Dra. Silvia Cecilia Irene Montañez Ojeda Grado: Doctor en Ciencias 1982 Escuela Nacional de Ciencias Biológicas del I.P.N. México Nivel SNI: 3 Tel: (55) 5747-3334 E-mail: cecim@cinvestav.mx

•    LÍNEAS DE INVESTIGACIÓN: Estudio de las bases moleculares de enfermedades hereditarias en el humano. A). Estudio de la expresión, distribución y función de las distrofinas cortas en un modelo de sistema nervioso y en células troncales neurales. B). Expresión y función de las isoformas de las distrofinas cortas que se expresan en cerebro y retina de roedores. C). Estudio del proteoma de células PC12 que sobreexpresan a las distrofinas cortas. D). Análisis del transcriptoma del cerebro, retina y células del Sistema Nervioso Central por RNA-Seq, para la expresión de las distrofinas y DAPs. E). Participación de las distrofinas cortas en la neuritogénesis.

PUBLICACIONES RELEVANTES:

García-Cruz C, Aragón J, Lourdel S, Annan A, Roger JE, Montanez C, Vaillend C Tissue- and cell-specific whole-transcriptome meta-analysis from brain and retina reveals differential expression of dystrophin complexes and new dystrophin spliced isoforms. .Hum Mol Genet. 2023 Jan 27;32(4):659-676. doi: 10.1093/hmg/ddac236.PMID: 36130212 Free PMC article.

Sánchez A, Aragón J, Ceja V, Rendon A, Montanez C. Nuclear transport and subcellular localization of the dystrophin Dp71 and Dp40 isoforms in the PC12 cell line. Biochem Biophys Res Commun. 2022 Nov 19;630:125-132. doi: 10.1016/j.bbrc.2022.09.035. Epub 2022 Sep 13.PMID: 36155058

García-Cruz C, Merino-Jiménez C, Aragón J, Ceja V, González-Assad B, Reyes-Grajeda JP, Montanez C. Overexpression of the dystrophins Dp40 and Dp40_L170P modifies neurite outgrowth and the protein expression profile of PC12 cells. Sci Rep. 2022 Jan 26;12(1):1410. doi: 10.1038/s41598-022-05271-2.PMID: 35082358 Free PMC article.

Romo-Yáñez J, Rodríguez-Martínez G, Aragón J, Siqueiros-Márquez L, Herrera-Salazar A, Velasco I, Montanez C dystrophin-associated proteins expression during neuronal or astrocytic differentiation. MethodsX. 2021 Mar 27;8:101325. doi: 10.1016/j.mex.2021.101325. eCollection 2021.PMID: 34430234 Free PMC article.

Paúl-González S, Aragón J, Rodríguez-Martínez G, Romo-Yáñez J, Montanez C. Differential expression of Dp71 and Dp40 isoforms in proliferating and differentiated neural stem cells: Identification of Dp40 splicing variants. Biochem Biophys Res Commun. 2021 Jun 30;560:152-158. doi: 10.1016/j.bbrc.2021.03.142. Epub 2021 May 12. PMID: 33989907

Tuy Nga Brignol, Dominique Mornet, Cecilia Montañez, Dora Fix Ventura, Cyrille Vaillend, et al.. What is the ocular phenotype associated with a single exon 78 deletion in Duchenne muscular dystrophy?. Journal of Human Genetics, Nature Publishing Group, 2020, 65 (8), pp.715-716. ⟨10.1038/s10038-020-0755-5⟩. ⟨hal-02640159⟩.

Romo-Yáñez J, Rodríguez-Martínez G, Aragón J, Siqueiros-Márquez L, Herrera-Salazar A, Velasco I, Montanez C. Characterization of the expression of dystrophins and dystrophin-associated proteins during embryonic neural stem/progenitor cell differentiation. Neurosci Lett. 2020 Sep 25;736:135247. doi: 10.1016/j.neulet.2020.135247. Epub 2020 Jul 12.PMID: 32668267

García-Cruz C, Merino-Jiménez C, Ceja V, Aragón J, Siqueiros-Márquez L, Reyes-Grajeda JP, Montañez C. The dystrophin isoform Dp71e_Δ71 is involved in neurite outgrowth and neuronal differentiation of PC12 cells. J Proteomics. 2019 Jan 16;191:80-87. doi: 10.1016/j.jprot.2018.03.027. Epub 2018 Apr 4.PMID: 29625189

Aragón J, González-Reyes M, Romo-Yáñez J, Vacca O, Aguilar-González G, Rendón A, Vaillend C, Montañez C. Dystrophin Dp71 Isoforms Are Differentially Expressed in the Mouse Brain and Retina: Report of New Alternative Splicing and a Novel Nomenclature for Dp71 Isoforms. Mol Neurobiol. 2018 Feb;55(2):1376-1386. doi: 10.1007/s12035-017-0405-x. Epub 2017 Jan 27.PMID: 28127699

Siqueiros-Marquez L, Bénard R, Vacca O, Charles-Messance H, Bolaños-Jimenez R, Guilloneau X, Sennlaub F, Montañez C, Sahel JA, Rendon A, Tadayoni R, Giocanti-Aurégan A. Protection of Glial Müller Cells by Dexamethasone in a Mouse Model of Surgically Induced Blood-Retinal Barrier Breakdown. .Invest Ophthalmol Vis Sci. 2017 Feb 1;58(2):876-886. doi: 10.1167/iovs.16-20617.PMID: 28159978

Herrera-Salazar A, García-Villegas R, Aragón J, Sánchez-Trujillo A, Ceja V, Martínez-Herrera A, Merino-Jiménez C, Montañez C. Overexpression of mutant dystrophin Dp71[INCREMENT]₇₈-₇₉ stimulates cell proliferation. Neuroreport. 2016 Jan 6;27(1):6-11. doi: 10.1097/WNR.0000000000000475.PMID: 26551922

Giocanti-Auregan A, Vacca O, Bénard R, Cao S, Siqueiros L, Montañez C, Paques M, Sahel JA, Sennlaub F, Guillonneau X, Rendon A, Tadayoni R. Altered astrocyte morphology and vascular development in dystrophin-Dp71-null mice. Glia. 2016 May;64(5):716-29. doi: 10.1002/glia.22956. Epub 2015 Dec 29.PMID: 26711882

Merino-Jiménez C, Aragón J, Ceja V, Rodríguez-Martínez G, Cázares-Raga FE, Chardonnet S, Pionneau C, Rendon A, Montañez C.  Dp71Δ78-79 dystrophin mutant stimulates neurite outgrowth in PC12 cells via upregulation and phosphorylation of HspB1. Proteomics. 2016 May;16(9):1331-40. doi: 10.1002/pmic.201500211. Epub 2016 Apr 13.PMID: 26936078

Vacca O, Charles-Messance H, El Mathari B, Sene A, Barbe P, Fouquet S, Aragón J, Darche M, Giocanti-Aurégan A, Paques M, Sahel JA, Tadayoni R, Montañez C, Dalkara D, Rendon A. AAV-mediated gene therapy in Dystrophin-Dp71 deficient mouse leads to blood-retinal barrier restoration and oedema reabsorption.Hum Mol Genet. 2016 Jul 15;25(14):3070-3079. doi: 10.1093/hmg/ddw159. Epub 2016 Jun 10.PMID: 27288449

Aragón J, Martínez-Herrera A, Romo-Yáñez J, Ceja V, Azotla-Vilchis C, Siqueiros-Márquez L, Soid-Raggi G, Herrera-Salazar A, Montañez C. Identification of Dp71 Isoforms Expressed in PC12 Cells: Subcellular Localization and Colocalization with β-Dystroglycan and α1-Syntrophin. J Mol Neurosci. 2016 Feb;58(2):201-9. doi: 10.1007/s12031-015-0657-8. Epub 2015 Sep 28.PMID: 26411569 

Aragón J, Martínez-Herrera A, Bermúdez-Cruz RM, Bazán ML, Soid-Raggi G, Ceja V, Coy-Arechavaleta AS, Alemán V, Depardón F, Montañez C. EF-hand domains are involved in the differential cellular distribution of dystrophin Dp40. Neurosci Lett. 2015 Jul 23;600:115-20. doi: 10.1016/j.neulet.2015.05.038. Epub 2015 May 21.PMID: 26004254

Martínez-Herrera A, Aragón J, Bermúdez-Cruz RM, Bazán ML, Soid-Raggi G, Ceja V, Santos Coy-Arechavaleta A, Alemán V, Depardón F, Montañez C. In silico analyses of dystrophin Dp40 cellular distribution, nuclear export signals and structure modeling. Data Brief. 2015 Jun 25;4:349-52. doi: 10.1016/j.dib.2015.06.007. eCollection 2015 Sep.PMID: 26217814 Free PMC article.

Saint Martín A, Aragón J, Depardon-Benítez F, Sánchez-Trujillo A, Mendoza-Hernández G, Ceja V, Montañez C. Identification of Dp71e, a new dystrophin with a novel carboxy-terminal end. FEBS J. 2012 Jan;279(1):66-77. doi: 10.1111/j.1742-4658.2011.08399.x. Epub 2011 Nov 17.PMID: 22008482 Free article.

Romo-Yáñez J, Montañez C, Salazar-Olivo LA. Dystrophins and DAPs are expressed in adipose tissue and are regulated by adipogenesis and extracellular matrix. Biochem Biophys Res Commun. 2011 Jan 14;404(2):717-22. doi: 10.1016/j.bbrc.2010.12.049. Epub 2010 Dec 11.PMID: 21147070

Aragón J, Romo-Yáñez J, Martínez-Herrera A, Ceja V, Rendon A, Montañez C. Characterization of Dp71Δ(78-79), a novel dystrophin mutant that stimulates PC12 cell differentiation. J Neurochem. 2011 Nov;119(4):697-707. doi: 10.1111/j.1471-4159.2011.07347.x. Epub 2011 Sep 22.PMID: 21668890 Free article.

Wong-Baeza C, Bustos I, Serna M, Tescucano A, Alcántara-Farfán V, Ibáñez M, Montañez C, Wong C, Baeza I. Membrane fusion inducers, chloroquine and spermidine increase lipoplex-mediated gene transfection. Biochem Biophys Res Commun. 2010 May 28;396(2):549-54. doi: 10.1016/j.bbrc.2010.04.143. Epub 2010 May 8.PMID: 20438715

Ilarraza-Lomeli R, Cisneros-Vega B, Cervantes-Gomez Mde L, Mornet D, Montañez C. Dp71, utrophin and beta-dystroglycan expression and distribution in PC12/L6 cell cocultures. Neuroreport. 2007 Oct 29;18(16):1657-61. doi: 10.1097/WNR.0b013e3282f0e42d.PMID: 17921863 Free PMC article.

Romo-Yáñez J, Ceja V, Ilarraza-Lomelí R, Coral-Vázquez R, Velázquez F, Mornet D, Rendón A, Montañez C. Dp71ab/DAPs complex composition changes during the differentiation process in PC12 cells. J Cell Biochem. 2007 Sep 1;102(1):82-97. doi: 10.1002/jcb.21281.PMID: 17390338

de León MB, Montañez C, Gómez P, Morales-Lázaro SL, Tapia-Ramírez V, Valadez-Graham V, Recillas-Targa F, Yaffe D, Nudel U, Cisneros B. Dystrophin Dp71 expression is down-regulated during myogenesis: role of Sp1 and Sp3 on the Dp71 promoter activity. J Biol Chem. 2005 Feb 18;280(7):5290-9. doi: 10.1074/jbc.M411571200. Epub 2004 Nov 18.PMID: 15550398 Free article.

Acosta R, Montañez C, Fuentes-Mera L, Gonzalez E, Gómez P, Quintero-Mora L, Mornet D, Alvarez-Salas LM, Cisneros B. Dystrophin Dp71 is required for neurite outgrowth in PC12 cells. Exp Cell Res. 2004 Jun 10;296(2):265-75. doi: 10.1016/j.yexcr.2004.01.015.PMID: 15149856

F G Marquez ¹, B Cisneros, F Garcia, V Ceja, F Velázquez, F Depardón, L Cervantes, A Rendón, D Mornet, H Rosas-vargas, M Mustre, C Montañez Differential expression and subcellular distribution of dystrophin Dp71 isoforms during differentiation process. Neuroscience Affiliations expand . 2003;118(4):957-66. doi: 10.1016/s0306-4522(03)00063-0. PMID: 12732241 DOI: 10.1016/s0306-4522(03)00063-0

Rosas-Vargas H, Montañez C, Rendón A, Mornet D, García F, Ceja V, Cisneros B. Expression and localization of utrophin in differentiating PC12 cells. Neuroreport. 2000 Jul 14;11(10):2253-7. doi: 10.1097/00001756-200007140-00038.PMID: 10923681

González E, Montañez C, Ray PN, Howard PL, García-Sierra F, Mornet D, Cisneros B. Alternative splicing regulates the nuclear or cytoplasmic localization of dystrophin Dp71. FEBS Lett. 2000 Oct 6;482(3):209-14. doi: 10.1016/s0014-5793(00)02044-5.PMID: 11024462 Free article.

Rodius F, Claudepierre T, Rosas-Vargas H, Cisneros B, Montanez C, Dreyfus H, Mornet D, Rendon A. Dystrophins in developing retina: Dp260 expression correlates with synaptic maturation. Neuroreport. 1997 Jul 7;8(9-10):2383-7. doi: 10.1097/00001756-199707070-00056.PMID: 9243645

Cisneros B, Rendon A, Genty V, Aranda G, Marquez F, Mornet D, Montañez C. Expression of dystrophin Dp71 during PC12 cell differentiation. Neurosci Lett. 1996 Aug 2;213(2):107-10. doi: 10.1016/0304-3940(96)12863-9.PMID: 8858620