Impaired maturation and differentiation of oligodendrocytes in the vacuole formation myelin mutant rat.

Λεπτομέρειες βιβλιογραφικής εγγραφής
Τίτλος:	Impaired maturation and differentiation of oligodendrocytes in the vacuole formation myelin mutant rat.
Συγγραφείς:	Tanaka M; Osaka Metropolitan University, Izumisano, Japan.; Kyoto University, Kyoto, Japan., Morita C; Osaka Metropolitan University, Izumisano, Japan., Izawa T; Osaka Metropolitan University, Izumisano, Japan., Yamate J; Osaka Metropolitan University, Izumisano, Japan., Franklin RJM; University of Cambridge, Addenbrookes Hospital, Cambridge, UK., Kuramoto T; Kyoto University, Kyoto, Japan.; Tokyo University of Agriculture, Atsugi, Japan., Serikawa T; Kyoto University, Kyoto, Japan., Kuwamura M; Osaka Metropolitan University, Izumisano, Japan.
Πηγή:	Veterinary pathology [Vet Pathol] 2026 Mar; Vol. 63 (2), pp. 315-324. Date of Electronic Publication: 2025 Oct 09.
Τύπος έκδοσης:	Journal Article
Γλώσσα:	English
Στοιχεία περιοδικού:	Publisher: Sage Country of Publication: United States NLM ID: 0312020 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1544-2217 (Electronic) Linking ISSN: 03009858 NLM ISO Abbreviation: Vet Pathol Subsets: MEDLINE
Imprint Name(s):	Publication: Jan. 2010- : Thousand Oaks, CA : Sage Original Publication: 1971- : Basel : New York : Karger.
Ιατρικοί όροι (MeSH):	Oligodendroglia/pathology , Oligodendroglia/metabolism , Oligodendroglia/cytology , Myelin Sheath/pathology , Myelin Sheath/genetics , Myelin Sheath/metabolism , Vacuoles/pathology , Cell Differentiation, Myelin Basic Protein/genetics ; Myelin Basic Protein/metabolism ; Spinal Cord/pathology ; Spinal Cord/metabolism ; Animals ; Rats ; Rats, Mutant Strains ; Male
Περίληψη:	The vacuole formation (VF) rat is an autosomal recessive myelin mutant characterized by generalized tremor, hypomyelination, and periaxonal VF of the central nervous system. We previously identified a nonsense mutation in the DOP1 leucine zipper-like protein A (Dop1a, also known as Dopey1) gene located on rat chromosome 8. In this study, we investigated the kinetics of oligodendrocyte progenitor cells (OPCs) and oligodendrocytes by assessing the expression patterns of transcription factors that are involved in oligodendrocyte development. The number of small, round oligodendrocytes lacking distinct cellular processes, immunolabeled with anti-adenomatous polyposis coli (APC, clone CC1) antibody, was increased in the spinal cords of the VF rats, suggesting a disrupted maturation of oligodendrocytes. In addition, the terminal differentiation and maturation of OPCs into myelinating mature oligodendrocytes may be impaired and compensatory myelination largely could fail in the VF rat. Our findings also demonstrated that the DOP1A protein is expressed in OPCs as well as mature oligodendrocytes. Finally, the intracellular trafficking of myelin basic protein (Mbp) mRNAs may be disrupted in oligodendrocytes of the VF rats. Our data suggested that DOP1A dysfunction causes impaired differentiation and maturation of oligodendrocyte-lineage cells, resulting in hypomyelination and an impaired compensatory myelination.
Competing Interests:	Declaration of Conflicting InterestsThe authors declared the following potential conflicts of interest with respect to the research, authorship, and/or publication of this article: Authors Takeshi Izawa and Mitsuru Kuwamura are members of the Editorial Board of Veterinary Pathology and have no further conflicts to declare. The authors did not take part in the peer review or decision-making process for this submission.
Contributed Indexing:	Keywords: Dop1a; hypomyelination; mutant; myelin vacuole; oligodendrocyte progenitor cells; oligodendrocytes; rats
Substance Nomenclature:	0 (Myelin Basic Protein)
Entry Date(s):	Date Created: 20251009 Date Completed: 20260209 Latest Revision: 20260209
Update Code:	20260209
DOI:	10.1177/03009858251379486
PMID:	41067867
Βάση Δεδομένων:	MEDLINE

Περιγραφή
ISSN:	1544-2217
DOI:	10.1177/03009858251379486