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Open AccessReview

Laron Syndrome Research Paves the Way for New Insights in Oncological Investigation

1
Department of Human Molecular Genetics and Biochemistry, Sackler School of Medicine, Tel Aviv University, Tel Aviv 69978, Israel
2
Shalom and Varda Yoran Institute for Human Genome Research, Tel Aviv University, Tel Aviv 69978, Israel
3
Endocrine and Diabetes Research Unit, Schneider Children’s Medical Center, Petah Tikva 49292, Israel
*
Author to whom correspondence should be addressed.
Cells 2020, 9(11), 2446; https://doi.org/10.3390/cells9112446
Received: 18 October 2020 / Revised: 4 November 2020 / Accepted: 5 November 2020 / Published: 9 November 2020
(This article belongs to the Special Issue GH and GHR Signaling in Disease and Health)
Laron syndrome (LS) is a rare genetic endocrinopathy that results from mutation of the growth hormone receptor (GH-R) gene and is typically associated with dwarfism and obesity. LS is the best characterized entity under the spectrum of the congenital insulin-like growth factor-1 (IGF1) deficiencies. Epidemiological analyses have shown that LS patients do not develop cancer, whereas heterozygous family members have a cancer prevalence similar to the general population. To identify genes and signaling pathways differentially represented in LS that may help delineate a biochemical and molecular basis for cancer protection, we have recently conducted a genome-wide profiling of LS patients. Studies were based on our collection of Epstein–Barr virus (EBV)-immortalized lymphoblastoid cell lines derived from LS patients, relatives and healthy controls. Bioinformatic analyses identified differences in gene expression in several pathways, including apoptosis, metabolic control, cytokine biology, Jak-STAT and PI3K-AKT signaling, etc. Genes involved in the control of cell cycle, motility, growth and oncogenic transformation are, in general, down-regulated in LS. These genetic events seem to have a major impact on the biological properties of LS cells, including proliferation, apoptosis, response to oxidative stress, etc. Furthermore, genomic analyses allowed us to identify novel IGF1 downstream target genes that have not been previously linked to the IGF1 signaling pathway. In summary, by ‘mining’ genomic data from LS patients, we were able to generate clinically-relevant information in oncology and, potentially, related disciplines. View Full-Text
Keywords: growth hormone; growth hormone receptor; insulin-like growth factor-1 (IGF1); Laron syndrome; cancer protection growth hormone; growth hormone receptor; insulin-like growth factor-1 (IGF1); Laron syndrome; cancer protection
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MDPI and ACS Style

Werner, H.; Sarfstein, R.; Nagaraj, K.; Laron, Z. Laron Syndrome Research Paves the Way for New Insights in Oncological Investigation. Cells 2020, 9, 2446. https://doi.org/10.3390/cells9112446

AMA Style

Werner H, Sarfstein R, Nagaraj K, Laron Z. Laron Syndrome Research Paves the Way for New Insights in Oncological Investigation. Cells. 2020; 9(11):2446. https://doi.org/10.3390/cells9112446

Chicago/Turabian Style

Werner, Haim; Sarfstein, Rive; Nagaraj, Karthik; Laron, Zvi. 2020. "Laron Syndrome Research Paves the Way for New Insights in Oncological Investigation" Cells 9, no. 11: 2446. https://doi.org/10.3390/cells9112446

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