL TRANSACTIONS OF THE ROYAL SOCIETY B-BIOLOGICAL SCIENCES