Partial Rescue of Ocular Pigment Cells and Structure by Inducible Ectopic Expression of Mitf-M in MITF-Deficient Mice
| العنوان: | Partial Rescue of Ocular Pigment Cells and Structure by Inducible Ectopic Expression of Mitf-M in MITF-Deficient Mice |
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| المؤلفون: | Amelework D Habtemichael, Corinne Rauck, Glenn Merlino, Kapil Bharti, Patricia Bunda, Helen T. Michael, Heinz Arnheiter, Cari Graff-Cherry, Chi-Ping Day, Sung Chin, Tunde Smith, Maria M Campos |
| المصدر: | Investigative Ophthalmology & Visual Science |
| بيانات النشر: | The Association for Research in Vision and Ophthalmology, 2018. |
| سنة النشر: | 2018 |
| مصطلحات موضوعية: | 0301 basic medicine, Male, Genotyping Techniques, Blotting, Western, retinal pigment epithelium, Embryonic Development, Iris, Mice, Transgenic, Biology, Microphthalmia, Retina, Ectopic Gene Expression, 03 medical and health sciences, chemistry.chemical_compound, Mice, medicine, Animals, Microphthalmos, Skin, Regulation of gene expression, Microphthalmia-Associated Transcription Factor, Retinal pigment epithelium, integumentary system, Choroid, Reverse Transcriptase Polymerase Chain Reaction, Gene Expression Profiling, Gene Expression Regulation, Developmental, Retinal, General Medicine, medicine.disease, Microphthalmia-associated transcription factor, Molecular biology, Immunohistochemistry, eye diseases, body regions, Intramolecular Oxidoreductases, Mice, Inbred C57BL, melanocytes, 030104 developmental biology, medicine.anatomical_structure, chemistry, microphthalmia, Retinal dysplasia, Ectopic expression, Female, sense organs, Dopachrome tautomerase |
| الوصف: | Purpose Complete deficiency of microphthalmia transcription factor (MITF) in Mitfmi-vga9/mi-vga9 mice is associated with microphthalmia, retinal dysplasia, and albinism. We investigated the ability of dopachrome tautomerase (DCT) promoter-mediated inducible ectopic expression of Mitf-M to rescue these phenotypic abnormalities. Methods A new mouse line was created with doxycycline-inducible ectopic Mitf-M expression on an Mitf-deficient Mitfmi-vga9 background (DMV mouse). Adult DMV mice were phenotypically characterized and tissues were collected for histology, immunohistochemistry, and evaluation of Mitf, pigmentary genes, and retinal pigment epithelium (RPE) gene expression. Results Ectopic Mitf-M expression was specifically induced in the eyes, but was not detected in the skin of DMV mice. Inducible expression of Mitf-M partially rescued the microphthalmia, RPE structure, and pigmentation as well as a subset of the choroidal and iris melanocytes but not cutaneous melanocytes. RPE function and vision were not restored in the DMV mice. Conclusions Ectopic expression of Mitf-M during development of Mitf-deficient mice is capable of partially rescuing ocular and retinal structures and uveal melanocytes. These findings provide novel information about the roles of Mitf isoforms in the development of mouse eyes. |
| اللغة: | English |
| تدمد: | 1552-5783 0146-0404 |
| URL الوصول: | https://explore.openaire.eu/search/publication?articleId=doi_dedup___::4061bc6239fdac26269a8d182457f8e5 http://europepmc.org/articles/PMC6314104 |
| حقوق: | OPEN |
| رقم الأكسشن: | edsair.doi.dedup.....4061bc6239fdac26269a8d182457f8e5 |
| قاعدة البيانات: | OpenAIRE |
| تدمد: | 15525783 01460404 |
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