Man

Danilo Bilches Medinas

Staff Scientist

Instituto de Neurociencia Biomedica

Santiago, Chile

Líneas de Investigación


Free Radicals and Oxidants; Protein Misfolding and Aggregation; Cellular Stress; Cell Signaling; Neurodegenerative Diseases; Neurodevelopmental Disorders; Gene Therapy.

Educación

  •  Biochemistry, UNIVERSIDADE DE SAO PAULO. Brasil, 2010
  •  Chemistry, UNIVERSIDADE DE SAO PAULO. Brasil, 2005

Experiencia Académica

  •   Postdoctoral Fellow Full Time

    STANFORD UNIVERSITY

    Palo Alto, Estados Unidos

    2010 - 2011

  •   postdoc Full Time

    UNIVERSIDAD DE CHILE

    Santiago, Chile

    2012 - 2015

Experiencia Profesional

  •   Staff Scientist Full Time

    Instituto de Neurociencia Biomedica

    Santiago, Chile

    2015 - A la fecha

Formación de Capital Humano


1. Giselle Aimé Espinosa Vásquez, Evaluation of the impact of conditional genetic ablation of Pdia3 on the cognitive capacity of mice. Director. Undergraduate Thesis in Biochemistry. University of Santiago de Chile. Ongoing.
2. José Vicente González Teuber, Study of the contribution of IRE1? signaling to experimental amyotrophic lateral sclerosis (ALS). Co-director. PhD Thesis in Biomedical Sciences. University of Chile. Ongoing.
3. Pablo Sebastián Rozas Rojas, Effects of Erp57 Overexpression in a Mutant Sod1 Mouse Model of Amyotrophic Lateral Sclerosis. Co-director. Master Thesis in Biological Sciences. University of Chile. 2017.
4. Martín Nicolás Sepúlveda Durand, Characterization of Transgenic Mouse Lines for Mutant Forms of ERp57 Associated to Amyotrophic Lateral Sclerosis. Co-director. Undergraduate Thesis of Molecular Biotechnology Engineering. University of Chile. 2015.


Premios y Distinciones

  •   Travel Award

    Society for Free Radical Biology and Medicine

    Uruguay, 2007

    Travel Award from the Society for Free Radical Biology and Medicine to present the work entitled “An Alternative Mechanism for Carbonate Radical Generation during Cu,ZnSod Peroxidase Activity”.

  •   Travel Award

    Society for Free Radical Biology and Medicine

    Estados Unidos, 2009

    Travel Award from the Society for Free Radical Biology and Medicine to present the work entitled “MS Characterization of the Human Sod1 Covalent Dimer Produced from the Enzyme Peroxidase Activity. Implication to ALS”.

  •   Dean’s Fellowship

    STANFORD UNIVERSITY

    Chile, 2010

    Postdoctoral Fellowship

  •   FAPESP Fellowship

    FAPESP

    Brasil, 2005

    Doctorate Fellowship


 

Article (23)

beta-catenin aggregation in models of ALS motor neurons: GSK3 beta inhibition effect and neuronal differentiation
Network approach identifies Pacer as an autophagy protein involved in ALS pathogenesis
Endoplasmic reticulum stress leads to accumulation of wild-type SOD1 aggregates associated with sporadic amyotrophic lateral sclerosis.
Disulfide cross-linked multimers of TDP-43 and spinal motoneuron loss in a TDP-43(A315T) ALS/FTD mouse model
Fine-Tuning ER Stress Signal Transducers to Treat Amyotrophic Lateral Sclerosis
IRE1 signaling exacerbates Alzheimer's disease pathogenesis
Adaptive preconditioning in neurological diseases - therapeutic insights from proteostatic perturbations
ALS-linked protein disulfide isomerase variants cause motor dysfunction
Creutzfeldt-Jakob disease. Report of one case
Functional Role of the Disulfide Isomerase ERp57 in Axonal Regeneration
Oligomerization of Cu,Zn-Superoxide Dismutase (SOD1) by Docosahexaenoic Acid and Its Hydroperoxides In Vitro=> Aggregation Dependence on Fatty Acid Unsaturation and Thiols.
The Protein-disulfide Isomerase ERp57 Regulates the Steady-state Levels of the Prion Protein.
Common ground: stem cell approaches find shared pathways underlying ALS.
Protein homeostasis: Modeling UPR adaptive responses.
The transcription factor CHOP, a central component of the transcriptional regulatory network induced upon CCl4 intoxication in mouse liver, is not a critical mediator of hepatotoxicity.
ER Dysfunction and Protein Folding Stress in ALS.
Proteostasis impairment: at the intersection between Alzheimer's disease and diabetes.
Generation of singlet oxygen by the glyoxal-peroxynitrite system.
A ditryptophan cross-link is responsible for the covalent dimerization of human superoxide dismutase 1 during its bicarbonate-dependent peroxidase activity.
Mechanism of the peroxidase activity of superoxide dismutase 1.
Peroxymonocarbonate and Carbonate Radical Displace the Hydroxyl-like Oxidant in the Sod1 Peroxidase Activity under Physiological Conditions
The carbonate radical and related oxidants derived from bicarbonate buffer
Tempol diverts peroxynitrite/carbon dioxide reactivity toward albumin and cells from protein-tyrosine nitration to protein-cysteine nitrosation

CaseReport (1)

Desafíos en el diagnóstico de enfermedad de Creutzfeldt-Jakob: Caso clínico

EditorialMaterial (1)

ER stress links aging to sporadic ALS

Note (1)

ERp57 as a novel cellular factor controlling prion protein biosynthesis: Therapeutic potential of protein disulfide isomerases

Proyecto (5)

DEFINING THE CONTRIBUTION OF ERP57 MUTANTS TO THE PATHOGENESIS OF AMYOTROPHIC LATERAL SCLEROSIS
DEFINING MECHANISMS AND THE ROLE OF AGGREGATION OF WILD-TYPE SUPEROXIDE DISMUTASE 1 (SOD1) IN AMYOTROPHIC LATERAL SCLEROSIS
Therapeutic Use of delta Protein Kinase C in Parkinson's Disease
Aldehyde Dehydrogenase Activators as Novel Drugs For the Treatment of Parkinson's Disease
Papéis de Oxidantes e Radicais Livres em Esclerose Lateral Amiotrófica (ELA). Implicações em Outros Processos Neurodegenerativos.

Review (2)

Proteostasis disturbance in amyotrophic lateral sclerosis
The ER proteostasis network in ALS: Determining the differential motoneuron vulnerability
17
Danilo Bilches

Staff Scientist

Instituto de Neurociencia Biomedica

Santiago, Chile

6
Claudio Hetz

Deputy Director

INSTITUTO DE CIENCIAS BIOMÉDICAS, FACULTAD DE MEDICINA, UNIVERSIDAD DE CHILE

SANTIAGO, Chile

5
Pablo Rozas

Asistente de Investigación

Instituto de Neurociencia Biomédica

Universidad de Chile

Santiago, Chile

3
Ute Wohlbier

Associate Professor

Center for Integrative Biology

Universidad Mayor de Chile

Santiago, Chile

2
Catherine Andreu

Estudiante de Doctorado

Escuela de Psicología

Pontificia Universidad Católica de Chile

Santiago, Chile

2
Maria Matus

Principal Investigator

Fundacion Ciencia & Vida

Santiago, Chile

2
JUAN PABLO HENRIQUEZ

Associate Professor

Cell Biology

UNIVERSIDAD DE CONCEPCIÓN, FACULTAD DE CIENCIAS BIOLÓGICAS, DEPARTAMENTO DE BIOLOGÍA CELULAR

Concepción, Chile

2
Felipe Court

Full Professor and Director

Center for Integrative Biology

Universidad Mayor

Huechuraba, Chile

2
Melissa Calegaro

Investigadora Principal

Centro de Biología Integrativa

Universidad Mayor

Santiago, Chile

1
Fernando Bustos

Profesor Asistente

Facultad de Medicina

Universidad Andres Bello

Santiago, Chile

1
Jose Matamala

Assistant Professor of Neurology

Department of Neurological Science

University of Chile

Santiago, Chile

1
Vicente Valenzuela

Investigador postdoctoral

Instituto de Neurociencia Biomédica

Santiago, Chile

1
Adrian Palacios

Profesor

Instituto de Neurosciencia

UNIVERSIDAD DE VALPARAISO

Valparaiso, Chile

1
Patricio Manque

VICE PRESIDENT FOR RESEARCH AND FULL PROFESSOR

UNIVERSIDAD MAYOR

Santiago, Chile

1
Diego Rojas

Assistant Professor

Center for Integrative Biology

Universidad Mayor

Santiago, Chile

1
René Vidal

Profesor Asistente

Universidad Mayor

Santiago, Chile

1
Alvaro Ardiles

Assistant Professor

Medicine

Universidad de Valparaíso

Valparaiso, Chile

1
Ariel Castro

Associate Professor

Department of Biochemistry and Molecular Biology

UNIVERSIDAD DE CONCEPCION

concepciòn, Chile

1
Alfredo Sagredo

Investigador Postdoctoral

ICBM

Universidad de Chile

Santiago, Chile

1
Viviana Pérez

Investigador Postdoctoral

Facultad de Medicina

Universidad Andrés Bello

Santiago, Chile

1
Jorge Ojeda

Académico investigador

Ciencias Básicas y Morfología

Universidad Católica de la Santisima Concepción

Concepción, Chile

1
Karina Palma

Postdoctorado

Instituto de Neurociencia Biomédica BNI

Universidad de Chile

Santiago, Chile

1
Brigitte van Zundert

Full Professor

UNIVERSIDAD ANDRÉS BELLO, CENTRO DE INVESTIGACIONES BIOMÉDICAS

Santiago, Chile

1
Pablo Henny

Profesor Asociado

Anatomía Normal

Pontificia Universidad Católica de Chile

Santiago, Chile

1
Ricardo Armisen

Associate Professor

Centro de Genética y Genómica

Universidad del Desarrollo

Santiago, Chile

1
Alicia Colombo

Profesor Asistente/Director Científico Biobanco de Tejidos y Fluidos de la Universidad de Chile

Departamento de Anatomía Patológica

Universidad de Chile

Santiago, Chile

1
Agustin Martinez

Profesor Titular

Instituto de Neurociencia

UNIVERSIDAD DE VALPARAÍSO, CENTRO INTERDISCIPLINARIO DE NEUROCIENCIAS DE VALPARAÍSO

Valparaíso, Chile

1
Fabiola Osorio

Associate Professor

Programa Disciplinario de Inmunologia

Universidad de Chile

Santiago, Chile