Current concepts of PLP and its role in the nervous system
I Griffiths, M Klugmann, TAC Thomson… - Microscopy research …, 1998 - Wiley Online Library
Proteolipid protein (PLP) and its smaller isoform DM20 constitute the major myelin proteins
of the CNS. Mutations of the X‐linked Plp gene cause the heterogeneous syndromes of …
of the CNS. Mutations of the X‐linked Plp gene cause the heterogeneous syndromes of …
The proteolipid protein gene and myelin disorders in man and animal models
The two proteins, proteolipid protein and DM20, which are encoded by alternative transcripts
from the proteolipid protein (PLP) gene, are major components of central nervous system …
from the proteolipid protein (PLP) gene, are major components of central nervous system …
A cellular mechanism governing the severity of Pelizaeus–Merzbacher disease
A Gow, RA Lazzarini - Nature genetics, 1996 - nature.com
Pelizaeus–Merzbacher disease (PMD) is a leukodystrophy linked to the proteolipid protein
gene (PLP). We report a cellular basis for the distinction between two disease subtypes …
gene (PLP). We report a cellular basis for the distinction between two disease subtypes …
Disrupted proteolipid protein trafficking results in oligodendrocyte apoptosis in an animal model of Pelizaeus-Merzbacher disease
A Gow, CM Southwood, RA Lazzarini - The Journal of cell biology, 1998 - rupress.org
Pelizaeus-Merzbacher disease (PMD) is a dysmyelinating disease resulting from mutations,
deletions, or duplications of the proteolipid protein (PLP) gene. Distinguishing features of …
deletions, or duplications of the proteolipid protein (PLP) gene. Distinguishing features of …
Monoclonal antibody O10 defines a conformationally sensitive cell-surface epitope of proteolipid protein (PLP): evidence that PLP misfolding underlies dysmyelination …
M Jung, I Sommer, M Schachner… - Journal of …, 1996 - Soc Neuroscience
Mutations in the gene for proteolipid protein (PLP) have been associated with CNS
dysmyelination and abnormal oligodendrocyte death in spontaneous mouse mutants and in …
dysmyelination and abnormal oligodendrocyte death in spontaneous mouse mutants and in …
Late‐onset neurodegeneration in mice with increased dosage of the proteolipid protein gene
TJ Anderson, A Schneider, JA Barrie… - Journal of …, 1998 - Wiley Online Library
Mutations of the proteolipid protein (Plp) gene cause a generalized central nervous system
(CNS) myelin deficit in Pelizaeus‐Merzbacher disease of man and various tremor …
(CNS) myelin deficit in Pelizaeus‐Merzbacher disease of man and various tremor …
Sp1 as a target site for metal-induced perturbations of transcriptional regulation of developmental brain gene expression
NH Zawia, R Sharan, M Brydie, T Oyama… - Developmental Brain …, 1998 - Elsevier
Differential gene expression is partially regulated by zinc finger proteins (ZFP) such as Sp1,
which may be potential targets for perturbations by environmental metals. In this paper, we …
which may be potential targets for perturbations by environmental metals. In this paper, we …
The evolution of lipophilin genes from invertebrates to tetrapods: DM-20 cannot replace proteolipid protein in CNS myelin
B Stecca, CM Southwood, A Gragerov… - Journal of …, 2000 - Soc Neuroscience
The proteolipid protein (PLP) gene encodes two myelin-specific protein isoforms, DM-20
and PLP, which are members of the highly conserved lipophilin family of transmembrane …
and PLP, which are members of the highly conserved lipophilin family of transmembrane …
Myelin proteolipid proteins promote the interaction of oligodendrocytes and axons
DA Yool, M Klugmann, M McLaughlin… - Journal of …, 2001 - Wiley Online Library
Although proteolipid protein (PLP) and its DM20 isoform are the major membrane proteins of
CNS myelin, their absence causes surprisingly few developmental defects. In comparison …
CNS myelin, their absence causes surprisingly few developmental defects. In comparison …
Overexpression of CHOP in myelinating cells does not confer a significant phenotype under normal or metabolic stress conditions
CM Southwood, B Fykkolodziej… - Journal of …, 2016 - Soc Neuroscience
The PKR-like endoplasmic reticulum kinase (PERK) pathway of the unfolded protein
response (UPR) is protective against toxic accumulations of misfolded proteins in the …
response (UPR) is protective against toxic accumulations of misfolded proteins in the …