Mbp

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..quot; Dysmyelination and oligodendrocyte death emerge as an epiphenomenon of other PLP mutations and have been uncoupled in the PLP null allele from the risk of premature myelin breakdown...

..Neither Olig gene is required for astrocytes. These findings, together with fate mapping analysis of Olig-expressing cells, indicate that oligodendrocytes are derived from Olig-specified progenitors that give rise also to neurons...

b>Shiverer is an important model of central nervous system dysmyelination characterized by a deletion in the gene encoding myelin basic protein with relevance to human dysmyelinating and demyelinating diseases...

..We show that Nkx2.2 is also expressed in mammalian oligodendrocyte progenitors and that the differentiation of MBP-positive and PLP-DM20-positive oligodendrocytes is dramatically retarded in Nkx2...

..Thus, Oct-6 appears to be required for the transition of promyelin cells to myelinating cells. Once these cells progress past this point, Oct-6 is no longer required, and myelination occurs normally...

..Sox10 directly regulates myelin gene expression in oligodendrocytes, but does not control erbB3 expression as in peripheral glia. Sox10 thus functions in peripheral and central glia at different stages and through different mechanisms...

..In support of this suggestion, inhibition of expression of these two transcription factors in culture by antisense oligonucleotides has an additive inhibitory effect on OLP differentiation and proteolipid protein (PLP) gene expression...

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..Therefore Krox-20 is likely to control a set of genes required for completion of myelination in the peripheral nervous system...

..Similar to Oct-6, Brn-1 down-regulated its own expression at later stages of myelination. Thus, class III POU domain proteins can fully replace each other in Schwann cell development...

..Our studies demonstrate that calcineurin and NFAT are essential for neuregulin and ErbB signaling, neural crest diversification, and differentiation of Schwann cells...

..and CNS myelination have equivalent effects on axons, neurofilaments, and microtubules in CNS, myelin-deficient shiverer axons were examined...

..Developmental studies in hypomyelinating Shiverer mice revealed dramatically disrupted axoglial junctions, aberrant Na(+) channel clusters, and little or no ..

..These results suggest a role for highly sialylglycosylated Opalin in an intermembranous function of the myelin paranodal loops in the central nervous system...

..We conclude that the extent of functional equivalence depends on the tissue and that, despite their relatedness, Sox8 and Sox10 have more unique functions than previously appreciated...

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..This upregulation was transient because from 1 month after the lesion, Sema4D expression was back to its normal level. These results indicate that Sema4D is a novel inhibitory factor for axonal regeneration expressed in myelin...

..1992), but little is known about effects of oligodendrocytes on CNS axons. The shiverer mutant mouse has a deletion in the myelin basic protein gene that eliminates compact myelin in the CNS...

..We demonstrate here that myelin basic protein (MBP) is processed in vivo by the MHC class I pathway leading to a MBP79-87/Kk complex...

..Therefore, cell survival controls override proliferation controls for determining the final number and distribution of mature oligodendrocytes...

Concentrations of neurosteroids have been measured in the brains of postnatal myelin mutants jimpy (jp) and shiverer (shi) mice and of their normal controls...

..We demonstrate that quaking*shiverer double-mutant mice can survive for at least 100 days without any CNS myelin whatsoever...

Oligodendrocytes from the shiverer mutant mouse are missing most of the myelin basic protein (Mbp) gene. In axon-free cultures, they produce membrane sheets with abnormally assembled microtubule and actin-based structures...

..glycoprotein ligands were studied using immunoblotting and affinoblotting techniques in the forebrain of quaking, shiverer and jimpy dysmyelinating mutant mice and their respective control littermates during the postnatal development...

..Neuromotor activity and coordination are considerably improved compared to the shiverer trait.

..We propose here that myelin basic protein (MBP) is prerequisite, which is more diffusely expressed than MAG in early stages of myelination...

..fused to its soluble receptor (IL6RIL6), causes induction of myelin genes such as myelin basic protein (MBP) and myelin protein zero (Po) in embryonic dorsal root ganglia Schwann cells...

..Using a model of spontaneous EAE, we demonstrated that myelin basic protein (MBP)-specific CD4(+) T cells up-regulate activation markers in the CNS-draining cervical lymph nodes at a time when ..

..RGC-derived Shh signalling isthus necessary in vivo for maintenance of astrocyte proliferation, affecting both axo-glial and normal glial cell development in the optic nerve...

..Here, we aimed to investigate whether possible brain structural network differences in the shiverer mouse mutant, a relevant animal model of myelin related diseases, can reflect intrinsic individual brain ..

..Our studies reveal a complex regulatory network between different groups of Sox proteins that is essential for proper progression of oligodendrocyte development...

..Our findings indicate complex regulation of Olig2 by stage- and lineage-specific regulatory elements. They further suggest that transcriptional regulation of Olig2 is involved in segregation of pMN neuroblasts...

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..Full-length coronin 3 among other areas localized to outgrowing neurites, whereas truncated proteins efficiently suppressed neurite formation. Our results favour a role for coronin 3 in neuron morphogenesis and possibly migration...

..affect the expression of mRNAs that encode myelin structural proteins, including that for the myelin basic protein (MBP), until postnatal day 13 (P13)...

..data, and the predictions of CaM-binding motifs using the Calmodulin Target Database (), three main segments of MBP were suggested for the interaction...

..The myelination-regulating transcription factor Oct6 is involved in a complex modulatory relationship with Ski. We conclude that Ski is a crucial signal in Schwann cell development and myelination...

..Thus, in postnatal mice, although growing vessels are formed primarily by angiogenesis from preexisting vessels, a minor population of arterial endothelia may be derived from tissue-resident vascular precursor cells...

..Small unbranched cells expressing DM-20 but not myelin basic protein (MBP) and probably representing progenitors were detectable by immunostaining in similar locations...

The myelin basic protein (MBP) gene is part of the golli-mbp gene complex...

..mRNA and total transcripts from the myelin basic protein gene in brains from mice of wild-type and homozygous shiverer genotypes at several ages spanning postnatal development...

b>Shiverer (shi) is an autosomal recessive mutation in the mouse characterized by an almost total lack of central nervous system myelin...

Peripheral nerves of the shiverer mouse, which are characterized by the absence of major dense lines and myelin basic proteins in CNS myelin, were analyzed...

Cross-transplantations of neural tissue between jimpy (jp) shiverer (shi) and normal mice have been performed under heterochronic conditions...

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The myelin-deficient Shiverer (Shi/Shi) mutant mouse may be a useful model in assessing the dependence of brain (Na+ + K+)-ATPase concentration and composition on myelin membrane formation...

..sections of the spinal cord, optic nerve and trigeminal ganglion of adult control (+/+) and CNS myelin-deficient shiverer (shi/shi) mice...

Mice homozygous for the mutation myelin deficient (mld), an allele of shiverer, exhibit decreased CNS myelination, tremors, and convulsions of progressively increasing severity leading to an early death...

..These results suggest an important role for the dilute gene product in the elaboration, maintenance, or function of cellular processes of melanocytes and neurons...

We have isolated cDNA clones encoding the four different forms of mouse myelin basic protein (MBP) and have analyzed the structure of the MBP gene...

..the levels of myelin basic protein (MBP) and proteolipid protein (PLP) in the brains of mice bred to carry both the shi/shi and jp/Y hypomyelination defects...

A cDNA (M41) corresponding to a mouse myelin basic protein (MBP) mRNA with a longer 5'-untranslated region than predicted from earlier studies of MBP gene structure has been isolated and characterized...

..This animal model provides in vivo evidence linking the expression of HIV-1 proteins in oligodendrocytes to the spinal cord damage found in vacuolar myelopathy...

..We now think that while the major MBPs are intracellular adhesion molecules, some of the quantitatively less abundant isoforms that are expressed very early in development may have regulatory effects on the myelination program...

..These findings underscore the complexity of the myelination process and highlight the numerous ways in which it can be disrupted...

..Since myelin basic protein also takes part in myelin compaction, we bred double CAII-deficient, myelin-deficient (Mld) mutant mice, in which the adults would have some compact myelin sheaths and a partial deficiency in myelin basic ..

..We have isolated a hemopoietic MBP-related (HMBPR) cDNA clone from a mouse bone marrow cDNA library screened with an MBP cDNA probe...

..19 separate oligodendroglial cell lines, 10 derived from normal mice and 9 derived from the neurological mutant shiverer, which is missing a large segment of the myelin basic protein gene...

..3-Tpi-10-11.8-(Egr-1, Hmg17-rs9)-2.1-Fgfa-2.2-Grl-1-10.1-(Cdx-1, Csfmr, Pdgfrb, Pdea, Rps14)-2.1-Adrb-2-22.9-Mbp. Pgk-1rs5, Tpi-10, Hmg17-rs9, and Rps14 had not been previously mapped in the mouse; Egr-1 had only been ..

..Additionally, STOP suppression in oligodendrocytes derived from shiverer mice that lack MBP led to the complete suppression of microtubule cold stability at all stages of cell ..

..of multiple sclerosis involves a breakdown in T cell tolerance to myelin proteins like myelin basic protein (MBP)...

..Tsc2-del3 mice also serve as a model for hypomorphic TSC2 missense mutations reported in TSC patients...

..ATP1A1, ATP1A2, ATP1A3, B2M, GSTP, and SMST; 5 mouse genes, Ncam, Ngfg, Pim-1, Tcp-1, and Trp53; and 5 human genes, MBP, MYB, NEFM, SCN2A, and TCRGC1, 17 structural genes including 15 newly assigned genes and 11 related DNA fragments ..

..Interestingly, some heterozygote groups also showed abnormalities in one or more VsEP response parameters, suggesting that vestibular dysfunction, although less severe, may be present in some heterozygous animals...

..Our results provide further evidence that ZO-1 serves functions unrelated to the tight junction complex and indicate molecular heterogeneity of these cell-cell contacts...

..of transgenic mice were generated expressing the SV40 large T-antigen under control of the myelin basic protein (MBP) promoter...

..Finally, our data support the hypothesis that the basket and stellate cells arise from neuronally restricted, migratory precursors located in the early postnatal cerebellar white matter...

..Similar swellings were absent in dysmyelinated shiverer mice, which lack myelin basic protein (MBP), but recurred in MBP*PLP double mutants...

..existence of both conserved and diverse amino acid sequences in the junctional regions of the myelin basic protein (MBP)-specific T cell receptors (TCR) in mice is presented...

..The MBP/c-myc transgenic mice developed severe neurological disturbances characterized by action tremors and recurrent ..

We analyzed by in situ hybridization the spatiotemporal expression of dm-20, myelin basic protein (MBP) and 2'-3' cyclic nucleotide phosphodiesterase (CNP) during embryonic and postnatal development of the normal mouse and two plp/dm-20 ..

..In this study the shiverer mouse mutant in which the oligodendrocyte population is numerically normal, but has both quantitatively ..

..To explore the pathogenesis of this abnormality we have exploited a dystrophic in equilibrium shiverer mouse chimera preparation in which both the basal lamina phenotype and the genotype of myelin-forming Schwann ..

b>Shiverer is an autosomal recessive trait in the mouse characterized by early generalized tremors that become prominent in the hindquarters with age...

..Results demonstrate that Fms, the genetic locus containing this sequence, maps to mouse chromosome 18. Mouse Fms is thus not linked to the same set of genes involved in growth regulation that human FMS is linked to...

..The primary sequences of four molecular mass variants (14, 17, 18.5, and 21.5 kDa) of the mouse myelin basic protein (MBP) have recently been determined through analysis of cDNA clones of their mRNAs...

..On the other hand, the expression of the CGT gene is similar to that of the MBP gene in the CNS and the PNS...

..We have found that large caliber fibre pathways in the brain of the mutant mouse shiverer (shi, gene on chromosome 18), whose developmental fate of myelination is averted by deletion of five exons in the ..

..These studies are relevant to demyelinating diseases, like MS and leukodystrophies, and will enhance our understanding of myelination, remyelination and the effects of hypomyelination on neural function. ..

..Safety measures (blood tests and gynecologic evaluations) will also be followed. The overall goal of this study will be the development of an oral treatment, estriol, for RRMS. ..

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..On the other hand, if immune measures differ between genotypes (XX vs. XY) within a given phenotype, we will conclude that gender differences in immune measures are due to both sex hormones and sex chromosomes. ..

..cytokine production of lymph node cells (LNCs) from SJL mice immunized with the autoantigen myelin basic protein (MBP)...

The golli proteins are one of two families of proteins produced from the MBP gene with more ubiquitous expression than the classic MBP family...

..The success of our project should yield significant insights into the etiology of human mental disorders, which can be caused by mutations of not only coding genes but also noncoding molecules such as microRNAs. ..

..These studies are relevant to demyelinating diseases, like MS and leukodystrophies, and will enhance our understanding of myelination, remyelination and the effects of hypomyelination on neural function. ..

..Safety measures (blood tests and gynecologic evaluations) will also be followed. The overall goal of this study will be the development of an oral treatment, estriol, for RRMS. ..

..cytokine production of lymph node cells (LNCs) from SJL mice immunized with the autoantigen myelin basic protein (MBP)...

..This disease was chosen in light of the 9:1 female preponderance of the human disease system lupus erythematosus. ..

..e. the myelin basic protein (MBP) and proteolipid protein (PLP) genes, has begun to change in view of new information about their structure, ..

..We have been successful at immortalizing oligodendrocytes from normal and shiverer mice with a retrovirus containing a temperature sensitive SV40 large T antigen oncogene, many of which may be ..

..We have been successful at immortalizing oligodendrocytes from normal and shiverer mice with a retrovirus containing a temperature sensitive SV40 large T antigen oncogene, many of which may be ..

DESCRIPTION: The Golli-mbp gene encodes two families of proteins, the Golli proteins and the myelin basic proteins...

..The overall objective of the proposed work is to examine, in detail, the expression of myelin basic protein (MBP) genes in normal mice and in dysmyelinating mutants which we have shown to have altered MBP metabolism...