[HTML][HTML] Endoplasmic reticulum protein quality control failure in myelin disorders
VG Volpi, T Touvier, M D'Antonio - Frontiers in molecular …, 2017 - frontiersin.org
Reaching the correct three-dimensional structure is crucial for the proper function of a
protein. The endoplasmic reticulum (ER) is the organelle where secreted and …
protein. The endoplasmic reticulum (ER) is the organelle where secreted and …
Neurogenetics of Pelizaeus–Merzbacher disease
MJ Osório, SA Goldman - Handbook of clinical neurology, 2018 - Elsevier
Pelizaeus–Merzbacher disease (PMD) is an X-linked disorder caused by mutations in the
PLP1 gene, which encodes the proteolipid protein of myelinating oligodendroglia. PMD …
PLP1 gene, which encodes the proteolipid protein of myelinating oligodendroglia. PMD …
The molecular and cellular defects underlying Pelizaeus–Merzbacher disease
KJ Woodward - Expert reviews in molecular medicine, 2008 - cambridge.org
Pelizaeus–Merzbacher disease (PMD) is a recessive X-linked dysmyelinating disorder of
the central nervous system (CNS). The most frequent cause of PMD is a genomic duplication …
the central nervous system (CNS). The most frequent cause of PMD is a genomic duplication …
Perturbed interactions of mutant proteolipid protein/DM20 with cholesterol and lipid rafts in oligodendroglia: implications for dysmyelination in spastic paraplegia
EM Krämer-Albers, K Gehrig-Burger… - Journal of …, 2006 - Soc Neuroscience
Missense mutations in the human PLP1 gene lead to dysmyelinating diseases with a broad
range of clinical severity, ranging from severe Pelizaeus–Merzbacher disease (PMD) to …
range of clinical severity, ranging from severe Pelizaeus–Merzbacher disease (PMD) to …
Myelinated, synapsing cultures of murine spinal cord – validation as an in vitro model of the central nervous system
CE Thomson, M McCulloch, A Sorenson… - European Journal of …, 2008 - Wiley Online Library
Research in central nervous system (CNS) biology and pathology requires in vitro models,
which, to recapitulate the CNS in vivo, must have extensive myelin and synapse formation …
which, to recapitulate the CNS in vivo, must have extensive myelin and synapse formation …
PLP overexpression perturbs myelin protein composition and myelination in a mouse model of Pelizaeus‐Merzbacher disease
SA Karim, JA Barrie, MC McCulloch, P Montague… - Glia, 2007 - Wiley Online Library
Duplication of PLP1, an X‐linked gene encoding the major myelin membrane protein of the
human CNS, is the most frequent cause of Pelizaeus‐Merzbacher disease (PMD) …
human CNS, is the most frequent cause of Pelizaeus‐Merzbacher disease (PMD) …
Concise review: stem cell-based treatment of Pelizaeus-Merzbacher disease
Pelizaeus-Merzbacher disease (PMD) is an X-linked disorder caused by mutation in the
proteolipid protein-1 (PLP1) gene, which encodes the proteolipid protein of myelinating …
proteolipid protein-1 (PLP1) gene, which encodes the proteolipid protein of myelinating …
Loss of lysophosphatidic acid receptor LPA1 alters oligodendrocyte differentiation and myelination in the mouse cerebral cortex
B García-Díaz, R Riquelme, I Varela-Nieto… - Brain Structure and …, 2015 - Springer
Lysophosphatidic acid (LPA) is an intercellular signaling lipid that regulates multiple cellular
functions, acting through specific G-protein coupled receptors (LPA 1–6). Our previous …
functions, acting through specific G-protein coupled receptors (LPA 1–6). Our previous …
Axon–glial interaction in the CNS: what we have learned from mouse models of Pelizaeus–Merzbacher disease
FI Gruenenfelder, G Thomson, J Penderis… - Journal of …, 2011 - Wiley Online Library
In the central nervous system (CNS) the majority of axons are surrounded by a myelin
sheath, which is produced by oligodendrocytes. Myelin is a lipid‐rich insulating material that …
sheath, which is produced by oligodendrocytes. Myelin is a lipid‐rich insulating material that …
Rho regulates membrane transport in the endocytic pathway to control plasma membrane specialization in oligodendroglial cells
Differentiation of oligodendrocytes is associated with dramatic changes in plasma
membrane structure, culminating in the formation of myelin membrane sheaths. Previous …
membrane structure, culminating in the formation of myelin membrane sheaths. Previous …