P‐0 Glycoprotein Overexpression Causes Congenital Hypomyelination Of Peripheral Nerves

Wrabetz, Lawrence; Feltri, M. Laura; Quattrini, Angelo; Imperiale, Daniele; Previtali, Stefano C.; D’Antonio, Maurizio; Martini, Rudolf; Yin, Xiaoju; Trapp, B. D.; Zhou, Lijuan; Chiu, Sally; Messing, Albee

doi:10.1046/j.1529-8027.2000.absjun-12.x

Cited by 53 publications

(101 citation statements)

References 53 publications

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“…Nevertheless, MPZ protein stimulation decreased after 72 h of treatment with LiCl (sevenfold stimulation). Such a discrepancy between MPZ transcript overexpression and protein decrease has already been described (12,13). We also observed that the increase in PMP22 The mean angular velocity of the whisker movements of all animals was analyzed by means of WhiskerWatcher software at DPI3, DPI8, and DPI14.…”

Section: Pharmacological Inhibition Of Gsk3β Activates Myelin Genesupporting

confidence: 68%

Lithium enhances remyelination of peripheral nerves

Makoukji

Belle

Meffre

et al. 2012

Proc. Natl. Acad. Sci. U.S.A.

102

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Glycogen synthase kinase 3β (GSK3β) inhibitors, especially the mood stabilizer lithium chloride, are also used as neuroprotective or anti-inflammatory agents. We studied the influence of LiCl on the remyelination of peripheral nerves. We showed that the treatment of adult mice with LiCl after facial nerve crush injury stimulated the expression of myelin genes, restored the myelin structure, and accelerated the recovery of whisker movements. LiCl treatment also promoted remyelination of the sciatic nerve after crush. We also demonstrated that peripheral myelin gene MPZ and PMP22 promoter activities, transcripts, and protein levels are stimulated by GSK3β inhibitors (LiCl and SB216763) in Schwann cells as well as in sciatic and facial nerves. LiCl exerts its action in Schwann cells by increasing the amount of β-catenin and provoking its nuclear localization. We showed by ChIP experiments that LiCl treatment drives β-catenin to bind to T-cell factor/lymphoid-enhancer factor response elements identified in myelin genes. Taken together, our findings open perspectives in the treatment of nerve demyelination by administering GSK3β inhibitors such as lithium.Wnt/β-catenin | neuropathy

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Section: Pharmacological Inhibition Of Gsk3β Activates Myelin Genesupporting

confidence: 68%

Lithium enhances remyelination of peripheral nerves

Makoukji

Belle

Meffre

et al. 2012

Proc. Natl. Acad. Sci. U.S.A.

102

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“…B. Jeng, unpublished observations). Wrabetz et al (2000) have reported a similar experience with transgenic lines that overexpress P 0 ; there is an "overexpression phenotype" that is partially ameliorated by breeding the transgene into a Mpz-null background.…”

Section: Effects Of Cx32 Overexpressionmentioning

confidence: 88%

Transgenic Expression of HumanConnexin32in Myelinating Schwann Cells Prevents Demyelination inConnexin32-Null Mice

Scherer¹,

Xu²,

Messing³

et al. 2005

J. Neurosci.

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Mutations in Gap Junction ␤1 (GJB1), the gene encoding the gap junction protein connexin32 (Cx32), cause the X-linked form of Charcot-Marie-Tooth disease (CMT1X), an inherited demyelinating neuropathy. We investigated the possibility that the expression of mutant Cx32 in other cells besides myelinating Schwann cells contributes to the development of demyelination. Human Cx32 was expressed in transgenic mice using a rat myelin protein zero (Mpz) promoter, which is exclusively expressed by myelinating Schwann cells. Male mice expressing the human transgene were crossed with female Gjb1/cx32-null mice; the resulting male offspring were all cx32-null (on the X chromosome), and one-half were transgene positive. In these transgenic mice, all of the Cx32 was derived from the expression of the transgene and was found in the sciatic nerve but not in the spinal cord or the liver. Furthermore, the Cx32 protein was properly localized (within incisures and paranodes) in myelinating Schwann cells. Finally, the expression of human Cx32 protein "rescued" the phenotype of cx32-null mice, because the transgenic mice have significantly fewer demyelinated or remyelinated axons than their nontransgenic littermates. These results indicate that the loss of Schwann-cell-autonomous expression of Cx32 is sufficient to account for demyelination in CMT1X.

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“…Morphology on semithin and ultrathin sections of nerves were conducted as described (Wrabetz et al, 2000).…”

Section: Methodsmentioning

confidence: 99%

6 4 Integrin and Dystroglycan Cooperate to Stabilize the Myelin Sheath

Nodari¹,

Previtali²,

Dati³

et al. 2008

Journal of Neuroscience

Self Cite

103

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Schwann cells integrate signals deriving from the axon and the basal lamina to myelinate peripheral nerves. Integrin α6β4 is a laminin receptor synthesized by Schwann cells and displayed apposed to the basal lamina. α6β4 integrin expression in Schwann cells is induced by axons at the onset of myelination, and rise in adulthood. The β4 chain has a uniquely long cytoplasmic domain that interacts with intermediate filaments such as dystonin, important in peripheral myelination. Furthermore, α6β4 integrin binds peripheral myelin protein 22, whose alteration causes the most common demyelinating hereditary neuropathy. All these data suggest a role for α6β4 integrin in peripheral nerve myelination. Here we show that ablating α6β4 integrin specifically in Schwann cells of transgenic mice does not affect peripheral nerve development, myelin formation, maturation or regeneration. However, consistent with maximal expression in adult nerves, α6β4 integrin-null myelin is more prone to abnormal folding with aging. When the laminin receptor dystroglycan is also ablated, major folding abnormalities occur, associated with acute demyelination in some peripheral nervous system districts. These data indicate that, similar to its role in skin, α6β4 integrin confers stability to myelin in peripheral nerves.

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P‐0 Glycoprotein Overexpression Causes Congenital Hypomyelination Of Peripheral Nerves

Cited by 53 publications

References 53 publications

Lithium enhances remyelination of peripheral nerves

Lithium enhances remyelination of peripheral nerves

Transgenic Expression of HumanConnexin32in Myelinating Schwann Cells Prevents Demyelination inConnexin32-Null Mice

6 4 Integrin and Dystroglycan Cooperate to Stabilize the Myelin Sheath

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