Hypomyelinating leukodystrophies — unravelling myelin biology

Hypomyelinating leukodystrophies constitute a subset of genetic white matter disorders characterized by a primary lack of myelin deposition. Most patients with severe hypomyelination present in infancy or early childhood and develop severe neurological deficits, but the clinical presentation can als...

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Bibliographic Details
Published in:Nature reviews. Neurology 2021-02, Vol.17 (2), p.88-103
Main Authors: Wolf, Nicole I., ffrench-Constant, Charles, van der Knaap, Marjo S.
Format: Article
Language:English
Subjects:
692/617/375/2764
692/617/375/366
Animals
Biology
Brain - diagnostic imaging
Brain - metabolism
Demyelinating Diseases - diagnostic imaging
Demyelinating Diseases - metabolism
Development and progression
Globoid cell leukodystrophy
Humans
Leukoencephalopathies - diagnostic imaging
Leukoencephalopathies - metabolism
Magnetic Resonance Imaging - methods
Medicine
Medicine & Public Health
Myelin proteins
Myelin Sheath - metabolism
Myelin Sheath - pathology
Neurology
Oligodendroglia - metabolism
Oligodendroglia - pathology
Physiological aspects
Proteins
Review Article
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