The Experts below are selected from a list of 204 Experts worldwide ranked by ideXlab platform

Akira Ishibashi - One of the best experts on this subject based on the ideXlab platform.

  • elastin peptides induce migration and terminal differentiation of cultured keratinocytes via 67 kda elastin receptor in vitro 67 kda elastin receptor is expressed in the keratinocytes eliminating elastic materials in Elastosis Perforans Serpiginosa
    Journal of Investigative Dermatology, 2000
    Co-Authors: Norihiro Fujimoto, Shingo Tajima, Akira Ishibashi
    Abstract:

    To delineate the molecular mechanism of transepidermal elimination of dermal elastic materials in Elastosis Perforans Serpiginosa, the interaction between elastin and cultured keratinocytes was studied in vitro . Synthetic elastin peptide VGVAPG elicited chemotactic responses to the cultured keratinocytes at the dose of 10 −9  M. Treatment of keratinocytes with 10 −6 or 10 −5  M elastin peptides resulted in the suppression of cell growth and the increased expression of involucrin and transglutaminase-1, markers of terminal differentiation. When cultured keratinocytes were treated with the elastin peptides, the expression of 67 kDa elastin receptor was increased. The induction of terminal differentiation by elastin peptides was attenuated by the treatment with the combination of anti-67 kDa elastin receptor antibody. The results indicate that elastin is a potent inducer of migration and terminal differentiation of cultured keratinocytes, which is mediated by the 67 kDa elastin receptor. In the lesional skins of patients with Elastosis Perforans Serpiginosa, the 67 kDa elastin receptor was specifically expressed in the epidermis immediately surrounding the elastic materials that were being eliminated. The elastin receptor may be involved in the interaction between keratinocytes and elastin in Elastosis Perforans Serpiginosa.

Norihiro Fujimoto - One of the best experts on this subject based on the ideXlab platform.

  • elastin peptides induce migration and terminal differentiation of cultured keratinocytes via 67 kda elastin receptor in vitro 67 kda elastin receptor is expressed in the keratinocytes eliminating elastic materials in Elastosis Perforans Serpiginosa
    Journal of Investigative Dermatology, 2000
    Co-Authors: Norihiro Fujimoto, Shingo Tajima, Akira Ishibashi
    Abstract:

    To delineate the molecular mechanism of transepidermal elimination of dermal elastic materials in Elastosis Perforans Serpiginosa, the interaction between elastin and cultured keratinocytes was studied in vitro . Synthetic elastin peptide VGVAPG elicited chemotactic responses to the cultured keratinocytes at the dose of 10 −9  M. Treatment of keratinocytes with 10 −6 or 10 −5  M elastin peptides resulted in the suppression of cell growth and the increased expression of involucrin and transglutaminase-1, markers of terminal differentiation. When cultured keratinocytes were treated with the elastin peptides, the expression of 67 kDa elastin receptor was increased. The induction of terminal differentiation by elastin peptides was attenuated by the treatment with the combination of anti-67 kDa elastin receptor antibody. The results indicate that elastin is a potent inducer of migration and terminal differentiation of cultured keratinocytes, which is mediated by the 67 kDa elastin receptor. In the lesional skins of patients with Elastosis Perforans Serpiginosa, the 67 kDa elastin receptor was specifically expressed in the epidermis immediately surrounding the elastic materials that were being eliminated. The elastin receptor may be involved in the interaction between keratinocytes and elastin in Elastosis Perforans Serpiginosa.

O. M. Vanakker - One of the best experts on this subject based on the ideXlab platform.

  • Severe early-onset manifestations of pseudoxanthoma elasticum resulting from the cumulative effects of several deleterious mutations in ENPP1, ABCC6 and HBB: transient improvement in ectopic calcification with sodium thiosulfate
    British Journal of Dermatology, 2020
    Co-Authors: L. Omarjee, Y. Nitschke, S. Verschuere, E. Bourrat, M-d Vignon, N. Navasiolava, G. Leftheriotis, G. Kauffenstein, F. Rutsch, O. M. Vanakker
    Abstract:

    Pseudoxanthoma elasticum (PXE) is a rare disorder characterized by fragmentation and progressive calcification of elastic fibres in connective tissues. Overlap has been reported between the inherited PXE phenotype associated with ENPP1, ABCC6 or NT5E mutations and acquired PXE clinical manifestations associated with haemoglobinopathies induced by HBB mutations. No treatment is currently available for PXE. A young boy presented with severe early-onset systemic calcifications occurring in the skin as Elastosis Perforans Serpiginosa (EPS) and in the arteries, causing mesenteric and limb ischaemia. Analyses revealed deleterious ABCC6, ENPP1 and HBB mutations. The diagnosis of severe PXE was retained and we have coined the term 'PXE+ syndrome' to describe the cumulative effects of the various mutations in this uncommon phenotype. Given the severity, rapid progression and a potentially fatal prognosis, intravenous sodium thiosulfate (STS) was initiated at 25 g three times weekly for 6 months. Numerous side-effects prompted dosage adjustment to 10 g intravenously daily. Treatment efficacy was evaluated at 6 months. Asthaenia, anorexia and pre-/postprandial pain had subsided, entailing weight gain. Abdominal EPS had diminished. Calcific stenosis of the coeliac and mesenteric arteries was no longer detectable on arterial ultrasonography. Follow-up revealed only transient efficacy of STS. Discontinuation of treatment to evaluate the persistence of effects resulted in relapse of the initial symptomatology after 4 months. STS efficacy is conceivably due to strong antioxidant properties and chelation of calcium to form soluble calcium thiosulfate complexes. This case is suggestive of PXE+ syndrome for which STS may represent potential treatment in severe cases. What's already known about this topic? Generalized arterial calcification of infancy may occur in association with ABCC6 mutations and pseudoxanthoma elasticum (PXE) can be linked to ENPP1 mutations. A PXE-like phenotype has also been reported in a subset of patients with inherited haemoglobinopathies, namely sickle cell disease or beta-thalassaemia, related to HBB mutations. To date, there is still no cure for PXE. What does this study add? We report a severe case of PXE resulting from the cumulative effects of several deleterious mutations in ENPP1, ABCC6 and HBB. We suggest the term 'PXE+ syndrome' to describe such patients. Sodium thiosulfate therapy could represent a potential option in severe cases of PXE+ syndrome.

Mary Seabury Stone - One of the best experts on this subject based on the ideXlab platform.

  • penicillamine induced Elastosis of the mucosal lip
    Journal of The American Academy of Dermatology, 2009
    Co-Authors: Bethany K H Lewis, Peggy L Chern, Mary Seabury Stone
    Abstract:

    Long-term penicillamine therapy has been associated with alterations in dermal elastic tissue. Well-described associated dermatoses include pseudo-pseudoxanthoma elasticum, acquired cutis laxa, Elastosis Perforans Serpiginosa, and anetoderma. Histologically, "lumpy-bumpy"" or "bramble-bush"" morphologic changes of elastic fibers in the dermis are characteristic. Previous reports of these findings in normal-appearing skin and internal organs suggest a systemic elastolytic process. Here we report an unusual case of penicillamine-induced Elastosis affecting the mucosa of the lip with characteristic histologic features.

Shingo Tajima - One of the best experts on this subject based on the ideXlab platform.

  • elastin peptides induce migration and terminal differentiation of cultured keratinocytes via 67 kda elastin receptor in vitro 67 kda elastin receptor is expressed in the keratinocytes eliminating elastic materials in Elastosis Perforans Serpiginosa
    Journal of Investigative Dermatology, 2000
    Co-Authors: Norihiro Fujimoto, Shingo Tajima, Akira Ishibashi
    Abstract:

    To delineate the molecular mechanism of transepidermal elimination of dermal elastic materials in Elastosis Perforans Serpiginosa, the interaction between elastin and cultured keratinocytes was studied in vitro . Synthetic elastin peptide VGVAPG elicited chemotactic responses to the cultured keratinocytes at the dose of 10 −9  M. Treatment of keratinocytes with 10 −6 or 10 −5  M elastin peptides resulted in the suppression of cell growth and the increased expression of involucrin and transglutaminase-1, markers of terminal differentiation. When cultured keratinocytes were treated with the elastin peptides, the expression of 67 kDa elastin receptor was increased. The induction of terminal differentiation by elastin peptides was attenuated by the treatment with the combination of anti-67 kDa elastin receptor antibody. The results indicate that elastin is a potent inducer of migration and terminal differentiation of cultured keratinocytes, which is mediated by the 67 kDa elastin receptor. In the lesional skins of patients with Elastosis Perforans Serpiginosa, the 67 kDa elastin receptor was specifically expressed in the epidermis immediately surrounding the elastic materials that were being eliminated. The elastin receptor may be involved in the interaction between keratinocytes and elastin in Elastosis Perforans Serpiginosa.