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• Delay in growth associated with mutations in IGF1 and IGF1R insulin-like

growth factor 1 receptor - IGF1R (delay in growth due to resistance to insu-
lin-like growth factor type 1 (growth impairments and intellectual disability).

• Genetic basis for and aspects clinical of hereditary angioedema type 3.

• Phenotyping of genetically modified mice within the framework of the activi-
ty conducted through the SEFALer platform.

Most relevant • IGF-I deficiency and hearing loss: molecular clues and clinical implications. Varela-
n i, M-c s, r- r l, l l c J. Ped.
scientific ietourillouestaodríGuezdela osaassalettaand ontreras
Endocrinol. Rev. Invited Review. 10:4, 460-472. 2013.
articles
• Programmed cell senescence during embryonic development. Muñoz-esPín d, cañaMero
M, M a, G-l G, c J, M-c s, r-b a, V-
araVeróMezóPezontrerasurillouestaodríGuezaezaarela
nieto i, ruberte J, collado M, serrano M. Cell. 21;155(5):1104-18. 2013.

• Skeletal abnormalities in insulin-like growth factor-I mouse mutants can be partially

compensated for by treatment with n- and c-terminal peptides of parathyroid
hormone-related protein. rodríGuez-de la rosa l, lóPez-herradón a, Portal-núñez s,
í
Murillo-cuesta s, lozano d, cediel r, Varela-nieto i* and esbrit P* (*Equal senior
contribution) PLoS ONE. 2014. Epub 2013.

• Spanish Society of Biochemistry and Molecular Biology: celebrating 50 years. V-
arela
nieto i, rodrGuez-tarduchy G, PaJares Ma, lara c, Galindo a and bautista JM (artículo ó
invitado de divulgacin) FEBS News 27-29, May 2013.

Our unit studies hereditary hearing loss and ER associated with deficits in the IGF
Highlights
system by using animal and cell models, and working closely with clinical groups

ENT specialists and allergists. To do this, we have captured national and interna-
tional private and public funding. In partnership with biotech and electronic devices

companies, and supported by 2 FP7 European projects, we are working on the
characterization of animal models of hearing loss and its use for preclinical testing

of new drug candidates (AFHELO, www.afhelo.eu/). The ITN Marie Curie, TARGEAR

(www.targear.eu), which I coordinate, is focused to study hearing loss combining
basic researchers with technology transfer and translational clinical research, nota-

bly the network will develop training activities for early stage researchers.

We are an international reference in the study of the actions of IGF -I, and in the
study of auditory pathophysiology as indicated by: i) the co - edition of Develop-

ment of the Auditory and Vestibular Systems (Elsevier, 2013); ii ) the invited lec-
tures to IGF Gordon conferences and ARO; and iii ) the organization of the 50th

Workshop “ Inner Ear Biology” (Madrid). In addition, I am a member of the follow-

ing international committees for biomedical research: BMBS -COST (from 2013), 13
ESF- Scientific Review Group and EUROPE_PMC -Wellcome Trust.
20
T
The objective of patenting research results has meant a delay in the publication OR
P
of the work done for DIGNA Biotech, Puleva and Affichem. Despite which, since RE
2011, we have produced a total of 21 scientific contributions and 1 outreach ar- L
A
ticle. During 2013 we have helped to define: 1) the molecular basis of embryonic NU
N
development of the inner ear; and 2) the deficit in IGF -I in hearing loss and growth A
delay. I have coordinated the “Laboratory Animal Phenotyping Network” (SEFALer R /
E
- CIBERER platform) and carried out annual training activities.
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