L-cycloserine slows the clinical and pathological course in mice with globoid cell leukodystrophy (twitcher mice)

Prevention of Lysosomal Storage in Tay-Sachs Mice Treated with N -Butyldeoxynojirimycin

1997 • 339 citations

Hematopoietic Stem-Cell Transplantation in Globoid-Cell Leukodystrophy

1998 • 317 citations

Delayed symptom onset and increased life expectancy in Sandhoff disease mice treated with N -butyldeoxynojirimycin

1999 • 263 citations

The twitcher mouse: an enzymatically authentic model of human globoid cell leukodystrophy (Krabbe disease)

1980 • 251 citations

Neurological Mutations Affecting Myelination

1981 • 242 citations

HEREDITARY LEUCODYSTROPHY IN THE MOUSE: THE NEW MUTANT TWITCHER

1980 • 236 citations

Myelin Galactolipids Are Essential for Proper Node of Ranvier Formation in the CNS

1998 • 221 citations

Molecular Cloning and Expression of cDNA for Murine Galactocerebrosidase and Mutation Analysis of the Twitcher Mouse, a Model of Krabbe's Disease

1996 • 187 citations

The turnover of myelin in the adult rat

1968 • 149 citations

Absence of MHC class ii molecules reduces CNS demyelination, microglial/macrophage infiltration, and twitching in murine globoid cell leukodystrophy

1994 • 112 citations

Inhibition of Sphingolipid Synthesis by Cycloserine In Vitro and In Vivo

1984 • 110 citations

Effect of bone marrow transplantation on enzyme levels and clinical course in the neurologically affected twitcher mouse.

1988 • 93 citations

IL-6 and TNFα expression in brains of twitcher, quaking and normal mice

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The turnover of myelin phospholipids in the adult and developing rat brain

1971 • 78 citations

Discussion

1998 • 74 citations

Demyelination and altered expression of myelin-associated glycoprotein isoforms in the central nervous system of galactolipid-deficient mice

1998 • 65 citations

Synthesis and turnover of cerebroside sulfate of myelin in adult and developing rat brain

1974 • 61 citations

Spacio-temporal progression of demyelination in twitcher mouse: with clinico-pathological correlation

1994 • 59 citations

Galactolipids in the formation and function of the myelin sheath

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IL-6 Deficiency Causes Enhanced Pathology in Twitcher (Globoid Cell Leukodystrophy) Mice

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Late onset globoid cell leukodystrophy (Krabbe's disease)– Swedish case with 15 years of follow‐up

1995 • 25 citations

Clinical and biochemical heterogeneity of globoid cell leukodystrophy

1981 • 20 citations

The long-term administration ofl-cycloserine to mice: Specific reduction of cerebroside level

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Cycloserine‐induced decrease of cerebroside in myelin

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A case of late variant form of infantile krabbe disease with a partial deficiency of galactocerebrosidase

1988 • 8 citations