Ultrasturcture of Centeral Nervous System Lesions in Metachromatic Leukkodystrophy With Special Reference to Morphogenesis

Liu, H. Mei

doi:10.1097/00005072-196810000-00007

Cited by 35 publications

(3 citation statements)

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“…Cho ϭ choline; Cr ϭ creatine. 25,26 The observed low levels of NAA reflect this pathology. Inspection of the metabolite maps obtained from our patients revealed a marked reduction of the NAA signal in deep white matter in patients with advanced disease.…”

Section: Figure 3 White Matter Spectramentioning

confidence: 85%

Brain N -acetylaspartate levels correlate with motor function in metachromatic leukodystrophy

Dali¹,

Hanson²,

Barton³

et al. 2010

Neurology

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Section: Figure 3 White Matter Spectramentioning

confidence: 85%

Brain N -acetylaspartate levels correlate with motor function in metachromatic leukodystrophy

Dali¹,

Hanson²,

Barton³

et al. 2010

Neurology

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“…Terry, 1971) and canine (Fletcher, Lee & Hammer, 1971) globoid leukodystrophy and Gaucher's disease in which tubules with a diameter of 30-50 nm are found but the ultrastructural appearance of the inclusions has only limited differential value. The type of inclusion more commonly seen in the geese resembled the granular and vesicular bodies which have been illustrated (Aurebeck, Osterberg, Blau, Chov & Nelson, 1964;Liu, 1968) in human MLD. Liu (1968) observed that the granular bodies were sometimes surrounded by a few lamellae suggesting a transition from the former to multilaminated masses; a similar impression was obtained in the geese.…”

Section: Discussionmentioning

confidence: 71%

“…Moser, 1972). They also occur in swellings on axons where they comprise 'torpedoes' (Liu, 1968). The anserine condition was similar in all these respects.…”

Section: Discussionmentioning

confidence: 99%

THE HISTOPATHOLOGY OF A CEREBRAL LIPODOSIS IN THE HAWAIIAN GOOSE, BRANTA SANDVICENSIS

Wight

1976

Neuropathology Appl Neurobio

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The histopathology of a cerebral lipidosis in the Hawaiian goose, Brunta sandvicensis Histopathological investigation of two Hawaiian geese which had shown abnormal neurological symptoms during life, revealed a cerebral lipidosis resembling metachromatic leucodystrophy. Abnormal deposits of lipid occurred in neurons and their processes, in perivascular macrophages and oligodendroglia especially in white matter, in Schwann cells and in kidney tubules. There was astrocytic gliosis, incomplete myelination in the central nervous system and demyelination of sciatic nerves. The abnormal deposits were sudanophilic, PAS-positive, metachromatic with acidified cresyl violet, birefringent, negative or weak in their response to phospholipid stains and gave a positive histochemical reaction for sulphate esters. Thin-layer chromatographic lipid analyses suggested excess amounts of sulphatide. Ultrastructurally the deposits comprised spherical granular inclusions, often containing some laminated membranes, and larger, irregular-shaped inclusions composed of compacted lamellae. The periodicity of the lamellae was 5-6 nm. 13-28chemistry and Cytochemistry 15, 421 in the dog.

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Microtubules as Targets for Drug and Toxic Chemical Action: The Mechanisms of Action of Colchicine and Vinblastine

Wilson

1986

The Cytoskeleton

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Ultrasturcture of Centeral Nervous System Lesions in Metachromatic Leukkodystrophy With Special Reference to Morphogenesis

Cited by 35 publications

References 0 publications

Brain N -acetylaspartate levels correlate with motor function in metachromatic leukodystrophy

Brain N -acetylaspartate levels correlate with motor function in metachromatic leukodystrophy

THE HISTOPATHOLOGY OF A CEREBRAL LIPODOSIS IN THE HAWAIIAN GOOSE, BRANTA SANDVICENSIS

Microtubules as Targets for Drug and Toxic Chemical Action: The Mechanisms of Action of Colchicine and Vinblastine

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