Increasing Sulfatide Synthesis in Myelin-Forming Cells of Arylsulfatase A-Deficient Mice Causes Demyelination and Neurological Symptoms Reminiscent of Human Metachromatic Leukodystrophy

Metachromatic leukodystrophy (MLD) is a lysosomal storage disorder caused by the deficiency of arylsulfatase A (ASA). This results in accumulation of sulfated glycosphingolipids, mainly 3-O-sulfogalactosylceramide (sulfatide), in the nervous system and various other organs. In patients, lipid storag...

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Veröffentlicht in:	The Journal of neuroscience 2007-08, Vol.27 (35), p.9482-9490
Hauptverfasser:	Ramakrishnan, Hariharasubramanian, Hedayati, Kerstin Khalaj, Lullmann-Rauch, Renate, Wessig, Carsten, Fewou, Simon Ngamli, Maier, Helena, Goebel, Hans-Hilmar, Gieselmann, Volkmar, Eckhardt, Matthias
Format:	Artikel
Sprache:	eng
Schlagworte:	Age Factors Animals Cerebroside-Sulfatase - deficiency Demyelinating Diseases - etiology Disease Models, Animal Electromyography - methods Hindlimb Suspension - methods Humans Leukodystrophy, Metachromatic - complications Leukodystrophy, Metachromatic - metabolism Leukodystrophy, Metachromatic - pathology Lipids - analysis Mice Mice, Knockout Microscopy, Electron, Transmission Motor Activity - genetics Myelin Basic Protein - metabolism Myelin Sheath - metabolism Myelin Sheath - pathology Myelin Sheath - ultrastructure Neural Conduction - physiology Neural Conduction - radiation effects Peripheral Nerves - metabolism Peripheral Nerves - pathology Peripheral Nerves - ultrastructure Rotarod Performance Test Sciatic Nerve - physiopathology Spinal Cord - metabolism Spinal Cord - pathology Spinal Cord - ultrastructure Sulfoglycosphingolipids - metabolism
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