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. 2016 Aug 23;7:153. doi: 10.3389/fgene.2016.00153

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Copyright © 2016 Robin and Magdinier.

This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) or licensor are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

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Telomeres-Lamins interaction at the NP. (A) In cells with long telomeres, Lamins A/C interact with TRF2, thus tethering some telomeres in a peripheral localization. Telomeres might also interact with soluble Lamins in the nucleoplasm. The silencing signature of telomeres can spread in cis (TPE-sensitive genes) but also in trans (TPE-OLD-sensitive genes) due to a mechanism yet to explore. With telomeres shortening, the diminution of repressive mark (TPE) or a local change in the chromatin conformation (TPE-OLD), modulate expression of TPE and TPE-OLD genes. (B) Both TPE and TPE-OLD have been described in FSHD, a muscular dystrophy genetically linked to the subtelomeric 4q35 locus. This subtelomeric locus and its abutting telomere are located at the NP and regulated by A-type Lamins. Moreover, the number of D4Z4 repeats influences the localization of the telomeres within the nuclei. In cells with few D4Z4 repeats the transcription of DUX4, a gene encoded by D4Z4 repeats, is increased upon telomere shortening through direct TPE while expression of SORBS2, located 5 Mb upstream is up-regulated through a TPE-OLD phenomenon.