Abstract
Jimpy is a murine mutation in myelin proteolipid protein, leading to premature death of oligodendrocytes and severe central nervous system hypomyelination. Jimpy is a bona fide model of human Pelizaeus-Merzbacher disease. This paper describes a severe reduction in expression of kappa-opioid receptors (KOP) in oligodendrocytes of jimpy mice. A cell-specific reduction of >90% is apparent by 5 days of age. Expression is not reduced in neurons, and mu-opioid receptor expression is normal. Mechanism(s) leading to deficient KOP expression in jimpy mice remain unclear. We speculate that loss of KOP may be related to increased [Ca(2+)](i) and premature death of jimpy oligodendrocytes.
Publication types
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Research Support, N.I.H., Extramural
MeSH terms
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Animals
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Calcium Signaling / genetics
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Cell Death / genetics
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Disease Models, Animal
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Down-Regulation / genetics
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Hereditary Central Nervous System Demyelinating Diseases / genetics
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Hereditary Central Nervous System Demyelinating Diseases / metabolism*
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Hereditary Central Nervous System Demyelinating Diseases / physiopathology
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Mice
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Mice, Jimpy
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Nerve Fibers, Myelinated / metabolism*
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Nerve Fibers, Myelinated / pathology
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Oligodendroglia / metabolism*
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Oligodendroglia / pathology
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Pelizaeus-Merzbacher Disease / genetics
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Pelizaeus-Merzbacher Disease / metabolism
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Pelizaeus-Merzbacher Disease / physiopathology
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Receptors, Opioid, kappa / genetics
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Receptors, Opioid, kappa / metabolism*