Decreased lysyl oxidase-like 2 expression in mid-dermal elastolysis
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Decreased lysyl oxidase-like 2 expression in mid-dermal elastolysis T. Gambichler • M. Skrygan
Received: 26 September 2012 / Revised: 3 December 2012 / Accepted: 4 December 2012 / Published online: 14 December 2012 Ó Springer-Verlag Berlin Heidelberg 2012
Abstract Mid-dermal elastolysis (MDE) is a rare disorder that is histopathologically characterized by selective loss of elastic fibres in the mid-dermis. Aetiopathogenesis of MDE is still obscure. We report for the first time on the expression of lysyl oxidase-like (LOXL) proteins in lesional and non-lesional skin of a patient with reticular variant of MDE. Real-time RT-PCR and immunohistochemistry were performed for matrix metalloproteinase-2 (MMP2), MMP7, MMP9, LOXL1, LOXL2, and LOXL3. LOXL1 and LOXL3 mRNA levels in lesional skin did not substantially differ from mRNA levels measured in healthy skin. For LOXL2, however, we found decreased mRNA expression in lesional skin as compared to healthy skin. mRNA expression of MMP2 and MMP7 of lesional skin did not substantially differ from healthy skin. However, MMP9 mRNA expression was massively increased in lesional skin when compared to healthy skin. Immunohistochemistry confirmed the altered expression of LOXL2 and MMP9 in lesional skin. In conclusion, our preliminary data suggest that not only increased elastolytic activity (e.g. MMP9 up-regulation) but also affected elastin renewal due to reduced LOXL expression may contribute to the pathogenesis of MDE. More research is warranted in MDE especially with regard to the LOX family, fibulins, fibrillins, and desmosines.
Introduction Mid-dermal elastolysis (MDE) is a rare disorder of the elastic tissue that is characterized histopathologically by selective loss of elastic fibres in the mid-dermis. Though about 80 cases of MDE have been described in the literature, the aetiopathogenesis of the disease is obscure [7, 23]. Previous reports indicate that elastin degradation in MDE may be caused by increased activity of elastases such as matrix metalloproteinases (MMPs). Female gender, hormones (e.g., contraceptives), smoking, and ultraviolet exposure have been discussed as predisposing and pathogenetic factors in MDE, respectively [7, 8, 10, 17, 23]. Besides elastase/anti-elastase alterations, an imbalance between degradation and elastin renewal might also play an important role in the pathogenesis of MDE. The lysyl oxidase (LOX) family is an emerging family of amine oxidases responsible for the formation of collagen and elastin fibrils in the extracellular matrix. In addition to LOX, four LOX-like proteins (LOXL1, LOXL2, LOXL3, and LOXL4) of the same gene family have also been identified which play a major role in biosynthesis and renewal of the elastic tissue [1–3, 12–16, 18, 20, 21, 24]. We report for the first time on the expression of LOXL proteins in lesional and non-lesional skin of a patient with the reticular variant of MDE.
Keywords Mid-dermal elastolysis Lysyl oxidases Lysyl oxidase-like proteins Elastic tissue Materials and methods
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