Abstract
Fetal growth is a complex process depending on the genetics of the fetus, the availability of nutrients to the fetus, maternal nutrition and various growth factors and hormones of maternal, fetal and placental origin. The IGF system, and more particularly IGF2, is one of the most important endocrine and paracrine growth systems regulating fetal and placental growth (reviewed in [1]). The IGF2 gene is regulated by genomic imprinting and is expressed only from the paternally-inherited allele in most tissues during fetal development and after birth.
Imprinted genes are tightly regulated and are therefore particularly susceptible to changes, including environmental and nutritional changes. Dysregulation of a cluster of imprinted genes, including the IGF2 gene within the 11p15 region, results in two fetal growth disorders (Silver-Russell and Beckwith-Wiedemann syndromes) with opposite growth phenotypes. Those two syndromes are model imprinting disorders to decipher the regulation of genomic imprinting.
Keywords: Genomic imprinting, 11p15 region, fetal growth, Silver-Russell syndrome, Beckwith-Wiedemann syndrome, imprinting cycle, cis-regulatory element, trans-acting regulatory factor, genetics, DNA
Current Medicinal Chemistry
Title: Epigenetic and Genetic Mechanisms of Abnormal 11p15 Genomic Imprinting in Silver-Russell and Beckwith-Wiedemann Syndromes
Volume: 18 Issue: 12
Author(s): J. Demars, Y. Le Bouc, A. El-Osta and C. Gicquel
Affiliation:
Keywords: Genomic imprinting, 11p15 region, fetal growth, Silver-Russell syndrome, Beckwith-Wiedemann syndrome, imprinting cycle, cis-regulatory element, trans-acting regulatory factor, genetics, DNA
Abstract: Fetal growth is a complex process depending on the genetics of the fetus, the availability of nutrients to the fetus, maternal nutrition and various growth factors and hormones of maternal, fetal and placental origin. The IGF system, and more particularly IGF2, is one of the most important endocrine and paracrine growth systems regulating fetal and placental growth (reviewed in [1]). The IGF2 gene is regulated by genomic imprinting and is expressed only from the paternally-inherited allele in most tissues during fetal development and after birth.
Imprinted genes are tightly regulated and are therefore particularly susceptible to changes, including environmental and nutritional changes. Dysregulation of a cluster of imprinted genes, including the IGF2 gene within the 11p15 region, results in two fetal growth disorders (Silver-Russell and Beckwith-Wiedemann syndromes) with opposite growth phenotypes. Those two syndromes are model imprinting disorders to decipher the regulation of genomic imprinting.
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Cite this article as:
Demars J., Le Bouc Y., El-Osta A. and Gicquel C., Epigenetic and Genetic Mechanisms of Abnormal 11p15 Genomic Imprinting in Silver-Russell and Beckwith-Wiedemann Syndromes, Current Medicinal Chemistry 2011; 18 (12) . https://dx.doi.org/10.2174/092986711795496764
DOI https://dx.doi.org/10.2174/092986711795496764 |
Print ISSN 0929-8673 |
Publisher Name Bentham Science Publisher |
Online ISSN 1875-533X |
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