Impaired myelination and reduced brain ferric iron in the mouse model of mucolipidosis IV

Impaired myelination and reduced brain ferric iron in the mouse model of mucolipidosis IV

Mucolipidosis type IV (MLIV) is a lysosomal storage disease caused by mutations in the MCOLN1 gene, which encodes the lysosomal transient receptor potential ion channel mucolipin-1 (TRPML1). MLIV causes impaired motor and cognitive development, progressive loss of vision and gastric achlorhydria. Ho...

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Bibliographic Details
Main Authors: Yulia Grishchuk, Karina A. Peña, Jessica Coblentz, Victoria E. King, Daniel M. Humphrey, Shirley L. Wang, Kirill I. Kiselyov, Susan A. Slaugenhaupt
Format: Article
Language:English
Published: The Company of Biologists 2015-12-01
Series:Disease Models & Mechanisms
Subjects:
Lysosome
Myelination
Oligodendrocytes
Transient receptor potential channel mucolipin-1
Online Access:http://dmm.biologists.org/content/8/12/1591

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