Computer analysis of the relation between hydrogen bond stability in SOD1 mutants and the survival time of amyotrophic lateral sclerosis patients

2022 ◽  
Vol 110 ◽  
pp. 108026
Author(s):  
Nikolay A. Alemasov ◽  
Vladimir S. Timofeev ◽  
Nikita V. Ivanisenko ◽  
Nikolay A. Kolchanov ◽  
Vladimir A. Ivanisenko
Keyword(s):  
Hydrogen Bond ◽  
Amyotrophic Lateral Sclerosis ◽  
Survival Time ◽  
Computer Analysis ◽  
Bond Stability ◽  
Lateral Sclerosis

Improved regression model to predict an impact of SOD1 mutations on ALS patients survival time based on analysis of hydrogen bond stability

2019 ◽  
Vol 86 ◽  
pp. 247-255
Author(s):  
Nikolay A. Alemasov ◽  
Nikita V. Ivanisenko ◽  
Bhupesh Taneja ◽  
Vibha Taneja ◽  
Srinivasan Ramachandran ◽  
...  
Keyword(s):  
Hydrogen Bond ◽  
Regression Model ◽  
Survival Time ◽  
Bond Stability ◽  
Als Patients

scholarly journals A Retrospective Study of the Clinical Phenotype and Predictors of Survival in non-Caucasian Hispanic Patients with Amyotrophic Lateral Sclerosis.

2019 ◽  
Author(s):  
Claudia Marisol Sánchez-Martínez ◽  
José Alberto Choreño-Parra ◽  
Lilia Nuñez-Orozco ◽  
Noel Isaías Placencia-Álvarez ◽  
Laura Marcela Alvis-Cataño ◽  
...  
Keyword(s):  
Overall Survival ◽  
Amyotrophic Lateral Sclerosis ◽  
Prognostic Factors ◽  
Retrospective Study ◽  
Survival Time ◽  
Clinical Characteristics ◽  
Clinical Phenotype ◽  
Age At Onset ◽  
Als Patients ◽  
Lateral Sclerosis

Abstract Background. Little is known about the clinical phenotype of amyotrophic lateral sclerosis (ALS) in non-Caucasian populations. Here, we aimed to describe the clinical characteristics, prognostic factors and survival of Mexican patients with ALS. Methods. We conducted a retrospective study by reviewing the medical records of patients with ALS that attended and were regularly followed at a third level hospital in Mexico City from 2000 to 2015. We calculated absolute and relative frequencies of the clinical characteristics from all the participants. We also estimated correlation coefficients between clinical features and overall survival. Additionally, survival rates were compared for all participants grouped according to different clinical features using the Kaplan-Meier method and the log-rank test. Results. We enrolled 45 ALS patients, 53.33% had spinal-onset ALS and 46.66% presented bulbar ALS. The male/female ratio was 0.8. The mean age at onset of symptoms was 58.11 years. Mean survival time from onset was 64.73 ± 34.83 months. Cumulative survival rate after 5 years of disease onset was 44.44%. Age at onset and age at diagnosis inversely correlated with overall survival time. Also, we found that bulbar-onset, short diagnostic delay, percutaneous endoscopic gastrostomy, mechanical ventilation, and lower total cholesterol serum levels were associated with short survival. Conclusions. The clinical characteristics of Mexican ALS patients differ from the disease phenotype observed in Caucasians. Nonetheless, the predictive value of certain well-recognized prognostic factors remains consistent in our population. The current study provides relevant information for a better understanding of prognostic factors in ALS patients from Mexico and other Latin American countries.

scholarly journals Adipose-derived Stem Cell Conditioned Media Extends Survival time of a mouse model of Amyotrophic Lateral Sclerosis

2015 ◽  
Vol 5 (1) ◽  
Author(s):  
Christine V. Fontanilla ◽  
Huiying Gu ◽  
Qingpeng Liu ◽  
Timothy Z. Zhu ◽  
Changwei Zhou ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Mouse Model ◽  
Survival Time ◽  
Neuronal Death ◽  
Immunohistochemical Analysis ◽  
Mitogen Activated Protein Kinase ◽  
Trophic Factors ◽  
Mitogen Activated Protein ◽  
Adipose Derived Stem Cell ◽  
Lateral Sclerosis

Abstract Adipose stromal cells (ASC) secrete various trophic factors that assist in the protection of neurons in a variety of neuronal death models. In this study, we tested the effects of human ASC conditional medium (ASC-CM) in human amyotrophic lateral sclerosis (ALS) transgenic mouse model expressing mutant superoxide dismutase (SOD1G93A). Treating symptomatic SOD1G93A mice with ASC-CM significantly increased post-onset survival time and lifespan. Moreover, SOD1G93A mice given ASC-CM treatment showed high motor neuron counts, less activation of microglia and astrocytes at an early symptomatic stage in the spinal cords under immunohistochemical analysis. SOD1G93A mice treated with ASC-CM for 7 days showed reduced levels of phosphorylated p38 (pp38) in the spinal cord, a mitogen-activated protein kinase that is involved in both inflammation and neuronal death. Additionally, the levels of α-II spectrin in spinal cords were also inhibited in SOD1G93A mice treated with ASC-CM for 3 days. Interestingly, nerve growth factor (NGF), a neurotrophic factor found in ASC-CM, played a significant role in the protection of neurodegeneration inSOD1G93A mouse. These results indicate that ASC-CM has the potential to develop into a novel and effective therapeutic treatment for ALS.

Dynamic properties of SOD1 mutants can predict survival time of patients carrying familial amyotrophic lateral sclerosis

2016 ◽  
Vol 35 (3) ◽  
pp. 645-656 ◽  
Author(s):  
Nikolay A. Alemasov ◽  
Nikita V. Ivanisenko ◽  
Sergey P. Medvedev ◽  
Suren M. Zakian ◽  
Nikolay A. Kolchanov ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Survival Time ◽  
Dynamic Properties ◽  
Familial Amyotrophic Lateral Sclerosis ◽  
Lateral Sclerosis

scholarly journals Prognostic value of decreased tongue strength on survival time in patients with amyotrophic lateral sclerosis

2012 ◽  
Vol 259 (11) ◽  
pp. 2360-2365 ◽  
Author(s):  
J. G. Weikamp ◽  
H. J. Schelhaas ◽  
J. C. M. Hendriks ◽  
B. J. M. de Swart ◽  
A. C. H. Geurts
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Survival Time ◽  
Prognostic Value ◽  
Tongue Strength ◽  
Lateral Sclerosis

scholarly journals Prognosis of amyotrophic lateral sclerosis with cognitive and behavioural changes based on a sixty-month longitudinal follow-up

PLoS ONE ◽  
2021 ◽  
Vol 16 (8) ◽  
pp. e0253279
Author(s):  
Shan Ye ◽  
Pingping Jin ◽  
Lu Chen ◽  
Nan Zhang ◽  
Dongsheng Fan
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Survival Time ◽  
Progression Rate ◽  
Behavioural Changes ◽  
Significant Difference ◽  
Long Term Follow Up ◽  
Behaviour Changes ◽  
Als Patients ◽  
Lateral Sclerosis

Objective Approximately 50% of amyotrophic lateral sclerosis (ALS) patients have cognitive and behavioural dysfunction in varying degrees and forms. Previous studies have shown that cognitive and behavioural changes may indicate a poor prognosis, and cognitive function gradually deteriorates over the course of disease, but the results of different studies have been inconsistent. In addition, there are relatively limited long-term follow-up studies tracking death as an endpoint. The purpose of this study was to investigate the clinical prognostic characteristics of ALS patients with cognitive behavioural changes through long-term follow-up in a cohort. Methods A total of 87 ALS patients from 2014 to 2015 in the Third Hospital of Peking University were selected and divided into a pure ALS group, an ALS with behavioural variant of frontotemporal dementia (ALS-bvFTD) group, and an ALS with cognitive and behaviour changes group. All patients were followed up for 60 months. The main end point was death and tracheotomy. Results There was no significant difference in survival curve between pure ALS and ALS with cognitive and behavioural change group, but the survival time of ALS-bvFTD group was significantly lower than the other two groups (P < 0.001). For those who was followed up to the endpoint, the survival time of the ALS-bvFTD group was significantly shorter than that of the pure ALS group (t = 5.33, p < 0.001) or the ALS with cognitive and behaviour changes group (t = 4.25, p < 0.001). The progression rate of ALS Functional Rating Scale–Revised (FRS-R) scores from recruitment to endpoint was significantly faster in the ALS-bvFTD group than in the pure ALS group (z = 2.68, p = 0.01) or the ALS with cognitive and behavioural changes group (z = 2.75, p = 0.01). There was no significant difference in survival time (t = 0.52, P = 0.60) or FRS-R score progression rate (z = 0.31, p = 0.76) between the pure ALS group and the ALS with cognitive and behavioural changes group. The total Edinburgh Cognitive and Behavioural Amyotrophic Lateral Sclerosis Screen (ECAS) score was positively correlated with survival time (r = 0.38, p = 0.01). Conclusion ALS-bvFTD patients have shorter survival time. The total ECAS score may be correlated with survival time.

scholarly journals Relationship between Dietary Fiber Intake and the Prognosis of Amyotrophic Lateral Sclerosis

2020 ◽  
Author(s):  
Haelim Yu ◽  
Seung Hyun Kim ◽  
Min-Young Noh ◽  
Sang-Gon Lee ◽  
Yongsoon Park
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Survival Time ◽  
Disease Progression ◽  
Dietary Fiber ◽  
Progression Rate ◽  
Fiber Intake ◽  
Dietary Fiber Intake ◽  
Disease Progression Rate ◽  
Lateral Sclerosis ◽  
Vegetable Fiber

The gut microbiota has been suggested as an important factor in the pathogenic mechanisms of amyotrophic lateral sclerosis (ALS). This study aimed to investigate whether the intake of different kinds of dietary fiber was related to the disease progression rate (∆FS) and survival time. In total, 272 sporadic ALS patients diagnosed according to the revised EI Escorial criteria were recruited from March 2011 and were followed-up until the occurrence of events or the end of September 2020. The events included percutaneous endoscopic gastrostomy, tracheostomy, and death. Dietary fiber intake was calculated based on a 24-hour dietary recall and classified according to five major fiber-rich foods: vegetables, fruits, grains, legumes, and nuts/seeds. Among the total participants, the group with ∆FS values lower than the mean ∆FS (0.75) was noted in the highest tertiles of total and vegetable fiber intake. Participants with the highest tertile of vegetable fiber intake showed longer survival in the Kaplan&ndash;Meier analysis (p = 0.033). Notably, vegetable fiber intake was negatively correlated with pro-inflammatory cytokine (interleukin [IL]-1&beta;, IL-6, and monocyte chemoattractant protein-1) levels in the cerebrospinal fluid. This study showed that vegetable fiber intake could influence the disease progression rate and survival time. Further clinical trials are needed to confirm whether dietary fiber supplementation improves the prognosis of ALS.

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