| Autor: |
Perez-Ortiz AC; Massachusetts General Hospital, Division of Surgery, 55 Fruit St, Boston, MA 02214, USA. andric@aya.yale.edu.; Laboratory of Epidemiology and Public Health, Yale University School of Public Health, 60 College St, New Haven, CT 06510, USA. andric@aya.yale.edu., Peralta-Ildefonso MJ; Facultad de Química, Universidad Nacional Autónoma de México, 04510 Ciudad de México, Mexico. martha.janneth2830@gmail.com.; Laboratorio de Biología Molecular, Universidad Panamericana, Escuela de Medicina, Donatello 59 Insurgentes Mixcoac Benito Juárez, 03920 Ciudad de México, Mexico. martha.janneth2830@gmail.com., Lira-Romero E; Laboratorio de Biología Molecular, Universidad Panamericana, Escuela de Medicina, Donatello 59 Insurgentes Mixcoac Benito Juárez, 03920 Ciudad de México, Mexico. elira@up.edu.mx., Moya-Albor E; Facultad de Ingeniería, Universidad Panamericana, Augusto Rodin 498, 03920 Ciudad de México, Mexico. emoya@up.edu.mx., Brieva J; Facultad de Ingeniería, Universidad Panamericana, Augusto Rodin 498, 03920 Ciudad de México, Mexico. jbrieva@up.edu.mx., Ramirez-Sanchez I; Sección de Estudios de Posgrado e Investigación, Escuela Superior de Medicina, Instituto Politécnico Nacional, 11340 Ciudad de México, Mexico. iramirez@up.edu.mx., Clapp C; Instituto de Neurobiología, Campus UNAM-Juriquilla, Universidad Nacional Autónoma de México (UNAM), 76230 Querétaro, Mexico. clapp@unam.mx., Luna-Angulo A; Departamento de Neurociencias, Instituto Nacional de rehabilitación, México-Xochimilco, No.289. Arenal de Guadalupe, 14389 Ciudad de México, Mexico. lunangulo@gmail.com., Rendon A; Institut De La Vision, Sorbonne Universites, F-75012 Paris, France. alvaro.rendon@inserm.fr., Adan-Castro E; Instituto de Neurobiología, Campus UNAM-Juriquilla, Universidad Nacional Autónoma de México (UNAM), 76230 Querétaro, Mexico. elva.adan@gmail.com., Ramírez-Hernández G; Instituto de Neurobiología, Campus UNAM-Juriquilla, Universidad Nacional Autónoma de México (UNAM), 76230 Querétaro, Mexico. gaby.farmafesc@gmail.com., Díaz-Lezama N; Department of Physiological Genomics, Ludwig-Maximilians-Universität München, Großhaderner Str. 9, 82152 Planegg-Martinsried, Germany. nundehuidiaz@gmail.com., Coral-Vázquez RM; Sección de Estudios de Posgrado e Investigación, Escuela Superior de Medicina, Instituto Politécnico Nacional, 11340 Ciudad de México, Mexico. rmcoralv@gmail.com.; Subdirección de Enseñanza e Investigación, Centro Médico Nacional '20 de Noviembre', Instituto de Seguridad y Servicios Sociales de los Trabajadores del Estado, 03100 Ciudad de México, Mexico. rmcoralv@gmail.com., Estrada-Mena FJ; Laboratorio de Biología Molecular, Universidad Panamericana, Escuela de Medicina, Donatello 59 Insurgentes Mixcoac Benito Juárez, 03920 Ciudad de México, Mexico. rcoral@ipn.mx. |
| Abstrakt: |
Age-related macular degeneration (AMD) is the leading cause of central vision loss and severe blindness among the elderly population. Recently, we reported on the association of the SGCD gene (encoding for δ-sarcoglycan) polymorphisms with AMD. However, the functional consequence of Sgcd alterations in retinal degeneration is not known. Herein, we characterized changes in the retina of the Sgcd knocked-out mouse (KO, Sgcd -/- ). At baseline, we analyzed the retina structure of three-month-old wild-type (WT, Sgcd +/+ ) and Sgcd -/- mice by hematoxylin and eosin (H&E) staining, assessed the Sgcd-protein complex (α-, β-, γ-, and ε-sarcoglycan, and sarcospan) by immunofluorescence (IF) and Western blot (WB), and performed electroretinography. Compared to the WT, Sgcd -/- mice are five times more likely to have retinal ruptures. Additionally, all the retinal layers are significantly thinner, more so in the inner plexiform layer (IPL). In addition, the number of nuclei in the KO versus the WT is ever so slightly increased. WT mice express Sgcd-protein partners in specific retinal layers, and as expected, KO mice have decreased or no protein expression, with a significant increase in the α subunit. At three months of age, there were no significant differences in the scotopic electroretinographic responses, regarding both a- and b-waves. According to our data, Sgcd -/- has a phenotype that is compatible with retinal degeneration. |
