Myelin deficient mice: Expression of myelin basic protein and generation of mice with varying levels of myelin

@article{Popko1987MyelinDM,
  title={Myelin deficient mice: Expression of myelin basic protein and generation of mice with varying levels of myelin},
  author={Brian Popko and Carmie Puckett and Eric Lai and H. David Shine and Carol Readhead and Naoki Takahashi and Stephen W. Hunt and Richard L. Sidman and Leroy E. Hood},
  journal={Cell},
  year={1987},
  volume={48},
  pages={713-721}
}
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184 Citations
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51
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184 Citations

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Expression of only one myelin basic protein allele in mouse is compatible with normal myelination.
A novel mutation in myelin-deficient mice results in unstable myelin basic protein gene transcripts
Morphometric Analysis of Normal, Mutant, and Transgenic CNS: Correlation of Myelin Basic Protein Expression to Myelinogenesis
TLDR
The presence of axons loosely wrapped with oligodendrocytic membrane in mice expressing lower amounts of MBP mRNA and protein suggested that the oligodENDroglia produced sufficient MBP to elicit axon wrapping but not enough to form compact myelin, suggesting that oligod endodendroglial function is governed in part by the degree ofMBP expression.
Expression of a myelin basic protein gene in transgenic shiverer mice: Correction of the dysmyelinating phenotype
Myelin‐Deficient Mutant Mice.
TLDR
Myelin-deficient mutant mice (rnld) are characterized by trembling of hind limbs, occasional seizures in adults, reduced fertility, and shortened life span, indicating a change in a single gene.
The duplicated myelin basic protein gene in mld mutant mice does not impair transcription
MOLECULAR BIOLOGY OF MYELIN BASIC PROTEIN : GENE REARRANGEMENT AND EXPRESSION OF ANTI-SENSE RNA IN MYELIN-DEFICIENT MUTANTS
Rearrangement and Reactivation of the Myelin Basic Protein Locus in Myelin‐Deficient (mld) Mouse Brain
TLDR
Polymerase chain reaction analysis was used to detect both reinverted and circularized MBP gene sequences in mld mouse tissues, indicating that DNA rearrangement at the MBP locus does occur and the significance of DNA rearrangeement in mammalian development is discussed.
Dysmyelination and reduced myelin basic protein gene expression by oligodendrocytes of SHP‐1‐deficient mice
TLDR
It is proposed that SHP‐1 is a critical regulator of developmental signals leading to terminal differentiation and myelin sheath formation by oligodendrocytes and MBP protein and mRNA levels were reduced in SHP-1‐deficient mice compared to that in the CNS of normal littermates.
Structure and expression of myelin basic protein gene sequences in the mld mutant mouse: reiteration and rearrangement of the MBP gene.
TLDR
The results indicate that both shi and mld are cis-acting codominant mutations that cause severely reduced steady state levels of MBP-specific mRNA and MBP polypeptides in the brain.
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References

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Mice heterozygous for the mld mutation have intermediate levels of myelin basic protein mRNA and its translation products.
Myelination in the CNS of mld mutant mice: comparison between composition and structure.
Animal Models of Genetic Disorders of Myelin
Improved understanding of the assembly and maintenance of myelin is afforded by the study of animals in which interference with these processes occurs as a result of genetic variation, toxic…
Characterization of cloned cDNA representing rat myelin basic protein: Absence of expression in brain of shiverer mutant mice
Chromosomal mapping of mouse myelin basic protein gene and structure and transcription of the partially deleted gene in shiverer mutant mice
Myelin Basic Protein Deposition in the Optic and Sciatic Nerves of Dysmyelinating Mutants Quaking, Jimpy, Trembler, MLD, and Shiverer During Development
TLDR
Myelin basic protein (MBP) was quantified by radioimmunoassay in the optic and sciatic nerves of mice from birth to adult stages, giving special attention to the premyelinating and early myeli‐nation periods.
Myelin deficient (shimld ) mutant allele: Morphological comparison with shiverer (shi) allele on a B6C3 mouse stock
Characterization of mouse myelin basic protein messenger RNAs with a myelin basic protein cDNA clone.
TLDR
The cDNA clone of mouse myelin basic protein, pNZ111, corresponds to the region of the mRNA that codes for an amino acid sequence present in all four major forms of MBP, and hybridized to multiple species of mouse mRNA.
Cloning and characterization of the myelin basic protein gene from mouse: one gene can encode both 14 kd and 18.5 kd MBPs by alternate use of exons
Developmental regulation of myelin basic protein expression in mouse brain.
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