Review
Dermatology
Leonie Huitema, Taylor Phillips, Vitali Alexeev, Olga Igoucheva
Summary: Hereditary epidermolysis bullosa is a mechanobullous skin fragility disorder characterized by skin blistering under mechanical stress, with no cure currently available. Recent data suggest systemic immunological defects in patients, particularly affecting antibacterial immunity.
EXPERIMENTAL DERMATOLOGY
(2021)
Editorial Material
Medicine, Research & Experimental
Christen L. Ebens
Summary: Recessive dystrophic epidermolysis bullosa (RDEB) is a genetic blistering skin disease caused by biallelic mutations in the COL7A1 gene. Clinical manifestations include fragile skin, pain, itch, high metabolic demand, and dermal fibrosis.
EMBO MOLECULAR MEDICINE
(2021)
Article
Dermatology
Vitali Alexeev, Leonie Huitema, Taylor Phillips, Rodrigo Cepeda, Diego de los Cobos, Regina Isabella Matus Perez, Mauricio Salas-Garza, Oscar R. Fajardo-Ramirez, Franziska Ringpfeil, Jouni Uitto, Julio Cesar Salas-Alanis, Olga Igoucheva
Summary: This study found that RDEB wounds and epithelial cells are frequently infected with Staphylococcus sp. and Pseudomonas sp. RDEB wound-associated T cells contain CD4(+) and CD8(+) peripheral memory T cells that respond to microbial antigens, and CD8(+) cytotoxic T lymphocytes can recognize and kill infected cells. However, in RDEB, immune suppression mediated by PD-1/Treg may inhibit adaptive T cell-mediated immunity.
EXPERIMENTAL DERMATOLOGY
(2022)
Review
Dermatology
K. S. Subramaniam, M. N. Antoniou, J. A. McGrath, S. M. Lwin
Summary: Epidermolysis bullosa (EB) is a group of inherited skin disorders with debilitating consequences. Recessive dystrophic EB (RDEB) is especially severe and can lead to chronic wounds and squamous cell carcinoma. Advances in molecular genetics and biotechnology have paved the way for gene and cell-based therapies for EB, and RDEB has become the focus of clinical trials. There is a global effort involving academia, industry, and patient organizations to develop targeted therapeutics for EB. Dermatologists should familiarize themselves with gene therapy and its applications.
BRITISH JOURNAL OF DERMATOLOGY
(2022)
Article
Dermatology
Christine Gretzmeier, Didier Pin, Johannes S. Kern, Mei Chen, David T. Woodley, Leena Bruckner-Tuderman, Mark P. de Souza, Alexander Nystroem
Summary: Recessive dystrophic epidermolysis bullosa (RDEB) is a genetic skin disease characterized by skin blistering and fibrosis. This study found that intravenous administration of recombinant C7 can reduce fibrosis in RDEB patients, and this treatment method is well-tolerated in mice and dogs.
JOURNAL OF INVESTIGATIVE DERMATOLOGY
(2022)
Article
Dermatology
S. J. Robertson, C. Prodinger, L. Liu, C. Skilbeck, G. Petrof, A. E. Martinez, J. E. Mellerio, D. T. Greenblatt
Summary: The rare inversa subtype of recessive dystrophic epidermolysis bullosa (RDEB-I) is characterized by predominant intertriginous skin blistering and marked mucosal involvement. This study found a higher prevalence of otological complications in RDEB-I patients than previously reported, and presented the first case of cholesteatoma in RDEB-I. Bone-anchored hearing aids (BAHA) and middle ear implants (MEI) were identified as safe and effective treatment options for hearing loss in RDEB-I patients.
CLINICAL AND EXPERIMENTAL DERMATOLOGY
(2022)
Article
Dermatology
C. L. Ebens, J. A. McGrath, J. A. Riedl, A. R. Keith, G. Lilja, S. Rusch, D. R. Keene, S. F. Tufa, M. J. Riddle, R. Shanley, A. E. Van Heest, J. Tolar
Summary: This study evaluated the effects of allogeneic epidermal skin grafts on wound healing and durability in patients with RDEB. The results showed significant reductions in wound area, successful fusion of epidermal grafts, and improvement in RDEB pseudosyndactyly.
BRITISH JOURNAL OF DERMATOLOGY
(2021)
Article
Biotechnology & Applied Microbiology
Jose Bonafont, Angeles Mencia, Esteban Chacon-Solano, Wai Srifa, Sriram Vaidyanathan, Rosa Romano, Marta Garcia, Rosario Hervas-Salcedo, Laura Ugalde, Blanca Duarte, Matthew H. Porteus, Marcela Del Rio, Fernando Larcher, Rodolfo Murillas
Summary: This study presents a gene-editing approach using CRISPR-Cas9 system to achieve gene correction in different cell types, showing therapeutic potential for RDEB.
Review
Biochemistry & Molecular Biology
Grace Tartaglia, Qingqing Cao, Zachary M. Padron, Andrew P. South
Summary: RDEB is a devastating skin blistering disease caused by mutations in the C7 gene, leading to rapid fibrosis development, chronic wounds, and squamous cell carcinoma. It serves as a model for understanding the molecular basis of fibrosis and rapidly developing aggressive cancer.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2021)
Article
Dermatology
Mohammad Reza Pourani, Hassan Vahidnezhad, Parvin Mansouri, Leila Youssefian, Azadeh Rakhshan, Behzad Hajimoradi, Fahimeh Abdollahimajd, Jouni Uitto
Summary: This case series reports the beneficial effects of losartan on RDEB as a potentially novel treatment. Losartan can improve the clinical features and severity of the disease, and enhance the quality of life for patients with RDEB.
DERMATOLOGIC THERAPY
(2022)
Article
Biotechnology & Applied Microbiology
Sung-Ah Hong, Song-Ee Kim, A-Young Lee, Gue-Ho Hwang, Jong Hoon Kim, Hiroaki Iwata, Soo-Chan Kim, Sangsu Bae, Sang Eun Lee
Summary: In this study, researchers identified and corrected pathogenic mutations in the COL7A1 gene using adenine base editors and prime editors in patients with recessive dystrophic epidermolysis bullosa (RDEB). The edited patient-derived skin equivalents showed positive outcomes in terms of C7 deposition and anchoring fibril formation, suggesting the feasibility of ex vivo gene editing as a potential treatment for RDEB.
Article
Biochemistry & Molecular Biology
Avina Rami, Lukasz Laczmanski, Jagoda Jackow-Nowicka, Joanna Jackow
Summary: A model for studying the development of RDEB-cSCC was established using cellular reprogramming and re-differentiation technology. RNA-seq analysis revealed distinct gene expression signatures and functional changes in RDEB-cSCC subjected to reprogramming and re-differentiation, offering a valuable tool to study cSCC and identify potential therapeutic targets for RDEB-cSCC.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2021)
Article
Biochemistry & Molecular Biology
Irina Gurevich, Pooja Agarwal, PeiPei Zhang, John A. Dolorito, Stacie Oliver, Henry Liu, Nicholas Reitze, Nikhil Sarma, Isin Sinem Bagci, Kunju Sridhar, Visesha Kakarla, Vamsi K. Yenamandra, Mark O'Malley, Marco Prisco, Sara F. Tufa, Douglas R. Keene, Andrew P. South, Suma M. Krishnan, M. Peter Marinkovich
Summary: This study evaluated the use of an engineered viral vector, B-VEC, for the treatment of recessive dystrophic epidermolysis bullosa (RDEB) patients with wounds. The results showed that B-VEC promotes wound healing and is well-tolerated.
Article
Dermatology
Yasushi Kikuchi, Tomoki Tamakoshi, Ryuichi Ishida, Ryosuke Kobayashi, Shiho Mori, Akemi Ishida-Yamamoto, Manabu Fujimoto, Yasufumi Kaneda, Katsuto Tamai
Summary: In this study, researchers developed an ex vivo gene therapy for recessive dystrophic epidermolysis bullosa (RDEB) using autologous mesenchymal stromal cells (MSCs). The gene-modified MSCs were injected into mice with type VII collagen deficiency, leading to continuous and widespread expression of type VII collagen. The therapy showed successful application in both early blistering skin and advanced ulcerative lesions in the RDEB mouse model.
JOURNAL OF INVESTIGATIVE DERMATOLOGY
(2023)
Article
Medicine, Research & Experimental
Sang Eun Lee, Seung-Ju Lee, Song-Ee Kim, Kinam Kim, Boyoung Cho, Kyounghwan Roh, Soo-Chan Kim
Summary: This study evaluated the safety and potential clinical efficacy of intravenous infusion of allogeneic human umbilical cord blood-derived mesenchymal stem cells in patients with Recessive Dystrophic Epidermolysis Bullosa (RDEB). The results showed that the treatment was well tolerated and led to transient clinical benefits, including improvements in wound conditions, pain, itch, and quality of life. Inflammatory skin conditions and C7 expression also showed some improvement after treatment.
Review
Biochemistry & Molecular Biology
Dejan Stojkovic, Marina Kostic, Marija Smiljkovic, Milena Aleksic, Perica Vasiljevic, Milos Nikolic, Marina Sokovic
CURRENT MEDICINAL CHEMISTRY
(2020)
Article
Dermatology
Tatjana Gazibara, Vesna Reljic, Slavenka Jankovic, Jelena Peric, Milos Nikolic, Natasa Maksimovic
Summary: This study examines the factors associated with the quality of life of children with atopic dermatitis after a one-year follow-up. It finds that children with less severe atopic dermatitis are more likely to improve their quality of life. The study also shows that lower initial severity of the disease and greater improvement after one year are associated with better quality of life.
INDIAN JOURNAL OF DERMATOLOGY VENEREOLOGY & LEPROLOGY
(2022)
Article
Biology
Jaclyn Smith, Yao Shi, Michael Benedikt, Milos Nikolic
Summary: In the field of biomedicine, there is a need for scalable data integration solutions due to limitations in large-scale data processing frameworks in analyzing complex data types. TraNCE is an automated framework designed to address the challenges of designing distributed analyses with complex biomedical data types, demonstrating its ability to outperform common alternatives.
Article
Rheumatology
Mirjana Gajic-Veljic, Branislav Lekic, Milos Nikolic, Jovan Lalosevic, Biljana Stojimirovic, Branka Bonaci-Nikolic
Summary: Evaluation of specificity, level, and avidity of antineutrophil cytoplasmic antibodies (ANCA) in systemic lupus erythematosus (SLE) showed that only LF-ANCA level correlated with SLE activity and standard serological markers, suggesting LF-ANCA as a promising serological marker for renal involvement in SLE. After follow-up, the number of LF-ANCA-positive patients decreased, indicating the potential use of LF-ANCA as a biomarker in SLE.
CLINICAL RHEUMATOLOGY
(2022)
Article
Polymer Science
Simonida Lj. Tomic, Jovana S. S. Vukovic, Marija M. Babic M. Radic, Vuk. V. Filipovic, Dubravka P. P. Zivanovic, Milos M. Nikolic, Jasmina Nikodinovic-Runic
Summary: Scaffolding biomaterials that combine different material types can be engineered to create hybrid scaffolds with advantageous properties for biomedical applications. In this research, a combination of Manuka honey and 2-hydroxyethyl methacrylate/gelatin hydrogel scaffolds was developed as a bioactive platform. The hybrid scaffolds showed desirable properties such as high porosity, pH- and temperature-dependent swelling, and good absorption capacity. In vitro studies demonstrated that the scaffolds had controlled degradation rates and exhibited biocompatibility with fibroblast and keratinocyte cell lines, with the highest Manuka honey content resulting in the best biocompatibility. These findings suggest that the synergy of Manuka honey and 2-hydroxyethyl methacrylate/gelatin hydrogel scaffolds holds promise for biomedical applications.
Article
Dermatology
Svetlana Popadic, Jovan Lalosevic, Branislav Lekic, Mirjana Gajic-Veljic, Branka Bonaci-Nikolic, Milos Nikolic
Summary: This study reviewed 86 cases of cutaneous mastocytosis in children and found that the majority of patients developed the disease within the first year of life. Maculopapular cutaneous mastocytosis/urticaria pigmentosa was the most common subtype. Follow-up results showed that most patients had improved symptoms and did not progress to systemic mastocytosis.
INTERNATIONAL JOURNAL OF DERMATOLOGY
(2023)
Article
Cell Biology
Verica Paunovic, Ljubica Vucicevic, Maja Misirkic Marjanovic, Vladimir Perovic, Biljana Ristic, Mihajlo Bosnjak, Milos Mandic, Danijela Stevanovic, Ljubica Harhaji-Trajkovic, Jovan Lalosevic, Milos Nikolic, Branka Bonaci-Nikolic, Vladimir Trajkovic
Summary: By measuring the levels of autophagy markers and cytokines in the blood of COVID-19 patients, it was found that the proinflammatory autophagy receptor p62 is reduced and is positively correlated with TNF, IL-10, IL-17, and IL-33. The SARS-CoV-2 proteins NSP5 and ORF3a can affect the levels of p62, which in turn impacts the inflammatory response. These findings reveal the interplay between autophagy and inflammation in COVID-19.
Article
Chemistry, Medicinal
Nikola Nedeljkovic, Vladimir Dobricic, Jelena Boskovic, Marina Vesovic, Jovana Bradic, Marijana Andic, Aleksandar Kocovic, Nevena Jeremic, Jovana Novakovic, Vladimir Jakovljevic, Zorica Vujic, Milos Nikolic
Summary: The aim of the study was to synthesize and investigate the dose-dependent anti-inflammatory effect of new thiourea derivatives of naproxen with selected aromatic amines and esters of aromatic amino acids. The in vivo study demonstrated that derivatives of m-anisidine (4) and N-methyl tryptophan methyl ester (7) showed the most potent anti-inflammatory activity four hours after injection of carrageenan, with inhibition percentages of 54.01% and 54.12% respectively. In vitro assays of COX-2 inhibition indicated that none of the compounds tested achieved 50% inhibition at concentrations lower than 100 mu M. On the other hand, the aromatic amine derivatives (1-5) exhibited significant inhibition of 5-LOX, with compound 4 showing the lowest IC50 value of 0.30 mu M. Therefore, compound 4, with its high anti-edematous activity and potent inhibition of 5-LOX, holds promise as a potential anti-inflammatory agent.
Article
Dermatology
Jovan Lalosevic, Mirjana Gajic-Veljic, Jelena Lalosevic Misovic, Milos Nikolic
Summary: Alopecia areata is an autoimmune disease in which zinc deficiency may play a role. Lower serum zinc levels were found in patients with alopecia areata and were negatively correlated with disease severity. Therefore, serum zinc assessment is necessary for patients with alopecia areata.
ACTA DERMATO-VENEREOLOGICA
(2023)
Article
Dermatology
Phillip Aitken, Ioana Stanescu, Laura Boddington, Caroline Mahon, Andras Fogarasi, Yi-Hua Liao, Marta Ivars, Ester Moreno-Artero, Doris Trauner, Steven T. DeRoos, Jasna Jancic, Milos Nikolic, Patricia Balazova, Harper N. Price, Kinga Hadzsiev, Kate Riney, Stacie Stapleton, Megha M. Tollefson, Derek Bauer, Blanka Pinkova, Hartley Atkinson
Summary: We conducted a phase II/III double-blind randomized placebo-controlled trial to evaluate the efficacy and safety of a novel rapamycin cream for treating facial angiofibromas. The topical rapamycin treatment showed significant improvement in the appearance of angiofibromas over a 26-week period and had a good safety profile.
BRITISH JOURNAL OF DERMATOLOGY
(2023)
Letter
Dermatology
Marko Demenj, Maja Vilotijevic, Snezana Minic, Mirjana Milinkovic Sreckovic, Dusan Skiljevic, Dubravka Zivanovic, Svetlana Popadic, Milos Nikolic
JOURNAL OF THE EUROPEAN ACADEMY OF DERMATOLOGY AND VENEREOLOGY
(2023)
Article
Computer Science, Theory & Methods
Ahmet Kara, Milos Nikolic, Dan Olteanu, Haozhe Zhang
Summary: This paper investigates the trade-offs in static and dynamic evaluation of hierarchical queries with arbitrary free variables. The trade-off in the static setting is between the time to compute the query result partially and the delay required to enumerate its tuples. In the dynamic setting, the time needed to update the query result under single-tuple inserts or deletes is also considered. The approach used in this research takes into account the degree of values in the database and applies different computation and maintenance strategies for high-degree and low-degree values.
LOGICAL METHODS IN COMPUTER SCIENCE
(2023)
Review
Agricultural Economics & Policy
Biljana Ilic, Gordana Djukic, Milos Nikolic
Summary: This paper investigates employee satisfaction in rural tourism in Eastern Serbia, focusing on motivating factors for employees in smaller rural households. Through the use of survey questionnaires and statistical methods, the research results can provide recommendations for managers of rural households on how to best motivate their employees.
EKONOMIKA POLJOPRIVREDA-ECONOMICS OF AGRICULTURE
(2022)
Article
Computer Science, Information Systems
Jaclyn Smith, Michael Benedikt, Brandon Moore, Milos Nikolic
Summary: Nested relational query languages are attractive for handling large hierarchical datasets, and TraNCE is a new system that processes nested data through a compiler and additional components for creating and visualizing queries, as well as data exploration and notebook execution targets. These techniques support the construction of large-scale data science applications.
PROCEEDINGS OF THE VLDB ENDOWMENT
(2021)
Meeting Abstract
Biophysics
Milos Nikolic, Giuliano Scarcelli, Kandice Tanner
BIOPHYSICAL JOURNAL
(2021)