Marked to Die-Cell Death Mechanisms for Keratinocyte Acantholysis in Pemphigus Diseases
Pemphigus is a group of blistering autoimmune diseases causing painful skin lesions, characterized by acantholysis and by the production of autoantibodies against, mainly, adhesion proteins. We reviewed the literature for molecules and/ or features involved in the 12 cell death pathways described by...
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MDPI AG
2022-02-01
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| author | Valéria Bumiller-Bini Hoch Larissa Schneider Anna Elisabeth Pumpe Emelie Lüders Jennifer Elisabeth Hundt Angelica Beate Winter Boldt |
| author_facet | Valéria Bumiller-Bini Hoch Larissa Schneider Anna Elisabeth Pumpe Emelie Lüders Jennifer Elisabeth Hundt Angelica Beate Winter Boldt |
| author_sort | Valéria Bumiller-Bini Hoch |
| collection | DOAJ |
| description | Pemphigus is a group of blistering autoimmune diseases causing painful skin lesions, characterized by acantholysis and by the production of autoantibodies against, mainly, adhesion proteins. We reviewed the literature for molecules and/ or features involved in the 12 cell death pathways described by Nomenclature Committee on Cell Death, taking place in pemphigus patients, cell lines, or human skin organ cultures treated with sera or IgG from pemphigus patients or in pemphigus mouse models, and found 61 studies mentioning 97 molecules involved in cell death pathways. Among the molecules, most investigated were pleiotropic molecules such as TNF and CASP3, followed by FASL and CASP8, and then by FAS, BAX, BCL2, and TP53, all involved in more than one pathway but interpreted to function only within apoptosis. Most of these previous investigations focused only on apoptosis, but four recent studies, using TUNEL assays and/or electron microscopy, disqualified this pathway as a previous event of acantholysis. For PV, apoptolysis was suggested as a cell death mechanism based on pathogenic autoantibodies diversity, mitochondrial dysfunction, and p38 MAPK signaling. To answer those many questions that remain on cell death and pemphigus, we propose well-controlled, statistically relevant investigations on pemphigus and cell death pathways besides apoptosis, to overcome the challenges of understanding the etiopathology of pemphigus diseases. |
| first_indexed | 2024-03-09T13:35:23Z |
| format | Article |
| id | doaj.art-bef9f379d65047b1931221c194752fc9 |
| institution | Directory Open Access Journal |
| issn | 2075-1729 |
| language | English |
| last_indexed | 2024-03-09T13:35:23Z |
| publishDate | 2022-02-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Life |
| spelling | doaj.art-bef9f379d65047b1931221c194752fc92023-11-30T21:13:10ZengMDPI AGLife2075-17292022-02-0112332910.3390/life12030329Marked to Die-Cell Death Mechanisms for Keratinocyte Acantholysis in Pemphigus DiseasesValéria Bumiller-Bini Hoch0Larissa Schneider1Anna Elisabeth Pumpe2Emelie Lüders3Jennifer Elisabeth Hundt4Angelica Beate Winter Boldt5Laboratory of Human Molecular Genetics, Department of Genetics, Federal University of Paraná, Curitiba 81531-980, BrazilLaboratory of Human Molecular Genetics, Department of Genetics, Federal University of Paraná, Curitiba 81531-980, BrazilLübeck Institute of Experimental Dermatology, University of Lübeck, 23562 Lübeck, GermanyLübeck Institute of Experimental Dermatology, University of Lübeck, 23562 Lübeck, GermanyLübeck Institute of Experimental Dermatology, University of Lübeck, 23562 Lübeck, GermanyLaboratory of Human Molecular Genetics, Department of Genetics, Federal University of Paraná, Curitiba 81531-980, BrazilPemphigus is a group of blistering autoimmune diseases causing painful skin lesions, characterized by acantholysis and by the production of autoantibodies against, mainly, adhesion proteins. We reviewed the literature for molecules and/ or features involved in the 12 cell death pathways described by Nomenclature Committee on Cell Death, taking place in pemphigus patients, cell lines, or human skin organ cultures treated with sera or IgG from pemphigus patients or in pemphigus mouse models, and found 61 studies mentioning 97 molecules involved in cell death pathways. Among the molecules, most investigated were pleiotropic molecules such as TNF and CASP3, followed by FASL and CASP8, and then by FAS, BAX, BCL2, and TP53, all involved in more than one pathway but interpreted to function only within apoptosis. Most of these previous investigations focused only on apoptosis, but four recent studies, using TUNEL assays and/or electron microscopy, disqualified this pathway as a previous event of acantholysis. For PV, apoptolysis was suggested as a cell death mechanism based on pathogenic autoantibodies diversity, mitochondrial dysfunction, and p38 MAPK signaling. To answer those many questions that remain on cell death and pemphigus, we propose well-controlled, statistically relevant investigations on pemphigus and cell death pathways besides apoptosis, to overcome the challenges of understanding the etiopathology of pemphigus diseases.https://www.mdpi.com/2075-1729/12/3/329pemphiguscell death pathwaysapoptosisapoptolysis |
| spellingShingle | Valéria Bumiller-Bini Hoch Larissa Schneider Anna Elisabeth Pumpe Emelie Lüders Jennifer Elisabeth Hundt Angelica Beate Winter Boldt Marked to Die-Cell Death Mechanisms for Keratinocyte Acantholysis in Pemphigus Diseases Life pemphigus cell death pathways apoptosis apoptolysis |
| title | Marked to Die-Cell Death Mechanisms for Keratinocyte Acantholysis in Pemphigus Diseases |
| title_full | Marked to Die-Cell Death Mechanisms for Keratinocyte Acantholysis in Pemphigus Diseases |
| title_fullStr | Marked to Die-Cell Death Mechanisms for Keratinocyte Acantholysis in Pemphigus Diseases |
| title_full_unstemmed | Marked to Die-Cell Death Mechanisms for Keratinocyte Acantholysis in Pemphigus Diseases |
| title_short | Marked to Die-Cell Death Mechanisms for Keratinocyte Acantholysis in Pemphigus Diseases |
| title_sort | marked to die cell death mechanisms for keratinocyte acantholysis in pemphigus diseases |
| topic | pemphigus cell death pathways apoptosis apoptolysis |
| url | https://www.mdpi.com/2075-1729/12/3/329 |
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