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Takikita S, Takano T, Narita T, et al. Increased apoptosis and hypomyelination in cerebral white matter of macular mutant mouse brain. Mol Genet Metab Rep. 2015;4:25-9doi: 10.1016/j.ymgmr.2015.05.005.
Takikita, S., Takano, T., Narita, T., & Maruo, Y. (2015). Increased apoptosis and hypomyelination in cerebral white matter of macular mutant mouse brain. Molecular genetics and metabolism reports, 425-9. https://doi.org/10.1016/j.ymgmr.2015.05.005
Takikita, Shoichi, et al. "Increased apoptosis and hypomyelination in cerebral white matter of macular mutant mouse brain." Molecular genetics and metabolism reports vol. 4 (2015): 25-9. doi: https://doi.org/10.1016/j.ymgmr.2015.05.005
Takikita S, Takano T, Narita T, Maruo Y. Increased apoptosis and hypomyelination in cerebral white matter of macular mutant mouse brain. Mol Genet Metab Rep. 2015 Jun 09;4:25-9. doi: 10.1016/j.ymgmr.2015.05.005. eCollection 2015 Sep. PMID: 26937406; PMCID: PMC4750634.
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Mol Genet Metab Rep. 2015 Jun 09;4:25-9. doi: 10.1016/j.ymgmr.2015.05.005. eCollection 2015 Sep.

Increased apoptosis and hypomyelination in cerebral white matter of macular mutant mouse brain.

Molecular genetics and metabolism reports

Shoichi Takikita, Tomoyuki Takano, Tsutomu Narita, Yoshihiro Maruo

Affiliations

  1. Department of Pediatrics, Takatsuki Red Cross Hospital, Takatsuki 569-1096, Japan.
  2. Department of Pediatrics, Shiga University of Medical Science, Otsu 520-2192, Japan.

PMID: 26937406 PMCID: PMC4750634 DOI: 10.1016/j.ymgmr.2015.05.005

Abstract

Hypomyelination in developing brain is often accompanied by congenital metabolic disorders. Menkes kinky hair disease is an X-linked neurodegenerative disease of impaired copper transport, resulting from a mutation of the Menkes disease gene, a transmembrane copper-transporting p-type ATPase gene (ATP7A). In a macular mutant mouse model, the murine ortholog of Menkes gene (mottled gene) is mutated, and widespread neurodegeneration and subsequent death are observed. Although some biochemical analysis of myelin protein in macular mouse has been reported, detailed histological study of myelination in this mouse model is currently lacking. Since myelin abnormality is one of the neuropathologic findings of human Menkes disease, in this study early myelination in macular mouse brain was evaluated by immunohistochemistry. Two-week-old macular mice and normal littermates were perfused with 4% paraformaldehyde. Immunohistochemical staining of paraffin embedded and vibratome sections was performed using antibodies against either CNPase, cleaved caspase-3 or O4 (marker of immature oligodendrocytes). This staining showed that cerebral myelination in macular mouse was generally hypoplastic and that hypomyelination was remarkable in internal capsule, corpus callosum, and cingulate cortex. In addition, an increased number of cleaved caspase-3 positive cells were observed in corpus callosum and internal capsule. Copper deficiency induced by low copper diet has been reported to induce oligodendrocyte dysfunction and leads to hypomyelination in this mouse model. Taken together, hypomyelination observed in this study in a mouse model of Menkes disease is assumed to be induced by increased apoptosis of immature oligodendrocytes in developing cerebrum, through deficient intracellular copper metabolism.

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