NDLI: Severe Convulsions and Dysmyelination in Both Jimpy and Cx32/47 $^{−/−}$ Mice may Associate Astrocytic L-Channel Function with Myelination and Oligodendrocytic Connexins with Internodal K$

Content Provider	Springer Nature Link
Author	Chaban, Y. H. Gerald Chen, Ye Hertz, Elna Hertz, Leif
Copyright Year	2017
Abstract	The Jimpy mouse illustrates the importance of interactions between astrocytes and oligodendrocytes. It has a mutation in Plp coding for proteolipid protein and DM20. Its behavior is normal at birth but from the age of ~2 weeks it shows severe convulsions associated with oligodendrocyte/myelination deficits and early death. A normally occurring increase in oxygen consumption by highly elevated K$^{+}$ concentrations is absent in Jimpy brain slices and cultured astrocytes, reflecting that Plp at early embryonic stages affects common precursors as also shown by the ability of conditioned medium from normal astrocytes to counteract histological abnormalities. This metabolic response is now known to reflect opening of L-channels for Ca$^{2+}$. The resulting deficiency in Ca$^{2+}$ entry has many consequences, including lack of K$^{+}$-stimulated glycogenolysis and release of gliotransmitter ATP. Lack of purinergic stimulation compromises oligodendrocyte survival and myelination and affects connexins and K$^{+}$ channels. Mice lacking the oligodendrocytic connexins Cx32 and 47 show similar neurological dysfunction as Jimpy. This possibly reflects that K$^{+}$ released by intermodal axonal K$_{v}$ channels is transported underneath a loosened myelin sheath instead of reaching the extracellular space via connexin-mediated transport to oligodendrocytes, followed by release and astrocytic Na$^{+}$,K$^{+}$-ATPase-driven uptake with subsequent Kir4.1-facilitated release and neuronal uptake.
Starting Page	1747
Ending Page	1766
Page Count	20
File Format	PDF
ISSN	03643190
Journal	Neurochemical Research
Volume Number	42
Issue Number	6
e-ISSN	15736903
Language	English
Publisher	Springer US
Publisher Date	2017-02-18
Publisher Place	New York
Access Restriction	One Nation One Subscription (ONOS)
Subject Keyword	Astrocytic L-channels for Ca$^{2+}$ Astrocytic-oligodendrocytic interaction Cx32/47$^{−/−}$ convulsions/dysmyelination Dysmyelination Glial connexins Gliotransmitter ATP Jimpy Leukemia inhibitory factor Proteolipid protein White matter K$^{+}$ homeostasis Neurosciences Neurochemistry Biochemistry Cell Biology Neurology
Content Type	Text
Resource Type	Article
Subject	Medicine Biochemistry Cellular and Molecular Neuroscience

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Cx29, cx32, cx47: implications from normal and connexin32 knockout mice.

A Combination of PLP and DM20 Transgenes Promotes Partial Myelination in the Jimpy Mouse

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Severe Convulsions and Dysmyelination in Both Jimpy and Cx32/47 $^{−/−}$ Mice may Associate Astrocytic L-Channel Function with Myelination and Oligodendrocytic Connexins with Internodal K$_{v}$ Channels

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