glycogen has been researched along with Leukodystrophy--Metachromatic* in 4 studies
2 review(s) available for glycogen and Leukodystrophy--Metachromatic
Article | Year |
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[Animal models for inborn lysosomal storage diseases (author's transl)].
Topics: Animals; Cats; Cattle; Disease Models, Animal; Dogs; G(M2) Ganglioside; Gangliosidoses; Gaucher Disease; Glycogen; Glycogen Storage Disease Type II; Glycopeptides; Humans; Leukodystrophy, Globoid Cell; Leukodystrophy, Metachromatic; Lipidoses; Lysosomes; Mannosidases; Metabolism, Inborn Errors; Mice; Niemann-Pick Diseases; Rabbits; Sphingolipids | 1977 |
Treatment related observations in solid tissues, fibroblast cultured and amniotic fluid cells of type II glycogenosis, Hurler disease and metachromatic leukodystrophy.
Topics: Blood Transfusion; Cells, Cultured; Child, Preschool; Cytoplasm; Epinephrine; Female; Fibroblasts; Glucosidases; Glycogen; Glycogen Storage Disease; Humans; Infant; Leukodystrophy, Metachromatic; Liver; Lysosomes; Male; Microscopy, Electron; Mucopolysaccharidosis I; Mucopolysaccharidosis II; Prenatal Diagnosis; Skin; Sulfatases | 1973 |
2 other study(ies) available for glycogen and Leukodystrophy--Metachromatic
Article | Year |
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Ultrastructural findings of peripheral nerve in a preclinical case of adult metachromatic leukodystrophy.
In a 13-year-old neurologically healthy boy from a family with adult-onset of metachromatic leukodystrophy (MLD) showing arylsulfatase A-deficiency in the adult, sural nerve biopsy probably was performed 2-3 decades before clinical manifestation of the disease could be expected. Ultrastructurally 4 basic types of inclusion bodies in Schwann cells could be demonstrated (pleo-morphic "zebra body"-like inclusions, double-lamellated inclusions, "tuff-stone"-like inclusions, granular osmiophilic inclusions). Additionally, endoplasmatic reticulum, mitochondria and lysosomes showed marked alterations. Advanced damage of myelin was only rarely seen, but initial segmental demyelination was a common finding. These early pathological changes in chronic MLD are thought to represent a subcellular metabolic insufficiency of Schwann cells in this disease. Topics: Adolescent; Cytoplasmic Granules; Endoplasmic Reticulum; Glycogen; Humans; Inclusion Bodies; Leukodystrophy, Metachromatic; Lipofuscin; Male; Microscopy, Electron; Mitochondria; Myelin Sheath; Peripheral Nerves; Schwann Cells; Sulfoglycosphingolipids | 1977 |
[Histological, histo-enzymatic and quantitative studies on normal and pathological skeletal muscles].
Topics: Adult; Aphonia; Child; Child, Preschool; Chronaxy; Citric Acid Cycle; Demyelinating Diseases; Energy Metabolism; Female; Glycogen; Histocytochemistry; Humans; Infant; Leukodystrophy, Metachromatic; Male; Middle Aged; Muscles; Muscular Atrophy; Nerve Degeneration; Syndrome | 1971 |