Abstract
METACHROMATIC leucodystrophy (MLD) is a progressive neurodegenerative disorder inherited in an autosomal recessive fashion and characterized by the deposition of cerebroside sulphate in brain, peripheral nerves and visceral organs1–4. The storage of cerebroside sulphate is due to a deficiency of cerebroside sulphatase or arylsulphatase A5–7, which also acts on synthetic substrates.
This is a preview of subscription content, access via your institution
Access options
Subscribe to this journal
Receive 51 print issues and online access
$199.00 per year
only $3.90 per issue
Buy this article
- Purchase on Springer Link
- Instant access to full article PDF
Prices may be subject to local taxes which are calculated during checkout
Similar content being viewed by others
References
Von Hirsch, T., and Peiffer, J., Arch. Psych. Neurol., 194, 88 (1955).
Suzuki, K., Suzuki, K., and Chen, G., J. Neuropath. Exp. Neurol., 26, 537 (1967).
Jatzkewitz, H., Hoppe-Seyler's Z. Physiol. Chem., 311, 279 (1958).
Austin, J. H., Proc. Soc. Exp. Biol. Med., 100, 361 (1959).
Austin, J., McAfee, D., Armstrong, D., O'Rourke, M., Shearer, L., and Bachhawat, B., Biochem. J., 93, 15c (1964).
Mehl, E., and Jatzkewitz, H., Biochem. Biophys. Res. Commun., 19, 407 (1965).
Mehl, E., and Jatzkewitz, H., Biochem. Biophys. Acta, 151, 619 (1968).
Bischel, M., Austin, J., and Kemeny, M., Arch. Neurol., 15, 13 (1966).
Percy, A. K., and Brady, R. O., Science, 161, 594 (1968).
DeMars, R., and Nance, W. E., Wistar Inst. Monogr., 1, 35 (1964).
DeMars, R., Nat. Cancer Inst. Monogr., 13, 181 (1963).
Oyama, V. I., and Eagle, H., Proc. Soc. Exp. Biol. Med., 91, 305 (1956).
Tappel, A. L., Comprehensive Biochemistry, Section 5, Chemical Biology (edit. by Florkin, M., and Stotz, E. H.), 23, 77 (Elsevier, NY, 1968).
Porter, M. T., Fluharty, A. L., and Kihara, H., Proc. US Nat. Acad. Sci., 62, 887 (1969).
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
LEROY, J., DUMON, J. & RADERMECKER, J. Deficiency of Arylsulphatase A in Leucocytes and Skin Fibroblasts in Juvenile Metachromatic Leucodystrophy. Nature 226, 553–554 (1970). https://doi.org/10.1038/226553a0
Received:
Issue Date:
DOI: https://doi.org/10.1038/226553a0
This article is cited by
-
Comparative ultrastructural observations on peripheral nerve abnormalities in the late infantile, juvenile and late onset forms of metachromatic leukodystrophy
Acta Neuropathologica (1977)
-
Metachromatische Leukodystrophie
Archiv f�r Psychiatrie und Nervenkrankheiten (1974)
Comments
By submitting a comment you agree to abide by our Terms and Community Guidelines. If you find something abusive or that does not comply with our terms or guidelines please flag it as inappropriate.
