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Pharmaceutical Applications of Collagen
Published in Amit Kumar Nayak, Md Saquib Hasnain, Dilipkumar Pal, Natural Polymers for Pharmaceutical Applications, 2019
K. Sangeetha, A. V. Jisha Kumari, E. Radha, P. N. Sudha
The deficiency of collagen may bring about heritable disorders like Ehlers-Dalnos syndrome, Osteogenesis imperfecta, Stickler syndrome, Alport syndrome, Epidermolysis bullosa, Marfan syndrome and autoimmune disorders like Systemic lupus erythematosus, Systemic sclerosis, Oral submucous fibrosis (Rose et al., 2001). The collagen loss was eventually the reason for skin aging (wrinkling and disorganized appearance), as we grow older. Other than this some of the external causes for collagen loss includes severe exposure to the sun, pollution in the air (i.e., continuous exposure to particulate matters in air will leads to loss of collagen), and so on.
Gene Therapy in Tissue Engineering: Prospects and Challenges
Published in Rajesh K. Kesharwani, Raj K. Keservani, Anil K. Sharma, Tissue Engineering, 2022
It has been a long battle to ensure that stimulation of stem cells in situ should lead to specific regeneration and not to scar formation or teratogenesis. There are persistent challenges namely inability to sustain growth factors’ presence in the culture media for indefinite period of time, correct dosage, and lack of activity of recombinant factors. Thus, research nowadays is focused on overcoming the hurdles and transduce cell in vivo to bring about suitable regeneration processes by investigating “gene-activated matrices” using plasmids coding as delivery vehicles (Bonadio, 2000). Stems cells herald the backbone of tissue engineering, but they are also the critical targets for correcting any genetic defects. Many of the hazards caused by direct gene transfer in human are now been avoided by genetically modifying these cells ex vivo using viral and nonviral transducing agents (Asahara et al., 2000). Gene therapy is the answer to the successful cure of junctional epidermolysis bullosa, a debilitating skin disease by transducing epidermal cells (Dellambra et al., 2000). Undoubtedly, care should be taken to avoid mutagenesis by silencing the gene. Strategies such as site-directed mutagenesis and homologous recombination should be the crucial goal, though many intermediate methods can also be employed such as antisense RNA, RNA interference, and hammerhead and hairpin RNase(s) to preclude expression of mutant gene. Thus, in an attempt to revolutionize tissue engineering, frontiers of genetic engineering are being merged, so that reconstruction is now termed as “correction”. tissue engineering has a new definition as engineering of tissue function via stem cell-mediated gene therapy and holds tremendous prospects in clinical application. The next section of the chapter provides an understanding to the readers about gene therapy.
Investigation on skin-protective clothing that addresses needs of epidermolysis bullosa patients/children with epidermolysis bullosa and their parents
Published in The Journal of The Textile Institute, 2022
Ngan Yi Kitty Lam, Xue Luo, Li Li
Epidermolysis bullosa (EB) is an inherited skin disorder that leads to skin fragility and blistering. There are at least 25 subtypes of EB defined by clinical findings, and the three major types of EB are, simplex (EBS), junctional (JEB), and dystrophic (DEB). (1) EBS: blisters occur within the epidermis (outer layer of skin), on hands and feet, inheritance is autosomal dominant; (2) JEB: blisters develop within the upper portion of the dermoepidermal junction (second part of skin, the tissue area between the outer layer and deep layer of skin), inheritance is either autosomal dominant or recessive, caused death in early infancy; and (3) DEB: the blisters occur beneath the skin in the sublamina densa (the basement membrane area that attached to bones and blood vessels). Inheritance is autosomal dominant, and the disease causesdeath in early to middle adulthood (Eady & Tidman, 1983; Fine, 2007). Infants or younger children with EB are called “butterfly children” because their skin is as fragile as butterfly’s wings. Because there is no specific cure and therapy to help the patients, special wound care is very important. Previous studies on EB have focused on its possible causes and treatment. Studies have also been conducted to determine the origins of EB and find a potential cure, such as the use of intravenous immunoglobulin therapy and the transplantation of genetically modified epidermal stem cells (De Rosa et al., 2014; Gupta et al., 2012; Hirose et al., 2015; Murauer et al., 2015; Umegaki-Arao et al., 2014). However, the only real treatment for patients with EB is daily wound care and bandaging to maintain their skin integrity and avoid infection (Denyer, 2010; Pagliarello & Tabolli, 2010; van Scheppingen et al., 2008; Williams et al., 2011). Little attention has also been given to daily apparel-based issues in terms of friction from clothing, which causes wounds discomfort and blisters, therefore requiring immediate care. Consequently, it is important to examine the daily apparel worn by EB patients so as to alleviate their symptoms and suffering.