Hypomyelinating leukodystrophy-associated mutation of RARS leads it to the lysosome, inhibiting oligodendroglial morphological differentiation
Pelizaeus-Merzbacher disease (PMD) is a central nervous system (CNS) demyelinating disease in human, currently known as prototypic hypomyelinating leukodystrophy 1 (HLD1). The gene responsible for HLD1 encodes proteolipid protein 1 (PLP1), which is the major myelin protein produced by oligodendrocyt...
Main Authors: | , , , , , , , , , , |
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Format: | Article |
Language: | English |
Published: |
Elsevier
2019-12-01
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Series: | Biochemistry and Biophysics Reports |
Online Access: | http://www.sciencedirect.com/science/article/pii/S2405580819300342 |