Dominant-negative action of the jimpy mutation in mice complemented with an autosomal transgene for myelin proteolipid protein.

PubWeight™: 0.83‹?›

🔗 View Article (PMC 41961)

Published in Proc Natl Acad Sci U S A on May 09, 1995

Authors

A M Schneider1, I R Griffiths, C Readhead, K A Nave

Author Affiliations

1: Zentrum für Molekulare Biologie (ZMBH), Universität Heidelberg, Germany.

Articles cited by this

Single-step method of RNA isolation by acid guanidinium thiocyanate-phenol-chloroform extraction. Anal Biochem (1987) 281.19

Transgenic mice with a rhodopsin mutation (Pro23His): a mouse model of autosomal dominant retinitis pigmentosa. Neuron (1992) 3.25

MUTANT MICE (QUAKING AND JIMPY) WITH DEFICIENT MYELINATION IN THE CENTRAL NERVOUS SYSTEM. Science (1964) 2.63

Expression of a myelin basic protein gene in transgenic shiverer mice: correction of the dysmyelinating phenotype. Cell (1987) 2.29

Nucleotide sequences of two mRNAs for rat brain myelin proteolipid protein. Cell (1985) 2.10

Splice site selection in the proteolipid protein (PLP) gene transcript and primary structure of the DM-20 protein of central nervous system myelin. Proc Natl Acad Sci U S A (1987) 1.58

Premature arrest of myelin formation in transgenic mice with increased proteolipid protein gene dosage. Neuron (1994) 1.50

Unwrapping the genes of myelin. Neuron (1988) 1.45

The structural gene coding for myelin-associated proteolipid protein is mutated in jimpy mice. Nature (1986) 1.36

Proteolipid protein (PLP) of CNS myelin: positions of free, disulfide-bonded, and fatty acid thioester-linked cysteine residues and implications for the membrane topology of PLP. Biochemistry (1992) 1.31

Glial cell degeneration and hypomyelination caused by overexpression of myelin proteolipid protein gene. Neuron (1994) 1.30

Jimpy mutant mouse: a 74-base deletion in the mRNA for myelin proteolipid protein and evidence for a primary defect in RNA splicing. Proc Natl Acad Sci U S A (1986) 1.28

Complete deletion of the proteolipid protein gene (PLP) in a family with X-linked Pelizaeus-Merzbacher disease. Am J Hum Genet (1991) 1.28

Major Myelin proteolipid: the 4-alpha-helix topology. J Membr Biol (1991) 1.23

Many naturally occurring mutations of myelin proteolipid protein impair its intracellular transport. J Neurosci Res (1994) 1.20

Oligodendroglial cell death in jimpy mice: an explanation for the myelin deficit. J Neurosci (1986) 1.13

A single nucleotide difference in the gene for myelin proteolipid protein defines the jimpy mutation in mouse. J Neurochem (1987) 1.07

Pelizaeus-Merzbacher disease: a valine to phenylalanine point mutation in a putative extracellular loop of myelin proteolipid. Proc Natl Acad Sci U S A (1991) 1.06

Neurological mouse mutants and the genes of myelin. J Neurosci Res (1994) 1.05

Structure and function of myelin protein genes. Annu Rev Neurosci (1991) 1.03

An AG----GG transition at a splice site in the myelin proteolipid protein gene in jimpy mice results in the removal of an exon. FEBS Lett (1987) 1.01

Jimpy, a new totally sexlinked gene in the house mouse. Z Indukt Abstamm Vererbungsl (1954) 0.97

Death of individual oligodendrocytes in jimpy brain precedes expression of proteolipid protein. Dev Neurosci (1990) 0.84

A combination of PLP and DM20 transgenes promotes partial myelination in the jimpy mouse. J Neurochem (1994) 0.83

Myelination in the jimpy mouse in the absence of proteolipid protein. Glia (1989) 0.82

Fate of jimpy-type oligodendrocytes in jimpy heterozygote. J Neurochem (1994) 0.79

Differences in levels of neuroglial cell death in jimpy male mice and carrier females. Dev Neurosci (1990) 0.79

Articles by these authors

The transcription factor Sox10 is a key regulator of peripheral glial development. Genes Dev (2001) 4.40

Axonal swellings and degeneration in mice lacking the major proteolipid of myelin. Science (1998) 3.58

Shaping of the autoreactive T-cell repertoire by a splice variant of self protein expressed in thymic epithelial cells. Nat Med (2000) 2.94

Expression of a myelin basic protein gene in transgenic shiverer mice: correction of the dysmyelinating phenotype. Cell (1987) 2.29

Nucleotide sequences of two mRNAs for rat brain myelin proteolipid protein. Cell (1985) 2.10

Assembly of CNS myelin in the absence of proteolipid protein. Neuron (1997) 1.93

Neuronal basic helix-loop-helix proteins (NEX and BETA2/Neuro D) regulate terminal granule cell differentiation in the hippocampus. J Neurosci (2000) 1.91

Mice deficient for the myelin-associated glycoprotein show subtle abnormalities in myelin. Neuron (1994) 1.71

Peripheral demyelination and neuropathic pain behavior in periaxin-deficient mice. Neuron (2000) 1.61

Cyclin A1 directly interacts with B-myb and cyclin A1/cdk2 phosphorylate B-myb at functionally important serine and threonine residues: tissue-specific regulation of B-myb function. Blood (2001) 1.60

Splice site selection in the proteolipid protein (PLP) gene transcript and primary structure of the DM-20 protein of central nervous system myelin. Proc Natl Acad Sci U S A (1987) 1.58

Two forms of 1B236/myelin-associated glycoprotein, a cell adhesion molecule for postnatal neural development, are produced by alternative splicing. Proc Natl Acad Sci U S A (1987) 1.57

Myelin deficient mice: expression of myelin basic protein and generation of mice with varying levels of myelin. Cell (1987) 1.55

Premature arrest of myelin formation in transgenic mice with increased proteolipid protein gene dosage. Neuron (1994) 1.50

Neuronal basic helix-loop-helix proteins (NEX, neuroD, NDRF): spatiotemporal expression and targeted disruption of the NEX gene in transgenic mice. J Neurosci (1998) 1.42

A transgenic rat model of Charcot-Marie-Tooth disease. Neuron (1996) 1.37

NEX-1: a novel brain-specific helix-loop-helix protein with autoregulation and sustained expression in mature cortical neurons. Mech Dev (1994) 1.36

Jimpy mutant mouse: a 74-base deletion in the mRNA for myelin proteolipid protein and evidence for a primary defect in RNA splicing. Proc Natl Acad Sci U S A (1986) 1.28

A myelin proteolipid protein-LacZ fusion protein is developmentally regulated and targeted to the myelin membrane in transgenic mice. J Cell Biol (1993) 1.24

Formation of compact myelin is required for maturation of the axonal cytoskeleton. J Neurosci (1999) 1.23

Monoclonal antibody O10 defines a conformationally sensitive cell-surface epitope of proteolipid protein (PLP): evidence that PLP misfolding underlies dysmyelination in mutant mice. J Neurosci (1996) 1.20

Rapid preimplantation detection of mutant (shiverer) and normal alleles of the mouse myelin basic protein gene allowing selective implantation and birth of live young. Proc Natl Acad Sci U S A (1990) 1.19

MR microscopy of transgenic mice that spontaneously acquire experimental allergic encephalomyelitis. Magn Reson Med (1998) 1.19

Identification of an embryonic isoform of myelin basic protein that is expressed widely in the mouse embryo. Proc Natl Acad Sci U S A (1993) 1.19

Current concepts of PLP and its role in the nervous system. Microsc Res Tech (1998) 1.17

Targeted inactivation of the X-linked adrenoleukodystrophy gene in mice. J Neurosci Res (1997) 1.16

Methylation of the cyclin A1 promoter correlates with gene silencing in somatic cell lines, while tissue-specific expression of cyclin A1 is methylation independent. Mol Cell Biol (2000) 1.14

Late-onset neurodegeneration in mice with increased dosage of the proteolipid protein gene. J Comp Neurol (1998) 1.10

Uncoupling of hypomyelination and glial cell death by a mutation in the proteolipid protein gene. Nature (1992) 1.10

Some aspects of the pathology and pathogenesis of the myelopathy caused by disc protrusions in the dog. J Neurol Neurosurg Psychiatry (1972) 1.09

SHARPs: mammalian enhancer-of-split- and hairy-related proteins coupled to neuronal stimulation. Mol Cell Neurosci (1997) 1.07

Spinal cord infarction due to emboli arising from the intervertebral discs in the dog. J Comp Pathol (1973) 1.07

Chronic degenerative radiculomyelopathy in the dog. J Small Anim Pract (1975) 1.06

Morphometric analysis of normal, mutant, and transgenic CNS: correlation of myelin basic protein expression to myelinogenesis. J Neurochem (1992) 1.04

Shaking pups: a disorder of central myelination in the spaniel dog. II. Ultrastructural observations on the white matter of the cervical spinal cord. J Neurocytol (1981) 1.03

Early vascular changes in the spinal grey matter following impact injury. Acta Neuropathol (1978) 1.01

Membrane traffic in myelinating oligodendrocytes. Microsc Res Tech (2001) 0.98

Myelinated, synapsing cultures of murine spinal cord--validation as an in vitro model of the central nervous system. Eur J Neurosci (2008) 0.98

Canine giant axonal neuropathy. Vet Rec (1977) 0.98

Multiple splice isoforms of proteolipid M6B in neurons and oligodendrocytes. Mol Cell Neurosci (2001) 0.95

Nerve fibres in spinal cord impact injuries. Part 1. Changes in the myelin sheath during the initial 5 weeks. J Neurol Sci (1983) 0.95

'Shaking pups': a disorder of central myelination in the spaniel dog. III. Quantitative aspects of glia and myelin in the spinal cord and optic nerve. Neuropathol Appl Neurobiol (1984) 0.95

SHARPs: mammalian enhancer-of-split- and hairy-related proteins coupled to neuronal stimulation. Mol Cell Neurosci (1997) 0.94

Development of lymphoid hyperplasia in transgenic mice expressing the HIV tat gene. AIDS Res Hum Retroviruses (1995) 0.94

Vascular permeability to protein and vasogenic oedema in experimental concussive injuries to the canine spinal cord. J Neurol Sci (1974) 0.93

A light and electron microscopic study of the neuropathy of equine idiopathic laryngeal hemiplegia. Neuropathol Appl Neurobiol (1979) 0.92

Peripheral polyneuropathies in dogs: a study of five cases. J Small Anim Pract (1977) 0.92

Spinal cord blood flow in dogs. 2. The effect of the blood gases. J Neurol Neurosurg Psychiatry (1973) 0.92

Rumpshaker: an X-linked mutation causing hypomyelination: developmental differences in myelination and glial cells between the optic nerve and spinal cord. Glia (1992) 0.92

Myelination in the absence of galactolipids and proteolipid proteins. Mol Cell Neurosci (1999) 0.91

Uncoupling of myelin assembly and schwann cell differentiation by transgenic overexpression of peripheral myelin protein 22. J Neurosci (2000) 0.91

Oligodendrocytes are not inherently programmed to myelinate a specific size of axon. J Comp Neurol (1998) 0.91

Distinct phenotypes associated with increasing dosage of the PLP gene: implications for CMT1A due to PMP22 gene duplication. Ann N Y Acad Sci (1999) 0.91

A progressive axonopathy of Boxer dogs affecting the central and peripheral nervous systems. J Small Anim Pract (1980) 0.90

Transgenic Lewis rats overexpressing the proteolipid protein gene: myelin degeneration and its effect on T cell-mediated experimental autoimmune encephalomyelitis. Acta Neuropathol (1999) 0.90

Shaking pups: a disorder of central myelination in the Spaniel dog. Part 1. Clinical, genetic and light-microscopical observations. J Neurol Sci (1981) 0.90

Inherited canine giant axonal neuropathy. Muscle Nerve (1981) 0.90

Pituitary-directed leukemia inhibitory factor transgene causes Cushing's syndrome: neuro-immune-endocrine modulation of pituitary development. Mol Endocrinol (1998) 0.90

Helix-loop-helix proteins in Schwann cells: a study of regulation and subcellular localization of Ids, REB, and E12/47 during embryonic and postnatal development. J Neurosci Res (1997) 0.89

Spinal cord compression and blood flow. I. The effect of raised cerebrospinal fluid pressure on spinal cord blood flow. Neurology (1978) 0.89

Genetic background determines phenotypic severity of the Plp rumpshaker mutation. J Neurosci Res (2003) 0.89

Age changes in the dorsal and ventral lumbar nerve roots of dogs. Acta Neuropathol (1975) 0.88

Mouse models of myelin diseases. Brain Pathol (1998) 0.88

Genetic background influences UPR but not PLP processing in the rumpshaker model of PMD/SPG2. Neurochem Res (2006) 0.87

Ultrastructural appearances of the spinal microvasculature between 12 hours and 5 days after impact injury. Acta Neuropathol (1978) 0.87

Oligodendrocyte progenitors in the embryonic spinal cord express DM-20. Neuropathol Appl Neurobiol (1996) 0.87

Central nervous system pathology in 25 dogs with chronic degenerative radiculomyelopathy. Vet Rec (2000) 0.87

Spinal cord blood flow and conduction during experimental cord compression in normotensive and hypotensive dogs. J Neurosurg (1979) 0.86

Myelin proteolipid proteins promote the interaction of oligodendrocytes and axons. J Neurosci Res (2001) 0.86

Neuromuscular disease in dogs: some aspects of its investigation and diagnosis. J Small Anim Pract (1973) 0.85

Differential expression of apoptotic markers in jimpy and in Plp overexpressors: evidence for different apoptotic pathways. J Neurocytol (2002) 0.85

Immunoradiometric and two-site assay of human follicle-stimulating hormone. J Endocrinol (1973) 0.85

Developmental expression of major myelin protein genes in the CNS of X-linked hypomyelinating mutant rumpshaker. J Neurosci Res (1992) 0.84

Phenotypic severity of murine Plp mutants reflects in vivo and in vitro variations in transport of PLP isoproteins. Glia (1997) 0.84

Age related changes in the motor nerve conduction velocity in dogs. Res Vet Sci (1977) 0.84

Familial cerebellar ataxia with hydrocephalus in bull mastiffs. Vet Rec (1983) 0.84

Myotonia in the dog: a report of four cases. Vet Rec (1973) 0.84

The pathology of a sensory neuropathy affecting Long Haired Dachshund dogs. Acta Neuropathol (1982) 0.84

PMP22 transgenic dorsal root ganglia cultures show myelin abnormalities similar to those of human CMT1A. Ann Neurol (2001) 0.84

Distal denervating disease: a degenerative neuropathy of the distal motor axon in dogs. J Small Anim Pract (1979) 0.83

In vitro studies of axonally-regulated Schwann cell genes during Wallerian degeneration. J Neurocytol (1993) 0.83

Age-related axonal and myelin changes in the rumpshaker mutation of the Plp gene. Acta Neuropathol (2004) 0.83

Current progress in preimplantation genetic diagnosis. J Assist Reprod Genet (1993) 0.83

Pituitary-directed leukemia inhibitory factor transgene forms Rathke's cleft cysts and impairs adult pituitary function. A model for human pituitary Rathke's cysts. J Clin Invest (1997) 0.82

Transgenic mouse models of CMT1A and HNPP. Ann N Y Acad Sci (1999) 0.82

Spinal cord blood flow after acute experimental cord injury in dogs. J Neurol Sci (1976) 0.82

Peripheral nervous system in a case of canine giant axonal neuropathy. Neuropathol Appl Neurobiol (1979) 0.82

Ischaemic neuromyopathy in cats. Vet Rec (1979) 0.82

The extensive myelopathy of intervertebral disc protrusions in dogs ('the ascending syndrome'). J Small Anim Pract (1972) 0.82

Molecular cloning and transfection studies of M6b-2, a novel splice variant of a member of the PLP-DM20/M6 gene family. J Neurosci Res (1998) 0.81

Expression of myelin protein gene transcripts by Schwann cells of regenerating nerve. J Neurosci Res (1990) 0.81

Heritable differences among gp70-like molecules on C3H ultraviolet light-induced fibrosarcomas. Transplant Proc (1981) 0.81