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This report describes a new X-linked mutation in mice, named rumpshaker (rsh) which is associated with hypomyelination of the central nervous system. Myelination commences appropriately but the majority of sheaths fail to develop normally. Oligodendrocytes are increased in number and have prominent Golgi apparatus, rough endoplasmic reticulum and free ribosomes. Occasional cisternae of rough endoplasmic reticulum are distended. Some dense lamellar inclusions occur in oligodendrocytes but overall, degenerative changes and cell death are uncommon. Immunostaining demonstrates a major defect in expression of PLP DM-20. Using site-specific antisera directed at different portions of the PLP/DM-20 molecule, the major defect appears to be with PLP where virtually no myelin sheaths are positive. Antiserum against the C-terminal common to PLP and DM-20 shows reduced but definite myelin staining. Genetic analysis indicates a locus at or close to the PLP/jimpy (jp) locus. The rsh mutation, however, differs from jp in that affected mice have normal longevity, can breed, produce substantially more myelin and have increased numbers of oligodendrocytes.
I R Griffiths, and
I Scott, and
M C McCulloch, and
J A Barrie, and
K McPhilemy, and
B M Cattanach
August 1991,
Neuropathology and applied neurobiology,
I R Griffiths, and
I Scott, and
M C McCulloch, and
J A Barrie, and
K McPhilemy, and
B M Cattanach
April 2003,
Journal of neuroscience research,
I R Griffiths, and
I Scott, and
M C McCulloch, and
J A Barrie, and
K McPhilemy, and
B M Cattanach
January 1992,
Glia,
I R Griffiths, and
I Scott, and
M C McCulloch, and
J A Barrie, and
K McPhilemy, and
B M Cattanach
January 1980,
The Journal of heredity,
I R Griffiths, and
I Scott, and
M C McCulloch, and
J A Barrie, and
K McPhilemy, and
B M Cattanach
April 2004,
Acta neuropathologica,
I R Griffiths, and
I Scott, and
M C McCulloch, and
J A Barrie, and
K McPhilemy, and
B M Cattanach
May 2006,
Glia,
I R Griffiths, and
I Scott, and
M C McCulloch, and
J A Barrie, and
K McPhilemy, and
B M Cattanach
November 1997,
Journal of inherited metabolic disease,
I R Griffiths, and
I Scott, and
M C McCulloch, and
J A Barrie, and
K McPhilemy, and
B M Cattanach
January 1988,
Current topics in microbiology and immunology,
I R Griffiths, and
I Scott, and
M C McCulloch, and
J A Barrie, and
K McPhilemy, and
B M Cattanach
September 1979,
Science (New York, N.Y.),
I R Griffiths, and
I Scott, and
M C McCulloch, and
J A Barrie, and
K McPhilemy, and
B M Cattanach
October 1989,
Proceedings of the National Academy of Sciences of the United States of America,
