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Over-expression of MBP and PLP messenger RNA in 8-day-old trembler brain
L Bascles1, J Bonnet, B Garbay
1Groupe d'étude de l'expression génétique, Institut de Biochimie Cellulaire du C.N.R.S., Bordeaux, France.
Abstract:
The trembler mouse suffers from a dominantly inherited mutation of the peripheral myelin protein 22 (PMP-22) gene which results in abnormal myelination of the peripheral nervous system. However, the clinical symptoms observed in the mutant suggest abnormalities in the central nervous system. Using the Northern blot technique, we investigated the steady-state levels of mRNA encoding myelin protein genes in 8-day-old normal and trembler brains. We measured a two- to four-fold increase for myelin basic protein (MBP) and proteolipid protein (PLP) mRNAS in the trembler samples. Whether a relationship exists between this observation and the onset of the clinical symptoms is still to be determined.
Insights
The trembler mouse model shows increased myelin basic protein (MBP) and proteolipid protein (PLP) mRNA in the brain, despite a mutation affecting peripheral myelin protein 22 (PMP-22). Further research is needed to link these central nervous system changes to clinical symptoms.
Area of Science:
- Neuroscience
- Genetics
- Molecular Biology
Background:
- The trembler mouse model exhibits a mutation in the peripheral myelin protein 22 (PMP-22) gene, leading to peripheral nervous system demyelination.
- Clinical manifestations in trembler mice suggest potential central nervous system (CNS) involvement, despite the primary genetic defect being in the peripheral nervous system.
Purpose of the Study:
- To investigate the steady-state messenger RNA (mRNA) levels of myelin protein genes in the brains of normal and trembler mice.
- To explore potential central nervous system abnormalities in the trembler mouse model by examining myelin gene expression.
Main Methods:
- Utilized the Northern blot technique to quantify mRNA levels.
- Analyzed brain tissue from 8-day-old normal and trembler mice.
Main Results:
- Observed a significant two- to four-fold increase in myelin basic protein (MBP) mRNA levels in trembler mouse brains compared to controls.
- Detected a similar two- to four-fold increase in proteolipid protein (PLP) mRNA levels in trembler mouse brains.
- These findings indicate altered gene expression of key myelin proteins within the central nervous system of the trembler model.
Conclusions:
- The trembler mouse model displays elevated mRNA levels for MBP and PLP in the brain, suggesting central nervous system myelin abnormalities.
- The relationship between these observed central nervous system molecular changes and the onset of clinical symptoms requires further investigation.