Literature DB >> 5463179

Tubules of globoid leukodystrophy: a right-handed helix.

E J Yunis, R E Lee.   

Abstract

Morphologic similarity between the cytoplasmic tubules of globoid leukodystrophy and Gaucher's disease (as demonstrated by thin sectioning, negative staining, and shadowing techniques) and their resemblance to negatively stained beef cerebroside are presented as evidence favoring the accumulation of cerebrosides in globoid cells. The tubules of globoid leukodystrophy have a 60-angstrom periodic banding similar to the tubules of Gaucher's disease. A right-handed helical twisting of the tubules is observed in both diseases.

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Year:  1970        PMID: 5463179     DOI: 10.1126/science.169.3940.64

Source DB:  PubMed          Journal:  Science        ISSN: 0036-8075            Impact factor:   47.728


  14 in total

1.  Krabbe's disease. A light and electron microscopic study.

Authors:  S S Schochet; W F McCormick; G F Powell
Journal:  Acta Neuropathol       Date:  1976-10-15       Impact factor: 17.088

Review 2.  Twenty five years of the "psychosine hypothesis": a personal perspective of its history and present status.

Authors:  K Suzuki
Journal:  Neurochem Res       Date:  1998-03       Impact factor: 3.996

3.  Globoid cell leukodystrophy in the bluetick hound dog. I. Clinical manifestations.

Authors:  B G Boysen; L Tryphonas; N W Harries
Journal:  Can Vet J       Date:  1974-11       Impact factor: 1.008

4.  Globoid cell (Krabbe's) leukodystrophy: heterozygote detection in cultured skin fibroblasts.

Authors:  D F Farrell; A K Percy; M M Kaback; G M McKhann
Journal:  Am J Hum Genet       Date:  1973-11       Impact factor: 11.025

5.  Modulation of nanotube formation by structural modifications of sphingolipids.

Authors:  V S Kulkarni; J M Boggs; R E Brown
Journal:  Biophys J       Date:  1999-07       Impact factor: 4.033

6.  Structure of core domain of fibril-forming PHF/Tau fragments.

Authors:  Hideyo Inouye; Deepak Sharma; Warren J Goux; Daniel A Kirschner
Journal:  Biophys J       Date:  2005-12-09       Impact factor: 4.033

7.  Macrophages counteract demyelination in a mouse model of globoid cell leukodystrophy.

Authors:  Yoichi Kondo; Jessica M Adams; Marie T Vanier; Ian D Duncan
Journal:  J Neurosci       Date:  2011-03-09       Impact factor: 6.167

8.  The morphologic similarities of human and canine globoid leukodystrophy. Thin section and freeze-fracture studies.

Authors:  E J Yunis; R E Lee
Journal:  Am J Pathol       Date:  1976-10       Impact factor: 4.307

9.  Bilayer nanotubes and helical ribbons formed by hydrated galactosylceramides: acyl chain and headgroup effects.

Authors:  V S Kulkarni; W H Anderson; R E Brown
Journal:  Biophys J       Date:  1995-11       Impact factor: 4.033

10.  Macrophages Expressing GALC Improve Peripheral Krabbe Disease by a Mechanism Independent of Cross-Correction.

Authors:  Nadav I Weinstock; Daesung Shin; Narayan Dhimal; Xinying Hong; Eric E Irons; Nicholas J Silvestri; Chelsey B Reed; Duc Nguyen; Oliver Sampson; Yung-Chih Cheng; Joseph T Y Lau; Ernesto R Bongarzone; Julia Kofler; Maria L Escolar; Michael H Gelb; Lawrence Wrabetz; M Laura Feltri
Journal:  Neuron       Date:  2020-05-05       Impact factor: 17.173

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