scholarly journals Potential Role of the Mitochondria for the Dermatological Treatment of Papillon-Lefèvre

Antioxidants ◽  
2021 ◽  
Vol 10 (1) ◽  
pp. 95
Author(s):  
Beatriz Castejón-Vega ◽  
Maurizio Battino ◽  
José L. Quiles ◽  
Beatriz Bullon ◽  
Mario D. Cordero ◽  
...  

The Papillon–Lefèvre syndrome (PLS) is a rare autosomal recessive disorder caused by mutations in the Cathepsin C (CTSC) gene, characterized by periodontitis and palmoplantar hyperkeratosis. The main inflammatory deficiencies include oxidative stress and autophagic dysfunction. Mitochondria are the main source of reactive oxygen species; their impaired function is related to skin diseases and periodontitis. The mitochondrial function has been evaluated in PLS and mitochondria have been targeted as a possible treatment for PLS. We show for the first time an important mitochondrial dysfunction associated with increased oxidative damage of mtDNA, reduced CoQ10 and mitochondrial mass and aberrant morphologies of the mitochondria in PLS patients. Mitochondrial dysfunction, determined by oxygen consumption rate (OCR) in PLS fibroblasts, was treated with CoQ10 supplementation, which determined an improvement in OCR and a remission of skin damage in a patient receiving a topical administration of a cream enriched with CoQ10 0.1%. We provide the first evidence of the role of mitochondrial dysfunction and CoQ10 deficiency in the pathophysiology of PLS and a future therapeutic option for PLS.

Molecules ◽  
2021 ◽  
Vol 26 (6) ◽  
pp. 1651
Author(s):  
Kippeum Lee ◽  
Hyeon Ji Kim ◽  
Soo A Kim ◽  
Soo-Dong Park ◽  
Jae-Jung Shim ◽  
...  

Skin aging occurs inevitably as a natural result of physiological changes over time. In particular, solar exposure of the skin accounts for up to 90% of skin damage. Numerous studies have examined the ability of dietary constituents to prevent skin aging, and recent research has emphasized the role of functional probiotics in intestinal function and skin aging. However, the mechanism of the interactions between aging and probiotics has not been elucidated yet. The aim of this study was to determine the role of exopolysaccharides (EPS) produced by lactic acid bacteria (LAB) identified as Lactobacillus plantarum HY7714 in regulating tight junctions in intestinal epithelial cells and increasing moisture retention in human dermal fibroblasts cells. We observed that HY7714 EPS controlled intestinal tight junctions in Caco-2 cells by upregulating the genes encoding occludin-1 (OCL-1) and zonula occluden-1 (ZO-1). In addition, HY7714 EPS effectively improved UVB-induced cytotoxicity and hydration capacity in HS68 cells by downregulating production of metalloproteinases (MMPs) and reactive oxygen species (ROS). In summary, HY7714 EPS is an effective anti-aging molecule in skin and may have therapeutic potential against skin diseases and UVB-induced damage. Therefore, HY7714 EPS serves as a functional substance in skin–gut axis communication.


Author(s):  
Sushree Sovana ◽  
Paquirissamy Oudeacoumar ◽  
Ramachandran Niranjjan ◽  
Saurabh Krishna Misra

<p class="abstract"><strong>Background:</strong> Fishermen always work and reside in environmental conditions that are harmful to their health. The primary objective of this study was to evaluate the magnitude of skin diseases among fishermen and the secondary objective was to assess the knowledge, attitude and practices related to skin diseases and their prevention.</p><p class="abstract"><strong>Methods:</strong> This was a cross-sectional study carried out among 420 fishermen and women belonging to fishing area of Puducherry for a period of three months from December 2018 to February 2019.<strong></strong></p><p class="abstract"><strong>Results:</strong> The majority of the study populations (26.67%) were in the age group of 30-40 years. The majority were males (1.43%), 88.57% were married, 16.19% were illiterate whereas 42.86% studied up to the secondary school level. The majority (54.29%) were working for more than 12 hours daily. The majority (30.48%) were in the fishing occupation for more than 20 years. The non-infectious skin disorders were: sunburns (25.71%), palmoplantar hyperkeratosis (19.52%), melasma (14.05%), acute urticaria (7.85%), photoaging (2.86%), wrinkles (2.14%) and freckels (1.90%). Among infections, 10.48% had tinea infections followed by pityriasis versicolor in 7.38%, bacterial infection in 3.57%, onychomycosis and warts in 1.19% each respectively and all the study population had only one type of infection. All men in the study were not using sunscreens and any protective wares and they were not aware of sun induced skin damage.</p><p class="abstract"><strong>Conclusions:</strong> The most common diseases encountered were sunburn, palmoplantar hyperkeratosis, melasma, acute urticaria, fungal infections, bacterial infection and photoaging. The fishermen were not using sunscreens and protective wares and they were unaware of solar skin damage.</p>


Diagnostics ◽  
2020 ◽  
Vol 10 (2) ◽  
pp. 107 ◽  
Author(s):  
Shu-Hua Kuo ◽  
Ching-Ju Shen ◽  
Ching-Fen Shen ◽  
Chao-Min Cheng

As a highly influential physiological factor, pH may be leveraged as a tool to diagnose physiological state. It may be especially suitable for diagnosing and assessing skin structure and wound status. Multiple innovative and elegant smart wound dressings combined with either pH sensors or drug control-released carriers have been extensively studied. Increasing our understanding of the role of pH value in clinically relevant diagnostics should assist clinicians and improve personal health management in the home. In this review, we summarized a number of articles and discussed the role of pH on the skin surface as well as the factors that influence skin pH and pH-relevant skin diseases, but also the relationship of skin pH to the wound healing process, including its influence on the activity of proteases, bacterial enterotoxin, and some antibacterial agents. A great number of papers discussing physiological pH value have been published in recent decades, far too many to be included in this review. Here, we have focused on the impact of pH on wounds and skin with an emphasis on clinically relevant diagnosis toward effective treatment. We have also summarized the differences in skin structure and wound care between adults and infants, noting that infants have fragile skin and poor skin barriers, which makes them more vulnerable to skin damage and compels particular care, especially for wounds.


2020 ◽  
Vol 29 (19) ◽  
pp. 3296-3311
Author(s):  
Pilar González-García ◽  
Agustín Hidalgo-Gutiérrez ◽  
Cristina Mascaraque ◽  
Eliana Barriocanal-Casado ◽  
Mohammed Bakkali ◽  
...  

Abstract Abnormalities of one carbon, glutathione and sulfide metabolisms have recently emerged as novel pathomechanisms in diseases with mitochondrial dysfunction. However, the mechanisms underlying these abnormalities are not clear. Also, we recently showed that sulfide oxidation is impaired in Coenzyme Q10 (CoQ10) deficiency. This finding leads us to hypothesize that the therapeutic effects of CoQ10, frequently administered to patients with primary or secondary mitochondrial dysfunction, might be due to its function as cofactor for sulfide:quinone oxidoreductase (SQOR), the first enzyme in the sulfide oxidation pathway. Here, using biased and unbiased approaches, we show that supraphysiological levels of CoQ10 induces an increase in the expression of SQOR in skin fibroblasts from control subjects and patients with mutations in Complex I subunits genes or CoQ biosynthetic genes. This increase of SQOR induces the downregulation of the cystathionine β-synthase and cystathionine γ-lyase, two enzymes of the transsulfuration pathway, the subsequent downregulation of serine biosynthesis and the adaptation of other sulfide linked pathways, such as folate cycle, nucleotides metabolism and glutathione system. These metabolic changes are independent of the presence of sulfur aminoacids, are confirmed in mouse models, and are recapitulated by overexpression of SQOR, further proving that the metabolic effects of CoQ10 supplementation are mediated by the overexpression of SQOR. Our results contribute to a better understanding of how sulfide metabolism is integrated in one carbon metabolism and may explain some of the benefits of CoQ10 supplementation observed in mitochondrial diseases.


2020 ◽  
Vol 21 (18) ◽  
pp. 6695 ◽  
Author(s):  
Iain Hargreaves ◽  
Robert A. Heaton ◽  
David Mantle

Coenzyme Q10 (CoQ10) has a number of vital functions in all cells, both mitochondrial and extramitochondrial. In addition to its key role in mitochondrial oxidative phosphorylation, CoQ10 serves as a lipid soluble antioxidant, plays an important role in fatty acid, pyrimidine and lysosomal metabolism, as well as directly mediating the expression of a number of genes, including those involved in inflammation. In view of the central role of CoQ10 in cellular metabolism, it is unsurprising that a CoQ10 deficiency is linked to the pathogenesis of a range of disorders. CoQ10 deficiency is broadly classified into primary or secondary deficiencies. Primary deficiencies result from genetic defects in the multi-step biochemical pathway of CoQ10 synthesis, whereas secondary deficiencies can occur as result of other diseases or certain pharmacotherapies. In this article we have reviewed the clinical consequences of primary and secondary CoQ10 deficiencies, as well as providing some examples of the successful use of CoQ10 supplementation in the treatment of disease.


Author(s):  
Krishna Prasad Lamichhane ◽  
Shaili Pradhan ◽  
Ranjita Shreshta Gorkhali ◽  
Pramod Kumar Koirala

Papillon-Lefèvre syndrome (PLS) is a rare autosomal recessive disorder associated with rapidly progressing periodontitis leading to premature loss of deciduous and permanent dentition and diffuse palmoplantar keratosis. Immunologic alterations, genetic mutations, and role of bacteria are some aetiologic factors. Patients present with early periodontal destruction, so periodontists play a significant role in diagnosis and management. This paper reports a case of Papillon- Lefevre syndrome with its clinical manifestations and challenges for periodontal management which was diagnosed in dental department.


2020 ◽  
pp. 19-23
Author(s):  
Lilia Shagvaleeva

The article discloses the role of the medical sister of the oncological department in the creation of a positive psychological microclimate, which is the key to the successful treatment and rehabilitation of the oncological patient. Experience of organization of nursing process in the department of tumors of external localizations is presented.


2020 ◽  
pp. 64-70
Author(s):  
Anastasiya Laknitskaya

Currently, one of the priority medical and social problems is the optimization of treatment methods for pyoderma associated with Streptococcus pyogenes — group A streptococcus (GAS). To date, the proportion of pyoderma, the etiological factor of which is Streptococcus pyogenes, is about 6 % of all skin diseases and is in the range from 17.9 to 43.9 % of all dermatoses. Role of the bacterial factor in the development of streptococcal pyoderma is obvious. Traditional treatment complex includes antibacterial drugs selected individually, taking into account the antibiotic sensitivity of pathognomonic bacteria, and it is not always effective. Currently implemented immunocorrection methods often do not take into account specific immunological features of the disease, the individual, and the fact that the skin performs the function of not only a mechanical barrier, but it is also an immunocompetent organ. Such an approach makes it necessary to conduct additional studies clarifying the role of factors of innate and adaptive immunity, intercellular mediators and antioxidant defense system, that allow to optimize the treatment of this pathology.


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