Early evidence of delayed oligodendrocyte maturation in the mouse model of mucolipidosis type IV

Mucolipidosis type IV (MLIV) is a lysosomal disease caused by mutations in the MCOLN1 gene that encodes the endolysosomal transient receptor potential channel mucolipin-1, or TRPML1. MLIV results in developmental delay, motor and cognitive impairments, and vision loss. Brain abnormalities include th...

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Autores principales: Molly Mepyans, Livia Andrzejczuk, Jahree Sosa, Sierra Smith, Shawn Herron, Samantha DeRosa, Susan A. Slaugenhaupt, Albert Misko, Yulia Grishchuk, Kirill Kiselyov
Formato: Artigo
Lenguaje:Inglês
Publicado: The Company of Biologists 2020-07-01
Colección:Disease Models & Mechanisms
Materias:
mucolipidosis type iv
lysosome
trpml1
mucolipin-1
myelination
oligodendrocyte
Acceso en línea:http://dmm.biologists.org/content/13/7/dmm044230
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