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 …

Cell death of oligodendrocytes or demyelination induced by overexpression of proteolipid protein depending on expressed gene dosage

Y Inoue, T Kagawa, Y Matsumura, K Ikenaka… - Neuroscience …, 1996 - Elsevier
The transgenic mice, which were produced by introducing the wild type proteolipid protein
(PLP) gene, revealed different neurological symptoms depending on expressed gene …

Transgenic and natural mouse models of proteolipid protein (PLP)‐related dysmyelination and demyelination

IR Griffiths, A Schneider, J Anderson… - Brain …, 1995 - Wiley Online Library
The X chromosome‐linked PLP/DM‐20 gene is the CNS myelin gene most frequently
associated with mutations, resulting in dysmyelination in several species including man …

Premature arrest of myelin formation in transgenic mice with increased proteolipid protein gene dosage

C Readhead, A Schneider, I Griffiths, KA Nave - Neuron, 1994 - Elsevier
Proteolipid protein (PLP) is an integral membrane protein of CNS myelin. Mutations of the X
chromosome-linked PLP gene cause glial cell death and myelin deficiency in jimpy mice …

Phenotypic severity of murine Plp mutants reflects in vivo and in vitro variatioans in transport of PLP isoproteins

CE Thomson, P Montague, M Jung, KA Nave… - Glia, 1997 - Wiley Online Library
Mutations of the major myelin gene, proteolipid protein (Plp), cause Pelizaeus‐Merzbacher
disease and some forms of spastic paraplegia in man and dysmyelinating phenotypes in …

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 …

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 …

Different proteolipid protein mutants exhibit unique metabolic defects

M Hüttemann, Z Zhang, C Mullins, D Bessert… - ASN …, 2009 - journals.sagepub.com
PMD (Pelizaeus–Merzbacher disease), a CNS (central nervous system) disease
characterized by shortened lifespan and severe neural dysfunction, is caused by mutations …

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 …

Transgenic Lewis rats overexpressing the proteolipid protein gene: myelin degeneration and its effect on T cell-mediated experimental autoimmune encephalomyelitis

M Bradl, J Bauer, T Inomata, J Zielasek, KA Nave… - Acta …, 1999 - Springer
Transgenic Lewis rats overexpressing proteolipid protein (PLP) genes in peripheral and
central nervous myelin were produced by microinjecting murine genomic PLP sequences …