Promising Perspective to Facioscapulohumeral Muscular Dystrophy Treatment: Nutraceuticals and Phytochemicals

2021 ◽  
Vol 19 ◽  
Author(s):  
Ceren Hangül ◽  
Sibel Berker Karaüzüm ◽  
Esra Küpeli Akkol ◽  
Devrim Demir-Dora ◽  
Zafer Çetin ◽  
...  

: Facioscapulohumeral muscular dystrophy (FSHD) is in the top three list of all dystrophies with an approximate 1:8000 incidence. It is not a life-threatening disease; however, progression of the disease extends over being wheel-chair bound. Despite some drug trials have been continuing, including DUX4 inhibition, TGF-ß inhibition and resokine which promote healthier muscle, there is not an applicable treatment option for FSHD today. Still, there is a need for new agent or agents to heal, to stop or at least to slow down the muscle wasting. Current FSHD studies with nutraceuticals as vitamin C, vitamin E, coenzyme Q10, zinc, selenium, and phytochemicals as curcumin or genistein, daidzein flavonoids provide promising treatment strategies. In this review we will present the clinical and molecular nature of FSHD and focus on nutraceuticals and phytochemicals that may alleviate FSHD. Via interconnection of impaired pathophysiological FSHD pathways together with nutraceuticals and phytochemicals in the light of literature, we present both studied and novel approaches that can contribute FSHD treatment.

2019 ◽  
Vol 10 (6) ◽  
pp. 1258-1265 ◽  
Author(s):  
Mauro Monforte ◽  
Francesco Laschena ◽  
Pierfrancesco Ottaviani ◽  
Maria Rosaria Bagnato ◽  
Anna Pichiecchio ◽  
...  

2020 ◽  
Vol 13 (9) ◽  
pp. dmm045369
Author(s):  
Tatianna Wai Ying Wong ◽  
Abdalla Ahmed ◽  
Grace Yang ◽  
Eleonora Maino ◽  
Sydney Steiman ◽  
...  

ABSTRACTDuchenne muscular dystrophy (DMD) is a life-threatening neuromuscular disease caused by the lack of dystrophin, resulting in progressive muscle wasting and locomotor dysfunctions. By adulthood, almost all patients also develop cardiomyopathy, which is the primary cause of death in DMD. Although there has been extensive effort in creating animal models to study treatment strategies for DMD, most fail to recapitulate the complete skeletal and cardiac disease manifestations that are presented in affected patients. Here, we generated a mouse model mirroring a patient deletion mutation of exons 52-54 (Dmd Δ52-54). The Dmd Δ52-54 mutation led to the absence of dystrophin, resulting in progressive muscle deterioration with weakened muscle strength. Moreover, Dmd Δ52-54 mice present with early-onset hypertrophic cardiomyopathy, which is absent in current pre-clinical dystrophin-deficient mouse models. Therefore, Dmd Δ52-54 presents itself as an excellent pre-clinical model to evaluate the impact on skeletal and cardiac muscles for both mutation-dependent and -independent approaches.


2019 ◽  
Vol 5 (3) ◽  
pp. 232-245
Author(s):  
Chuku Okorie ◽  
Kola Ajibesin ◽  
Adekunle Sanyaolu ◽  
Adeena Islam ◽  
Selciya Lamech ◽  
...  

Moringa oleifera (M. oleifera) is an angiosperm plant that is a member of the Moringaceae family. It is a natural plant that is native to the sub-Himalayan northern regions of India, Bangladesh, Pakistan, and Afghanistan. The plant grows abundantly throughout tropical and subtropical areas of the world. For several centuries, many cultures have utilized various parts of the moringa plant as traditional medicine to treat common illnesses and control life-threatening conditions such as hypertension (HTN), diabetes, hyperlipidemia, inflammation, etc. This article reviewed the current literature on the therapeutic benefits of M. oleifera on hypertension, primarily focusing on identifying the plant’s key components and its roles in hindering the common pathophysiological pathways associated with hypertension. The number of people living with HTN has been predicted to increase to 1.56 billion worldwide by 2025 in spite of the myriads of preventive and treatment strategies available today. Therefore, it would be of great value to explore alternative complementary ways of controlling high blood pressure. HTN is commonly defined as blood pressure equal to or higher than 140/90 mm Hg. HTN itself is not a disease condition and does not elicit specific symptoms, however, if left untreated for a long time, it can lead to complicated cardiovascular diseases such as angina, congestive heart failure, myocardial infarction as well as stroke and chronic kidney diseases. Primary hypertension is diagnosed when there is no known identifiable underlying cause for the onset of the condition. Secondary hypertension is diagnosed when there is evidence of a disease or disorder triggering the onset of the condition. It is apparent that understanding the role of M. oleifera in the management of hypertension would expand the valuable strategies for the control of this condition.


Author(s):  
Jayaraj R. ◽  
Veena G. Rao ◽  
Jyothi Nagalikar

Ducchen’s muscular dystrophy is most common X-linked recessive disorder affecting 30 in 100,000 live male births. The primary cause of this disease is mutations in Dystrophin gene which is essential for the structural and functional integrity of muscle. It is a progressive muscle wasting disease in which patients frequently develop contractures and lose the ability to walk between 6 and 12 years of age. With progressive disease most patients succumb to death from respiratory failure and cardiac dysfunction in their twenties. As this is a genetic disorder we can consider it as Adibala Pravritta Vyadhi. As Mamsa Kshaya is seen at some muscles and Mamsa Vriddhi at other this is an Avarana Vata Vyadhi. In both Upsthambha and Nirupasthmbha Vatavyadhi, Basthi is considered as prime choice of treatment. A Variety of Ksheerabasti in the form of Kalabasti is studied in this condition by taking subjective and objective parameters. As this has given better improvement with no adverse effects in the patient, it can be tried in large number of patients.


2021 ◽  
Vol 9 (6) ◽  
pp. 1311
Author(s):  
Xiuqin Chen ◽  
Eric Banan-Mwine Daliri ◽  
Akanksha Tyagi ◽  
Deog-Hwan Oh

The initiation and development of cariogenic (that is, caries-related) biofilms are the result of the disruption of homeostasis in the oral microenvironment. There is a daily accumulation of dental biofilm on the surface of teeth and its matrix of extracellular polymers supports the host in its defense against invading microbes, thus helping to achieve oral microbial homeostasis. However, the homeostasis can be broken down under certain circumstances such as during long-term exposure to a low pH environment which results in the dominance of acidogenic and acid-tolerating species in the dental biofilm and, thus, triggers the shift of harmless biofilm to an acidic one. This work aims to explore microbial diversity and the quorum sensing of dental biofilm and their important contributions to oral health and disease. The complex and multispecies ecosystems of the cariogenic biofilm pose significant challenges for the modulation of the oral microenvironment. Promising treatment strategies are those that target cariogenic niches with high specificity without disrupting the balance of the surrounding oral microbiota. Here, we summarized the recent advances in modulating cariogenic biofilm and/or controlling its pathogenic traits.


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