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Expression of mBRI2 in mice
Fiona Pickford1, Luisa Onstead, Carolina Camacho-Prihar
1Department of Neuroscience, Mayo Clinic, 4500 San Pablo Rd, Jacksonville, Florida, FL 32224, USA.
Abstract:
Mutations in the BRI(2) gene cause the autosomal dominant neurodegenerative diseases familial British dementia (FBD) and familial Danish dementia (FDD). BRI(2) is a member of a family of type 2 integral transmembrane spanning proteins, including mBRI(2), its murine homologue. The function of BRI(2) is unknown. Northern and Western analyses and in situ hybridization were employed to determine the expression of mBRI(2) in the mouse. mBRI(2) mRNA was expressed in several tissues including the liver, heart, lung, and ubiquitously throughout the brain. mBRI(2) protein was detected at high levels in many brain regions. Murine BRI(2) expression is similar to that described in the human brain but does not fully explain the distribution of pathology seen in FBD and FDD.
Insights
Researchers investigated the expression of murine BRI(2) (mBRI(2)) in mice to understand its role in neurodegenerative diseases. While mBRI(2) is widespread in the brain, its expression pattern doesn't fully account for the pathology observed in familial British and Danish dementias.
Area of Science:
- Neuroscience
- Genetics
- Molecular Biology
Background:
- Familial British dementia (FBD) and familial Danish dementia (FDD) are autosomal dominant neurodegenerative diseases linked to mutations in the BRI(2) gene.
- BRI(2) is a type 2 integral transmembrane protein with an unknown function.
- Understanding BRI(2) expression is crucial for elucidating its role in these conditions.
Purpose of the Study:
- To determine the expression pattern of the murine homologue of BRI(2), mBRI(2), in various tissues and brain regions.
- To investigate the potential correlation between mBRI(2) expression and the neuropathology of FBD and FDD.
Main Methods:
- Northern and Western blot analyses were used to detect mBRI(2) mRNA and protein levels.
- In situ hybridization was employed to visualize the spatial expression of mBRI(2) within the mouse brain.
Main Results:
- mBRI(2) mRNA was detected in multiple tissues, including the liver, heart, and lung, and was ubiquitously expressed throughout the brain.
- High levels of mBRI(2) protein were found in numerous brain regions.
- The expression pattern of mBRI(2) in the mouse brain mirrors that observed in humans.
Conclusions:
- Murine BRI(2) exhibits broad expression in both peripheral tissues and the central nervous system.
- Despite widespread expression, the observed mBRI(2) distribution does not fully explain the specific pathological localization in familial British and Danish dementias.
- Further research is needed to fully understand the function of BRI(2) and its precise role in the pathogenesis of FBD and FDD.
