Expression pattern of the V5-Ostm1 protein in bacterial artificial chromosome transgenic mice
| Autor: | Jean Vacher, Pardis Yousefi Behzadi, Monica Pata |
|---|---|
| Rok vydání: | 2021 |
| Předmět: |
Genetically modified mouse
Chromosomes Artificial Bacterial Transgene Mice Transgenic Biology 03 medical and health sciences Mice 0302 clinical medicine Endocrinology In vivo Genetics Animals Gene 030304 developmental biology 0303 health sciences Bacterial artificial chromosome Membrane Proteins Cell Biology Phenotype Transmembrane protein Recombinant Proteins Cell biology Mice Inbred C57BL Disease Models Animal Osteopetrosis Mutation Homologous recombination 030217 neurology & neurosurgery |
| Zdroj: | Genesis (New York, N.Y. : 2000)REFERENCES. 59(3) |
| ISSN: | 1526-968X |
| Popis: | Mutations in the osteopetrotic transmembrane protein 1 (Ostm1) gene are responsible for the most severe form of autosomal recessive osteopetrosis both in humans and in the gray lethal (gl/gl) mouse. This defect leads to increased bone mass with bone marrow occlusion and hematopoietic defects. To establish the expression profile of the mouse Ostm1 protein in vivo, homologous recombination in bacteria was designed to generate a V5-Ostm1 bacterial artificial chromosome (BAC) that was subsequently integrated in the mouse genome. Tissue expression of the transgene V5-Ostm1 RNA and protein in transgenic mice follow the endogenous expression profile. Immunohistochemistry analysis demonstrated expression in neuronal populations from central and peripheral nervous system and defined a unique cellular expression pattern. Importantly, together with appropriate protein post-translational modification, in vivo rescue of the osteopetrotic bone gl/gl phenotype in BAC V5-Ostm1 gl/gl mice is consistent with the expression of a fully functional and active protein. These mice represent a unique tool to unravel novel Ostm1 functions in individual tissue and neuronal cell populations and the V5-Ostm1 transgene represents an easy visual marker to monitor the expression of Ostm1 in vitro and in vivo. |
| Databáze: | OpenAIRE |
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