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 …

The proteolipid protein gene and myelin disorders in man and animal models

DA Yool, JM Edgar, P Montague… - Journal of the …, 2000 - Wiley Online Library
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 …

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 …

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 …

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 …

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 …

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 …

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 …

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 …

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 …