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Late‐onset neurodegeneration in mice with increased dosage of the proteolipid protein gene
TLDR
It is demonstrated that a low‐level increase in Plp gene expression in transgenic mice causes significant axonal degeneration and demyelination with predilection for specific tracts, relevant to the pathogenesis of PLP‐associated disorders and probably to other myelin‐related diseases. Expand
Expression of the dm-20 isoform of the plp gene in olfactory nerve ensheathing cells: evidence from developmental studies
TLDR
Developmental expression of the two plp gene isoforms, plp and dm-20, and their products in the mouse olfactory bulb are described and a role for DM-20 other than as a structural myelin protein is implied. Expand
Expression of myelin protein genes in Schwann cells
TLDR
In addition to providing data on the cellular expression of myelin protein genes, these studies have shown that teased fibres are invaluable in allowing the localization of low abundance mRNAs. Expand
LECITHIN FATTY ACID COMPOSITION IN BILE AND PLASMA OF MAN, DOGS, RATS, AND OXEN.
TLDR
The lecithins of bile and plasma from nine patients, three dogs, four rats, and two steers were extracted and their fatty acid patterns were determined, indicating either the presence of two functionally distinct pools of leCithin in the liver, or selection from a single heterogeneous pool. Expand
The effect of bile salts upon lecithin synthesis.
TLDR
The data suggest that there is one site within the liver for lecithin synthesis which provides this leCithin for bile, liver, and plasma, and that bile salts stimulated lecithsin synthesis within this common pool which, in turn, raised the specific activities in hepatic, Plasma, and biliary lecitins. Expand
Studies of red cell stromal proteins in Tay-Sachs disease.
TLDR
The observations suggest that the red cell membrane in patients with Tay-Sachs disease contains a significant excess of a glycoprotein or proteins, as compared with normal, and that the metabolic defect in this disease, therefore, affects glycoproteins as well as complex lipids. Expand
Primary demyelination induced by exposure to tellurium alters Schwann cell gene expression: a model for intracellular targeting of NGF receptor
TLDR
The results demonstrate that demyelination is associated with reversion of the affected Schwann cells to a precursor cell phenotype, and suggest that these changes in Schwann cell gene expression do not require input from a degenerating axon, but instead may depend on whether concerted synthesis of myelin is occurring. Expand
STUDIES OF RED-CELL STROMAL LIPIDS IN TAY-SACHS DISEASE AND OTHER LIPIDOSES.
In vitro studies of axonally-regulated Schwann cell genes during Wallerian degeneration
TLDR
The results indicate that Ca2+ may play a role in the expression of the nerve growth factor receptor gene during Wallerian degeneration and provide some indication that this effect may be directly on the Schwann cell rather than operating indirectly via the axon. Expand
Evidence that some oligodendrocyte progenitors in the developing optic pathway express the plp gene.
TLDR
Evidence is presented that increase in expression of the plp gene along the O-2A lineage differentiation is not progressive but that downregulation at the proligodendroblast (O4+/O1-) stage probably occurs and it is suggested that progenitors express the dm-20 isoform while oligodendedrocytes express predominantly the plP isoform. Expand
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