Informatics Publishing Limited

Mohd. Yaqub Khan ¹, Poonam Gupta ¹, Bipin Bihari ¹, Vinod Kumar Singh ¹, Sanjay Kumar Yadav ¹, Aparna Misra ¹

Affiliations
1 Saroj Institute of Technology & Management, Ahimamau P.O. Arjunganj, Sultanpur Road, Lucknow-226002, IN

Abstract

Epidermolysis bullosa (EB) is an inherited connective tissue disease causing blisters in the skin and mucosal membranes, with an incidence of 1/50,000. Its severity ranges from mild to lethal. The skin is extremely fragile: minor mechanical friction or trauma will separate the layers of the skin and form blisters. People with this condition have an increased risk of cancers of the skin, and many will eventually be diagnosed with it as a complication of the chronic damage done to the skin. The skin has three layers; the outermost layer is the epidermis, and the middle layer is the dermis. In individuals with healthy skin, there are protein anchors between the layers that prevent them from moving independently from one another (shearing). In people born with EB, these top skin layers lack the protein anchors that hold them together, and any action that creates friction between them (like rubbing or pressure) will create blisters and painful sores. Sufferers of EB have compared the sores with third-degree burns. Current clinical research at the University of Minnesota has included a bone marrow transplant to a 2-year-old child who is one of 2 brothers with EB. Sulforaphane, a compound found in broccoli, was found to reduce blistering in a mouse model to the point where affected pups could not be identified visually, when injected into pregnant mice (5 μmol/day = 0.9 mg) and applied topically to newborns (1 μmol/day = 0.2 mg in jojoba oil).

Keywords

Extremely Fragile, Protein Anchors, Broccoli.

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Shweta Shukla ¹, Hina Masroor ¹, Sarita Pandey ¹, Ramesh Chaurasiya ¹, Rahul Gupta ², Md. Arshad ³, Aparna Misra ⁴, Madhu Gupta ¹

Affiliations
1 Metabolic Bone Disease Research Lab, Department of Zoology, University of Lucknow, Lucknow 226 007, IN
2 Amity Institute of Biotechnology, Amity University Uttar Pradesh, Lucknow Campus, Lucknow 226 028, IN
3 Cellular and Molecular Endocrinology Lab, Department of Zoology, Aligarh Muslim University, Aligarh 202 002, IN
4 Department of Biochemistry, Era’s Lucknow Medical College and Hospital, Era University, Lucknow 226 003, IN

Abstract

Late diagnosis and relapse after chemotherapy or surgical resection in case of osteosarcoma always remain a challenge. With low five-year survival rates, disease recurrence and poor prognosis, a better understanding of the molecular pathways involved in the progression of osteosarcoma is a prerequisite. A deeper understanding of osteosarcoma pathogenesis had led to an increase in translational studies for the development of more safer and targetted drugs. Recently, novel therapeutics have emerged which can be promising in increasing survival rates among osteosarcoma patients with minimal drug resistance. The treatment strategies include combination therapy, hormone therapy, immunotherapy, stem cell transplant, targetted therapy miRNA, CRISPR/Cas9, epitherapeutics, etc. Therapeutics like immunomodulators, cytokines, cancer vaccines, monoclonal antibodies, etc. are the major advancements in modern translational medicine especially in patients with resi­stance towards conventional treatment regimens. The validation and application of these novel therapies in the context of osteosarcoma prognosis, prophylaxis, diagnosis and treatment are subject to further researches and outcomes of clinical trials.

Keywords

CRISPR/Cas9, immunotherapy, Onco-miRs, osteosarcoma, translational science.

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