Central nervous system dysfunction in a mouse model of FA2H deficiency - sprookjes - yvandenbroek - TriplyDB

Central nervous system dysfunction in a mouse model of FA2H deficiency

Central nervous system dysfunction in a mouse model of FA2H deficiency

http://www.wikidata.org/entity/Q28591388

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Neurodegeneration with brain iron accumulation: update on pathogenic mechanisms 2-Hydroxylated sphingomyelin profiles in cells from patients with mutated fatty acid 2-hydroxylase Delving into the complexity of hereditary spastic paraplegias: how unexpected phenotypes and inheritance modes are revolutionizing their nosology Developing therapeutic approaches for metachromatic leukodystrophy Nimodipine but Not Nifedipine Promotes Expression of Fatty Acid 2-Hydroxylase in a Surgical Stress Model Based on Neuro2a Cells Dual functions of the trans-2-enoyl-CoA reductase TER in the sphingosine 1-phosphate metabolic pathway and in fatty acid elongation.Exome sequencing and SNP analysis detect novel compound heterozygosity in fatty acid hydroxylase-associated neurodegeneration.Gene co-expression networks shed light into diseases of brain iron accumulation.Disrupted SOX10 function causes spongiform neurodegeneration in gray tremor mice.Stereospecificity of fatty acid 2-hydroxylase and differential functions of 2-hydroxy fatty acid enantiomers.Activated immune response in an inherited leukodystrophy disease caused by the loss of oligodendrocyte gap junctions.On the complexity of clinical and molecular bases of neurodegeneration with brain iron accumulation.Review: Insights into molecular mechanisms of disease in neurodegeneration with brain iron accumulation: unifying theories.Mutation for nonsyndromic mental retardation in the trans-2-enoyl-CoA reductase TER gene involved in fatty acid elongation impairs the enzyme activity and stability, leading to change in sphingolipid profile 2'-Hydroxy ceramide in membrane homeostasis and cell signaling.Syndromes of neurodegeneration with brain iron accumulation (NBIA): an update on clinical presentations, histological and genetic underpinnings, and treatment considerations.Cellular pathways of hereditary spastic paraplegia.Excess iron harms the brain: the syndromes of neurodegeneration with brain iron accumulation (NBIA).Pathophysiology and treatment of neurodegeneration with brain iron accumulation in the pediatric population.Multispectral imaging: a review of its technical aspects and applications in anatomic pathology.The neuropathology of neurodegeneration with brain iron accumulation.Human genetic disorders of sphingolipid biosynthesis.Defective lipid metabolism in neurodegeneration with brain iron accumulation (NBIA) syndromes: not only a matter of iron.Key Questions for Translation of FFA Receptors: From Pharmacology to Medicines.Deferiprone Rescues Behavioral Deficits Induced by Mild Iron Exposure in a Mouse Model of Alpha-Synuclein Aggregation.Phytosphingosine degradation pathway includes fatty acid α-oxidation reactions in the endoplasmic reticulum.Genetics and Pathophysiology of Neurodegeneration with Brain Iron Accumulation (NBIA).Mutations in FA2H in three Arab families with a clinical spectrum of neurodegeneration and hereditary spastic paraparesis.Functional annotation of genes differentially expressed between primary motor and prefrontal association cortices of macaque brain.Independent association of plasma hydroxysphingomyelins with physical function in the Atherosclerosis Risk in Communities (ARIC) study.Loss of Cdk5 function in the nucleus accumbens decreases wheel running and may mediate age-related declines in voluntary physical activity.Ulk4 deficiency leads to hypomyelination in mice.Altered distribution of ATG9A and accumulation of axonal aggregates in neurons from a mouse model of AP-4 deficiency syndrome.

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Central nervous system dysfunction in a mouse model of FA2H deficiency

http://www.wikidata.org/entity/Q28591388

description

2011 թուականի Յուլիսին հրատարակուած գիտական յօդուած

@hyw

2011 թվականի հուլիսին հրատարակված գիտական հոդված

@hy

artículu científicu espublizáu en 2011

@ast

im Juli 2011 veröffentlichter wissenschaftlicher Artikel

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scientific journal article

@en

vedecký článok (publikovaný 2011/07/01)

@sk

vědecký článek publikovaný v roce 2011

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wetenschappelijk artikel (gepubliceerd op 2011/07/01)

@nl

наукова стаття, опублікована в липні 2011

@uk

مقالة علمية (نشرت في يوليو 2011)

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name

Central nervous system dysfunction in a mouse model of FA2H deficiency

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Central nervous system dysfunction in a mouse model of FA2H deficiency

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Central nervous system dysfunction in a mouse model of FA2H deficiency

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type

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Central nervous system dysfunction in a mouse model of FA2H deficiency

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Central nervous system dysfunction in a mouse model of FA2H deficiency

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Central nervous system dysfunction in a mouse model of FA2H deficiency

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prefLabel

Central nervous system dysfunction in a mouse model of FA2H deficiency

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Central nervous system dysfunction in a mouse model of FA2H deficiency

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Central nervous system dysfunction in a mouse model of FA2H deficiency

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Central nervous system dysfunction in a mouse model of FA2H deficiency

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Hiroko Hama

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Hua Cheng

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Jacek Bielawski

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Jagadish Kummetha Venkata

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Jian J. Li

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Kathleen A. Potter

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Sabrina W. Yum

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P2860

Global analyses of cellular lipidomes directly from crude extracts of biological samples by ESI mass spectrometry: a bridge to lipidomics

Shotgun lipidomics: electrospray ionization mass spectrometric analysis and quantitation of cellular lipidomes directly from crude extracts of biological samples

Absence of 2-hydroxylated sphingolipids is compatible with normal neural development but causes late-onset axon and myelin sheath degeneration

Disruption of Cnp1 uncouples oligodendroglial functions in axonal support and myelination

A mammalian fatty acid hydroxylase responsible for the formation of alpha-hydroxylated galactosylceramide in myelin

ZFP191 is required by oligodendrocytes for CNS myelination

FA2H-dependent fatty acid 2-hydroxylation in postnatal mouse brain

Place navigation impaired in rats with hippocampal lesions

Axonal transection in the lesions of multiple sclerosis

A genetic mouse model of adult-onset, pervasive central nervous system demyelination with robust remyelination.

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