Bovine spastic syndrome: a review

2018 ◽  
Vol 182 (24) ◽  
pp. 693-693 ◽  
Author(s):  
Victoria Goeckmann ◽  
Sophie Rothammer ◽  
Ivica Medugorac
Keyword(s):  
Multiple Sclerosis ◽  
Physical Therapy ◽  
Clinical Signs ◽  
Hereditary Disease ◽  
Neuromuscular Disorder ◽  
Future Research ◽  
Interesting Aspect ◽  
Similar Disease ◽  
Spastic Paresis ◽  
First Time

Bovine spastic syndrome (BSS) was described for the first time in 1941. The disease occurs in various—maybe even all—cattle breeds and is a chronic-progressive neuromuscular disorder that commonly affects cattle of at least three years of age. Typical clinical signs of the disease are clonic-tonic cramps of the hindlimbs that occur in attacks. Since BSS does not recover, affected animals can only be treated symptomatically by improving welfare conditions and management factors, or with physical therapy or drugs. Although still not irrevocably proven, BSS is assumed to be a hereditary disease. Therefore, affected animals should be excluded from breeding, which negatively affects economics and breeding. Besides epidemiology, clinical signs, aetiopathogenesis, diagnosis and treatment, this review discusses genetic aspects and differences to the similar disease bovine spastic paresis. Furthermore, this review also picks up the discussion on possible parallels between human multiple sclerosis and BSS as a further interesting aspect, which might be of great interest for future research.

scholarly journals Genetic mutations and molecular mechanisms of Fuchs endothelial corneal dystrophy

2021 ◽  
Vol 8 (1) ◽  
Author(s):  
Xuerui Liu ◽  
Tao Zheng ◽  
Chuchu Zhao ◽  
Yi Zhang ◽  
Hanruo Liu ◽  
...  
Keyword(s):  
Endothelial Cells ◽  
Molecular Mechanisms ◽  
Epithelial Mesenchymal Transition ◽  
Corneal Transplantation ◽  
Clinical Signs ◽  
Corneal Dystrophy ◽  
Hereditary Disease ◽  
Future Research ◽  
Apoptosis Pathway ◽  
Mesenchymal Transition

Abstract Background Fuchs endothelial corneal dystrophy is a hereditary disease and the most frequent cause of corneal transplantation in the worldwide. Its main clinical signs are an accelerated decrease in the number of endothelial cells, thickening of Descemet’s membrane and formation of guttae in the extracellular matrix. The cornea’s ability to maintain stromal dehydration is impaired, causing painful epithelial bullae and loss of vision at the point when the amount of corneal endothelial cells cannot be compensated. At present, apart from corneal transplantation, there is no other effective treatment that prevents blindness. Main text In this review, we first summarized the mutations of COL8A2, TCF4, TCF8, SLC4A11 and AGBL1 genes in Fuchs endothelial corneal dystrophy. The molecular mechanisms associated with Fuchs endothelial corneal dystrophy, such as endoplasmic reticulum stress and unfolded protein response pathway, oxidative stress, mitochondrial dysregulation pathway, apoptosis pathway, mitophagy, epithelial-mesenchymal transition pathway, RNA toxicity and repeat-associated non-ATG translation, and other pathogenesis, were then explored. Finally, we discussed several potential treatments related to the pathogenesis of Fuchs endothelial corneal dystrophy, which may be the focus of future research. Conclusions The pathogenesis of Fuchs endothelial corneal dystrophy is very complicated. Currently, corneal transplantation is an important method in the treatment of Fuchs endothelial corneal dystrophy. It is necessary to continuously explore the pathogenesis of Fuchs endothelial corneal dystrophy and establish the scientific foundations for the development of next-generation corneal therapeutics.

scholarly journals Tryptamine Attenuates Experimental Multiple Sclerosis Through Activation of Aryl Hydrocarbon Receptor

2021 ◽  
Vol 11 ◽  
Author(s):  
Nicholas Dopkins ◽  
William Becker ◽  
Kathryn Miranda ◽  
Mike Walla ◽  
Prakash Nagarkatti ◽  
...  
Keyword(s):  
Multiple Sclerosis ◽  
T Cells ◽  
Aryl Hydrocarbon Receptor ◽  
Clinical Signs ◽  
Wild Type ◽  
Autoimmune Encephalomyelitis ◽  
Aryl Hydrocarbon ◽  
First Time ◽  
Butyrate Production ◽  
Experimental Autoimmune

Tryptamine is a naturally occurring monoamine alkaloid which has been shown to act as an aryl hydrocarbon receptor (AHR) agonist. It is produced in large quantities from the catabolism of the essential amino acid tryptophan by commensal microorganisms within the gastrointestinal (GI) tract of homeothermic organisms. Previous studies have established microbiota derived AHR ligands as potent regulators of neuroinflammation, further defining the role the gut-brain axis plays in the complex etiology in multiple sclerosis (MS) progression. In the current study, we tested the ability of tryptamine to ameliorate symptoms of experimental autoimmune encephalomyelitis (EAE), a murine model of MS. We found that tryptamine administration attenuated clinical signs of paralysis in EAE mice, decreased the number of infiltrating CD4+ T cells in the CNS, Th17 cells, and RORγ T cells while increasing FoxP3+Tregs. To test if tryptamine acts through AHR, myelin oligodendrocyte glycoprotein (MOG)-sensitized T cells from wild-type or Lck-Cre AHRflox/flox mice that lacked AHR expression in T cells, and cultured with tryptamine, were transferred into wild-type mice to induce passive EAE. It was noted that in these experiments, while cells from wild-type mice treated with tryptamine caused marked decrease in paralysis and attenuated neuroinflammation in passive EAE, similar cells from Lck-Cre AHRflox/flox mice treated with tryptamine, induced significant paralysis symptoms and heightened neuroinflammation. Tryptamine treatment also caused alterations in the gut microbiota and promoted butyrate production. Together, the current study demonstrates for the first time that tryptamine administration attenuates EAE by activating AHR and suppressing neuroinflammation.

Retrospective Diagnosis of Pontocerebellar Hypoplasia Type 1B in a Family with Two Deceased Newborn Children

2020 ◽  
Author(s):  
Irena Bradinova ◽  
Silvia Andonova ◽  
Alexey Savov
Keyword(s):  
Clinical Signs ◽  
Final Diagnosis ◽  
Population Based ◽  
Neuromuscular Disorder ◽  
Population Characteristics ◽  
High Risk Population ◽  
Pontocerebellar Hypoplasia ◽  
Clinical Genetic ◽  
Retrospective Diagnosis ◽  
Newborn Children

AbstractPontocerebellar hypoplasia type 1B is a severe autosomal recessive neurologic disorder characterized by a combination of cerebellar and spinal motor neuron degeneration beginning at birth. Pontocerebellar hypoplasia type 1B is caused by mutations in EXOSC3 gene. High prevalence of the p.Gly31Ala mutation was found recently, especially in the Roma ethnic minority. We present a young Bulgarian Roma family with two deceased newborn children manifesting severe neuromuscular disorder including severe muscle weakness, respiratory distress, and multiple joint contractures. Based on the clinical signs and family's population characteristics, DNA testing for the previously described EXOSC3 in Bulgarian Roma mutation c.92G > C; p.Gly31Ala was performed on blood samples of both parents and they were found to be heterozygous carriers. This finding indirectly confirmed the diagnosis of pontocerebellar hypoplasia type B in the deceased offspring. Knowledge of population-specific molecular bases of genetic conditions was the key to final diagnosis in the presented family. Designing of population-based clinical-genetic panels may be a powerful diagnostic tool for patients with such origin. Preconception carrier screening in high-risk population groups is a feasible option to discuss.

A Bibliometric Review on Outcome-Based Learning for Graduate Employability: Mapping the Research Front

2021 ◽  
pp. 002205742110164
Author(s):  
Mohammad Zahir Raihan ◽  
Md. Abul Kalam Azad
Keyword(s):  
Research Trend ◽  
Future Research ◽  
Research Front ◽  
Important Research ◽  
R Software ◽  
Future Research Agenda ◽  
Graduate Employability ◽  
Important Research Topic ◽  
Bibliometric Review ◽  
First Time

The outcome-based learning for graduate employability in higher education has been an important research topic among the policymakers, academicians, and researchers over the years. Yet, no bibliometric review on this topic has been published. This study, for the first time, examines bibliometric analysis on this topic examining current research trend and future research agenda. The bibliometrix package in R software and VOSviewer software are used for visualization and interpretation of results. A content analysis is performed to manually examine the bibliometric results.

scholarly journals Exploring Secondary Metabolite Profiles of Stachybotrys spp. by LC-MS/MS

Toxins ◽  
2019 ◽  
Vol 11 (3) ◽  
pp. 133 ◽  
Author(s):  
Annika Jagels ◽  
Viktoria Lindemann ◽  
Sebastian Ulrich ◽  
Christoph Gottschalk ◽  
Benedikt Cramer ◽  
...  
Keyword(s):  
Secondary Metabolites ◽  
Future Research ◽  
Structural Modifications ◽  
Metabolite Profiles ◽  
The Individual ◽  
First Time ◽  
Analytical Standards ◽  
Respective Species

The genus Stachybotrys produces a broad diversity of secondary metabolites, including macrocyclic trichothecenes, atranones, and phenylspirodrimanes. Although the class of the phenylspirodrimanes is the major one and consists of a multitude of metabolites bearing various structural modifications, few investigations have been carried out. Thus, the presented study deals with the quantitative determination of several secondary metabolites produced by distinct Stachybotrys species for comparison of their metabolite profiles. For that purpose, 15 of the primarily produced secondary metabolites were isolated from fungal cultures and structurally characterized in order to be used as analytical standards for the development of an LC-MS/MS multimethod. The developed method was applied to the analysis of micro-scale extracts from 5 different Stachybotrys strains, which were cultured on different media. In that process, spontaneous dialdehyde/lactone isomerization was observed for some of the isolated secondary metabolites, and novel stachybotrychromenes were quantitatively investigated for the first time. The metabolite profiles of Stachybotrys species are considerably influenced by time of growth and substrate availability, as well as the individual biosynthetic potential of the respective species. Regarding the reported adverse effects associated with Stachybotrys growth in building environments, combinatory effects of the investigated secondary metabolites should be addressed and the role of the phenylspirodrimanes re-evaluated in future research.

scholarly journals Deservingness for "Family 500 +" Benefit in Poland: Qualitative Study of Internet Debates

2021 ◽  
Author(s):  
Piotr Michoń
Keyword(s):  
Future Research ◽  
Online Forums ◽  
Time Data ◽  
Social Surveys ◽  
Cash Benefit ◽  
Internet Forums ◽  
Welfare Attitudes ◽  
New Criterion ◽  
First Time ◽  
The Relationship

AbstractThe need for qualitative research of deservingness perception is strongly emphasised in the literature. This article studies the perception of deservingness for a "Family 500 +"—cash benefit in Poland. For the first time, data from online forums was used in the studies of deservingness and welfare attitudes. It allowed to avoid numerous limitations associated with social surveys. The qualitative analysis showed how participants of Internet debates perceive the criteria of deservingness: control, attitude, reciprocity, identity, need, and what are the relations between the criteria. The impurity of all deservingness criteria was indicated and a new criterion “adequacy” was proposed. Moreover due to the fact that the study concerned a concrete, non-abstract family cash benefit addressed the relationship between the perceived deservingness of children and their parents was pointed out. The vast majority of posts on Internet forums referred to deservingness of parents, not children. This is particularly evident in relation to the criteria of control and reciprocity. Presenting the hypothesis of jealousy and scapegoat strategy, the article also shows the direction of future research on deservingness.

scholarly journals Ambulatory Neuroproprioceptive Facilitation and Inhibition Physical Therapy Improves Clinical Outcomes in Multiple Sclerosis and Modulates Serum Level of Neuroactive Steroids: A Two-Arm Parallel-Group Exploratory Trial

Life ◽  
2020 ◽  
Vol 10 (11) ◽  
pp. 267
Author(s):  
Gabriela Angelova ◽  
Tereza Skodova ◽  
Terezie Prokopiusova ◽  
Magdalena Markova ◽  
Natalia Hruskova ◽  
...  
Keyword(s):  
Multiple Sclerosis ◽  
Physical Therapy ◽  
Serum Level ◽  
Clinical Outcomes ◽  
Endocrine System ◽  
Motor Program ◽  
Neuroactive Steroids ◽  
Patient Reported ◽  
Serial Addition ◽  
Post Assessment

Background: Only few studies have monitored the potential of physical activity training and physical therapy to modulate the reaction of the endocrine system. In this study, the effect of neuroproprioceptive facilitation and inhibition physical therapy on clinical outcomes and neuroactive steroids production in people with multiple sclerosis was evaluated. Moreover, we were interested in the factors that influence the treatment effect. Methods: In total, 44 patients with multiple sclerosis were randomly divided into two groups. Each group underwent a different kind of two months ambulatory therapy (Motor program activating therapy and Vojta’s reflex locomotion). During the following two months, participants were asked to continue the autotherapy. Primary (serum level of cortisol, cortisone, 7α-OH-DHEA, 7β-OH-DHEA, 7-oxo-DHEA, DHEA) and secondary (balance, cognition and patient-reported outcomes) outcomes were examined three times (pre, post, and washout assessments). Results: In both groups, there is a decreasing trend of 7-oxo-DHEA concentration in post-assessment and 7β-OH-DHEA in washout versus pre-assessment. A higher impact on neuroactive steroids is visible after Vojta’s reflex locomotion. As for clinical outcomes, the Paced Auditory Serial Addition Test and Multiple Sclerosis Impact Scale significantly improved between post-assessment and washout assessment. The improvement was similar for both treatments. Conclusions: Neuroproprioceptive facilitation and inhibition improved the clinical outcomes and led to non-significant changes in neuroactive steroids. Trial registration (NCT04379193).

scholarly journals Disease phenotype analyses of relapsing-remitting multiple sclerosis in Canada and Saudi Arabia

2015 ◽  
Vol 42 (S1) ◽  
pp. S32-S32
Author(s):  
M Alluqmani ◽  
M Alqermli ◽  
G Blevins ◽  
B Alotibi ◽  
F Giuliani ◽  
...  
Keyword(s):  
Multiple Sclerosis ◽  
Saudi Arabia ◽  
Geographic Location ◽  
Clinical Findings ◽  
Cross Sectional ◽  
Relapsing Remitting ◽  
Similar Disease ◽  
The Mean ◽  
Sectional Analysis ◽  
Relapsing Remitting Multiple Sclerosis

Background: Multiple sclerosis (MS) exhibits a spectrum of clinical findings, especially in relapsing-remitting MS (RR-MS). To assess the effects of geographic location and ethnicity on RR-MS phenotype, we investigated RR-MS patients in Canada and Saudi Arabia. Methods: A retrospective cross-sectional analysis of patients receiving active care in MS Clinics was performed in Medina, Saudi Arabia and Edmonton, Alberta. Demographic and clinical data was collected for each patient. Results: 98 patients with treated RR-MS were recruited (n=51, Medina; n=47, Edmonton); 40 patients were Caucasian (Edmonton) while 46 patients were Bedouin (Medina). Although the disease duration was longer in the Edmonton (5.7+2.3 yr) compared to the Medina group (4.4+1.4 yr) (p<0.05), the mean age of RR-MS onset, relapse rate and EDSS change were similar. The female:male ratio was comparable in Edmonton (35:12) and Medina (32:19), as was the risk of optic neuritis. The likelihood of an infratentorial lesion-associated presentation differed (Edmonton, n=23; Medina; n=13) among groups (p<0.05). Spinal cord lesions on MRI were more frequent in Edmonton (n=18) compared to Medina (n=1) patients (p<0.05). Conclusions: Despite differences in location, ethnicity, and a predominance of infratentorial lesion burden the Edmonton group, the RR-MS phenotype displayed similar disease severity and trajectory in these cohorts.

scholarly journals Host Genetics and Gut Microbiome: Perspectives for Multiple Sclerosis

Genes ◽  
2021 ◽  
Vol 12 (8) ◽  
pp. 1181
Author(s):  
Alessandro Maglione ◽  
Miriam Zuccalà ◽  
Martina Tosi ◽  
Marinella Clerico ◽  
Simona Rolla
Keyword(s):  
Multiple Sclerosis ◽  
Environmental Factors ◽  
Lupus Erythematosus ◽  
Gut Microbiome ◽  
Complex Disease ◽  
Current Knowledge ◽  
Association Studies ◽  
Future Research ◽  
Genome Wide Association Studies ◽  
Host Genetics

As a complex disease, Multiple Sclerosis (MS)’s etiology is determined by both genetic and environmental factors. In the last decade, the gut microbiome has emerged as an important environmental factor, but its interaction with host genetics is still unknown. In this review, we focus on these dual aspects of MS pathogenesis: we describe the current knowledge on genetic factors related to MS, based on genome-wide association studies, and then illustrate the interactions between the immune system, gut microbiome and central nervous system in MS, summarizing the evidence available from Experimental Autoimmune Encephalomyelitis mouse models and studies in patients. Finally, as the understanding of influence of host genetics on the gut microbiome composition in MS is in its infancy, we explore this issue based on the evidence currently available from other autoimmune diseases that share with MS the interplay of genetic with environmental factors (Inflammatory Bowel Disease, Rheumatoid Arthritis and Systemic Lupus Erythematosus), and discuss avenues for future research.

CRYAB modulates the activation of CD4+ T cells from relapsing–remitting multiple sclerosis patients

2013 ◽  
Vol 19 (14) ◽  
pp. 1867-1877 ◽  
Author(s):  
Que Lan Quach ◽  
Luanne M Metz ◽  
Jenna C Thomas ◽  
Jonathan B Rothbard ◽  
Lawrence Steinman ◽  
...  
Keyword(s):  
Multiple Sclerosis ◽  
T Cells ◽  
Cytokine Production ◽  
Clinical Signs ◽  
Healthy Controls ◽  
Relapsing Remitting ◽  
Remitting Multiple Sclerosis ◽  
Multiple Sclerosis Patients ◽  
Relapsing Remitting Multiple Sclerosis

Background: Suppression of activation of pathogenic CD4+ T cells is a potential therapeutic intervention in multiple sclerosis (MS). We previously showed that a small heat shock protein, CRYAB, reduced T cell proliferation, pro-inflammatory cytokine production and clinical signs of experimental allergic encephalomyelitis, a model of MS. Objective: We assessed whether the ability of CRYAB to reduce the activation of T cells translated to the human disease. Methods: CD4+ T cells from healthy controls and volunteers with MS were activated in vitro in the presence or absence of a CRYAB peptide (residues 73–92). Parameters of activation (proliferation rate, cytokine secretion) and tolerance (anergy, activation-induced cell death, microRNAs) were evaluated. Results: The secretion of pro-inflammatory cytokines by CD4+ T cells was decreased in the presence of CRYAB in a subset of relapsing–remitting multiple sclerosis (RRMS) participants with mild disease severity while no changes were observed in healthy controls. Further, there was a correlation for higher levels of miR181a microRNA, a marker upregulated in tolerant CD8+ T cells, in CD4+ T cells of MS patients that displayed suppressed cytokine production (responders). Conclusion: CRYAB may be capable of suppressing the activation of CD4+ T cells from a subset of RRMS patients who appear to have less disability but similar age and disease duration.

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