scholarly journals Pathogenesis of Bone Alterations in Gaucher Disease: The Role of Immune System

2015 ◽  
Vol 2015 ◽  
pp. 1-6 ◽  
Author(s):  
Juan Marcos Mucci ◽  
Paula Rozenfeld

Gaucher, the most prevalent lysosomal disorder, is an autosomal recessive inherited disorder due to a deficiency of glucocerebrosidase. Glucocerebrosidase deficiency leads to the accumulation of glucosylceramide primarily in cells of mononuclear-macrophage lineage. Clinical alterations are visceral, hematological, and skeletal. Bone disorder in Gaucher disease produces defects on bone metabolism and structure and patients suffer from bone pain and crisis. Skeletal problems include osteopenia, osteoporosis, osteolytic lesions, and osteonecrosis. On the other hand a chronic stimulation of the immune system is a well-accepted hallmark in this disease. In this review we summarize the latest findings in the mechanisms leading to the bone pathology in Gaucher disease in relationship with the proinflammatory state.

2013 ◽  
Vol 2013 ◽  
pp. 1-6 ◽  
Author(s):  
Maria Felicia Faienza ◽  
Annamaria Ventura ◽  
Flaviana Marzano ◽  
Luciano Cavallo

In the last years, new evidences of the relationship between immune system and bone have been accumulated both in animal models and in humans affected by bone disease, such as rheumatoid arthritis, bone metastasis, periodontitis, and osteoporosis. Osteoporosis is characterized by low bone mass and microarchitectural deterioration of bone tissue with a subsequent increase in bone fragility and susceptibility to fractures. The combined effects of estrogen deprivation and raising of FSH production occurring in menopause cause a marked stimulation of bone resorption and a rapid bone loss which is central for the onset of postmenopausal osteoporosis. This review focuses on the role of immune system in postmenopausal osteoporosis and on therapeutic strategies targeting osteoimmunology pathways.


Immunotherapy ◽  
2020 ◽  
Vol 12 (11) ◽  
pp. 833-855 ◽  
Author(s):  
Mohadeseh Haji Abdolvahab ◽  
Behrad Darvishi ◽  
Mohammad Zarei ◽  
Keivan Majidzadeh-A ◽  
Leila Farahmand

Interferons (IFNs) are a group of signaling cytokines, secreted by host cells to induce protection against various disorders. IFNs can directly impact on tumor cells or indirectly induce the immune system to protect host cells. The expression levels of IFNs and its functions of are excellently modulated in a way to protect host cells from probable toxicities caused by extreme responses. The efficacy of anticancer therapies is correlated to IFNs signaling. Although IFN signaling is involved in induction of antitumor responses, chronic stimulation of the IFN signaling pathway can induce resistance to various antineoplasm therapies. Hence, IFNs are expressed by both cancer and immune cells, and modulate their biological function. Understanding this mechanism of action might be a key target of combination therapies.


2020 ◽  
Vol 16 ◽  
Author(s):  
Behnam Emamgolizadeh Gurt Tapeh ◽  
Mohammad Sadegh Hashemzadeh ◽  
Ali Mir Hoseini

Aims: Encouraging results have been indicated preclinically and in patients using the bacterial super antigen. This review article intends to summarize the role of the super antigens that have been recently used in the treatment of cancer. In addition, the vector systems including lentiviral vectors, adeno-associated vector systems and retroviral vectors that are increasingly being used in basic and applied research were discussed. Most importantly, the new CRISPR technique has also been discussed in this literature review. Discussion: More successful therapies can be achieved by manipulating bacterial vector systems through incorporating genes related to the super antigens and cytokines. The products of SAg and cytokine genes contributes to the strong stimulation of immune system against tumor cells. They bind to MHC II molecules as well as the V beta regions of TCR and lead to the production of IL2 and other cytokines, the activation of antigen-presenting cells and T lymphocytes. Additionally, super antigens can be used to eradicate tumor cells. Better results in cancer treatment can be achieved by transferring super antigen genes and subsequent strong immune stimulation along with other cancer immunotherapy agents. Conclusion: Super antigens induce the proliferation of T lymphocytes and antigen-presenting cells by binding to MHCII molecules and V beta regions in T cell receptors. Therefore, the presentation of tumor cell antigens is increased. Additionally, the production of important cytokines by T cells and APCs contributes to the stimulation of immune response against tumor cells. The manipulation of bacterial vector systems through incorporating genes related to SAgs and other immune response factors is a good strategy for immune system stimulating and eradicating of tumor cells along with other immunotherapy agents.


2016 ◽  
Vol 9 (1) ◽  
pp. 12-21 ◽  
Author(s):  
William F. Finn

The clinical manifestations and consequence of acute and chronic gout are closely associated with the activation of the innate immune system, stimulation of the NLP3 inflammasome and secretion of interleukin-1β and interleukin-18 via caspace-1 activity. This leads to cytokine release and an inflammatory response. It is now clear that a similar involvement of the innate immune system occurs in many forms of acute and chronic kidney disease with accentuation of renal tubular injury and stimulation of tubulointerstitial fibrosis. The local and systemic activation of the innate immune system may help explain the close association of these conditions and provide a target for therapeutic interdiction.


Author(s):  
A V Pechersky ◽  
V I Pechersky ◽  
E S Shpilenya ◽  
A H Gaziev ◽  
V F Semiglazov

Introduction. The cicatrix as the local site of a sclerosis, replaces wound defect or focus of a necrosis. Lead to cicatrization various type of a trauma, including operational, and also some of diseases. Cicatrization quite often leads to stricture and other complications.Materials and methods. The methodology of stimulation of regeneration for reduction of intensity of cicatrization of the damaged tissues is shown on the example of the patient from a stricture urethra.Results. In 5 months after an endoscopic urethrotomy of a stricture of bulbous part of urethra of 3 mm and the developed recurrence - the stricture of bigger length - 5 mm the repeated endoscopic urethrotomy was carried out. In 5 months after carrying out a repeated endoscopic urethrotomy and the beginning of the complex treatment directed on stimulation of regeneration and prevention of formation of cicatricial tissue according to a control urethrography the bulbous part of urethra was passable.Conclusion. Complex stimulation of regeneration taking into account a role of immune system in this process, on an equal basis with purpose of proteolytic ferment preparations, can be used for reduction of expressiveness of sclerosis of the damaged tissues.


2009 ◽  
Vol 297 (6) ◽  
pp. R1742-R1748 ◽  
Author(s):  
LaShon C. Sturgis ◽  
Joseph G. Cannon ◽  
Derek A. Schreihofer ◽  
Michael W. Brands

Knockout (KO) of IL-6 has been shown to attenuate ANG II hypertension, and mineralocorticoid receptors (MR) have been reported to contribute to the increase in IL-6 during acute ANG II infusion. This study determined whether that MR action is sustained with chronic ANG II infusion and whether it plays a role in mediating ANG II hypertension. ANG II infusion (90 ng/min) increased plasma IL-6 from 1.6 ± 0.6 to 22.7 ± 2.2 and 19.9 ± 3.2 pg/ml on days 7 and 14, respectively, and chronic MR blockade with spironolactone attenuated that only at day 7 (7.2 ± 2.2 pg/ml). ANG II increased MAP (19 h/day with telemetry) ∼40 mmHg, but in ANG II+spironolactone mice (25 or 50 mg·kg−1·day−1), mean arterial pressure (MAP) was not significantly different despite a tendency for lower pressure the first 6 days. To isolate further the mineralocorticoid link to IL-6 and blood pressure, DOCA-salt hypertension was induced in IL-6 KO and wild-type (WT) mice. Plasma IL-6 increased from 4.1 ± 1.7 to 34.5 ± 7.0 pg/ml by day 7 of DOCA treatment in the WT mice but was back to control levels by day 14. An IL-6 bioassay using the murine B9, B-cell hybridoma cell line demonstrated that plasma IL-6 measurements reflected actual IL-6 bioactivity. The hypertension was not different and virtually superimposable in WT vs. IL-6 KO mice, averaging 145 ± 2 and 144 ± 3 mmHg, respectively. Both experiments confirm chronic stimulation of IL-6 by mineralocorticoids but show that it is transient. In addition, IL-6 was not required for mineralocorticoid hypertension. This suggests that aldosterone contributes to the increase in plasma IL-6 in the early stage of ANG II hypertension but that the blood pressure actions of IL-6 in that model are linked most likely to ANG II rather than aldosterone.


2019 ◽  
Vol 11 (2) ◽  
pp. 79-86
Author(s):  
Cindy Ayustin Noya ◽  
Angkit Kinasih ◽  
Venti Agustina ◽  
R.Rr Maria Dyah Kurniasari

Infeksi saluran pernafasan akut atau yang sering disebut ISPA merupakan infeksi pada saluran pernafasan baik saluran pernafasan atas atau bawah.ISPA juga kebanyakan terjadi pada anak balita karena daya tahan tubuh mereka tidak kuat dalam menghadapi penyakit ISPA. ISPA mengakibatkan kematiansekitar15%-20% per tahun pada usia balita di Negara berkembang. Tujuan penelitian ini adalah untuk mengetahui dan menganalisa peran ibu dalam meningkatkan sistem imun anak dengan ISPA.Metode penelitian yang digunakan dalam penelitian ini adalah kualitatif deskriptif dengan sampel purposive sampling.Populasi dan sampel penelitian ini adalah ibu yang mempunyai anak dengan riwayat dan saat ini menderita penyakit ISPA di Batu Gajah Kota Ambon.Partisipan dalam penelitian ini berjumlah 5 orang. Hasil dari penelitian mendapati 4 kategori yaitu pemberian nutrisi pada anak untuk memenuhi kebutuhan agar sistem imunnya terjaga, kebersihan lingkungan, peran ibu dalam melakukan pencegahan pada anaknya yang mengalami ISPA, dan  peran ibu dalam menjaga dan mempertahankan kesehatan anaknya.   Kata kunci: peran ibu, sistem imun, ispa THE ROLE OF MOTHERS IN INCREASING IMMUNE SYSTEM OF CHILDREN WITH ACUTE RESPIRATORY INFECTION    ABSTRACT Acute respiratory infections or often called ARI is an infection of the upper or lower respiratory tract. ARI occurs mostly in children under the age of five because their endurance is not strong in dealing with ARI. ARI results in deaths of around 15%-20% per year at the age of under-five in developing countries. The purpose of this study was to determine and analyze the role of mothers in improving the immune system of children against ARI. The research method used in this study was qualitative descriptive with a purposive sampling sample. Respondents and samples of this study were five mothers who had children with a history of ARI and currently suffering from the disease in Batu Gajah, Ambon City. The results of the study found 4 categories, namely providing nutrition to children to meet their needs so that their immune systems are maintained, clean environment, mother's role in preventing children with ARI, and mother's role to preserve and maintain the health of their children. The findings indicated that in terms of coping or improving the immune system of a child to avoid ARI, it is necessary to have role the of mothers in providing nutrition so that the immune system is boosted, besides that the mother can prevent and protect her child from various diseases, especially ARI. Keywords: role of mothers, immune system, acute respiratory infections


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