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International Cooperation to Enable the Diagnosis of All Rare Genetic Diseases.

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posted on 2018-05-11, 15:27 authored by K. M. Boycott, A. Rath, J. X. Chong, T. Hartley, F. S. Alkuraya, G. Baynam, Anthony J. Brookes, M. Brudno, A. Carracedo, JT den Dunnen, S. O. M. Dyke, X. Estivill, J. Goldblatt, C. Gonthier, SC Groft, I. Gut, A. Hamosh, P. Hieter, S. Höhn, M. E. Hurles, P. Kaufmann, B. M. Knoppers, J. P. Krischer, M. Macek, G. Matthijs, A. Olry, S. Parker, J. Paschall, A .A. Philippakis, H. L. Rehm, P. N. Robinson, P-C. Sham, R. Stefanov, D. Taruscio, D. Unni, M. R. Vanstone, F. Zhang, H. Brunner, M. J. Bamshad, H. Lochmüller
Provision of a molecularly confirmed diagnosis in a timely manner for children and adults with rare genetic diseases shortens their "diagnostic odyssey," improves disease management, and fosters genetic counseling with respect to recurrence risks while assuring reproductive choices. In a general clinical genetics setting, the current diagnostic rate is approximately 50%, but for those who do not receive a molecular diagnosis after the initial genetics evaluation, that rate is much lower. Diagnostic success for these more challenging affected individuals depends to a large extent on progress in the discovery of genes associated with, and mechanisms underlying, rare diseases. Thus, continued research is required for moving toward a more complete catalog of disease-related genes and variants. The International Rare Diseases Research Consortium (IRDiRC) was established in 2011 to bring together researchers and organizations invested in rare disease research to develop a means of achieving molecular diagnosis for all rare diseases. Here, we review the current and future bottlenecks to gene discovery and suggest strategies for enabling progress in this regard. Each successful discovery will define potential diagnostic, preventive, and therapeutic opportunities for the corresponding rare disease, enabling precision medicine for this patient population.

History

Citation

American Journal of Human Genetics, 2017, 100 (5), pp. 695-705

Author affiliation

/Organisation/COLLEGE OF LIFE SCIENCES/Biological Sciences/Genetics and Genome Biology

Version

  • VoR (Version of Record)

Published in

American Journal of Human Genetics

Publisher

Elsevier

issn

0002-9297

eissn

1537-6605

Copyright date

2017

Available date

2018-05-11

Publisher version

https://www.sciencedirect.com/science/article/pii/S0002929717301477?via=ihub

Language

en

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