Correction of the Galactocerebrosidase Deficiency in Globoid Cell Leukodystrophy-Cultured Cells by SL3-3 Retroviral-Mediated Gene Transfer

1996 ◽  
Vol 218 (3) ◽  
pp. 766-771 ◽  
Author(s):  
Miguel A. Gama Sosa ◽  
Rita De Gasperi ◽  
Samir Undevia ◽  
Joseph Yeretsian ◽  
Strutha C. Rouse II ◽  
...  
Keyword(s):  
Gene Transfer ◽  
Cultured Cells ◽  
Globoid Cell Leukodystrophy ◽  
Globoid Cell

scholarly journals Identification of a Molecular Target of Psychosine and Its Role in Globoid Cell Formation

2001 ◽  
Vol 153 (2) ◽  
pp. 429-434 ◽  
Author(s):  
Dong-Soon Im ◽  
Christopher E. Heise ◽  
Tuan Nguyen ◽  
Brian F. O'Dowd ◽  
Kevin R. Lynch
Keyword(s):  
Cell Death ◽  
Cultured Cells ◽  
Cell Formation ◽  
Molecular Target ◽  
Globoid Cell Leukodystrophy ◽  
Multinuclear Cells ◽  
G Protein Coupled ◽  
Degradative Enzyme ◽  
Globoid Cell ◽  
Globoid Cells

Globoid cell leukodystrophy (GLD) is characterized histopathologically by apoptosis of oligodendrocytes, progressive demyelination, and the existence of large, multinuclear (globoid) cells derived from perivascular microglia. The glycosphingolipid, psychosine (d-galactosyl-β-1,1′ sphingosine), accumulates to micromolar levels in GLD patients who lack the degradative enzyme galactosyl ceramidase. Here we document that an orphan G protein–coupled receptor, T cell death–associated gene 8, is a specific psychosine receptor. Treatment of cultured cells expressing this receptor with psychosine or structurally related glycosphingolipids results in the formation of globoid, multinuclear cells. Our discovery of a molecular target for psychosine suggests a mechanism for the globoid cell histology characteristic of GLD, provides a tool with which to explore the disjunction of mitosis and cytokinesis in cell cultures, and provides a platform for developing a medicinal chemistry for psychosine.

scholarly journals Mechanisms of demyelination and neurodegeneration in globoid cell leukodystrophy

Glia ◽  
2021 ◽  
Author(s):  
M. Laura Feltri ◽  
Nadav I. Weinstock ◽  
Jacob Favret ◽  
Narayan Dhimal ◽  
Lawrence Wrabetz ◽  
...  
Keyword(s):  
Globoid Cell Leukodystrophy ◽  
Globoid Cell

scholarly journals Human iPSC-based neurodevelopmental models of globoid cell leukodystrophy uncover patient- and cell type-specific disease phenotypes

2021 ◽  
Author(s):  
Elisabeth Mangiameli ◽  
Anna Cecchele ◽  
Francesco Morena ◽  
Francesca Sanvito ◽  
Vittoria Matafora ◽  
...  
Keyword(s):  
Globoid Cell Leukodystrophy ◽  
Cell Type ◽  
Disease Phenotypes ◽  
Specific Disease ◽  
Cell Type Specific ◽  
Human Ipsc ◽  
Globoid Cell

scholarly journals The protective effect of cardiac gene transfer of CuZn–sod in comparison with the cardioprotector monohydroxyethylrutoside against doxorubicin-induced cardiotoxicity in cultured cells

2003 ◽  
Vol 10 (4) ◽  
pp. 270-277 ◽  
Author(s):  
M A I Abou El Hassan ◽  
M Heijn ◽  
M J W E Rabelink ◽  
W J F van der Vijgh ◽  
A Bast ◽  
...  
Keyword(s):  
Gene Transfer ◽  
Protective Effect ◽  
Cultured Cells ◽  
Cardiac Gene

scholarly journals A Novel Method for the Production of Transgenic Cloned Pigs: Electroporation-Mediated Gene Transfer to Non-Cultured Cells and Subsequent Selection with Puromycin1

2005 ◽  
Vol 72 (2) ◽  
pp. 309-315 ◽  
Author(s):  
Satoshi Watanabe ◽  
Masaki Iwamoto ◽  
Shun-ichi Suzuki ◽  
Daiichiro Fuchimoto ◽  
Daisuke Honma ◽  
...  
Keyword(s):  
Gene Transfer ◽  
Cultured Cells ◽  
Novel Method ◽  
Subsequent Selection
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