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Myelination of a fetus with Pelizaeus–Merzbacher disease: Immunopathological study
Author(s) -
Shiraishi Kazuhiro,
Itoh Masayuki,
Sano Kenji,
Takashima Sachio,
Kubota Takeo
Publication year - 2003
Publication title -
annals of neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 4.764
H-Index - 296
eISSN - 1531-8249
pISSN - 0364-5134
DOI - 10.1002/ana.10660
Subject(s) - proteolipid protein 1 , gene duplication , fetus , myelin , oligodendrocyte , biology , myelin proteolipid protein , immunohistochemistry , myelin basic protein , pathology , gene , medicine , immunology , endocrinology , pregnancy , genetics , central nervous system
We report an autopsied case of a 21‐gestational‐week fetus with duplication of the proteolipid protein (PLP) gene ( PLP1 ). An immunohistochemical study, which can detect the specific expression of PLP, myelin basic protein, myelin‐associated glycoprotein, and platelet‐derived growth factor receptor α subunit in brain tissues, showed that the myelination was almost the same as that of age‐matched controls. This result suggests that the development and migration of the oligodendrocyte is normal in Pelizaeus–Merzbacher disease until midgestation. To our knowledge, this is the first report of the myelination of a fetus with duplication of the PLP1 gene.