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The Latest Articles on Amyotrophic Lateral Sclerosis

Published on: 28 Dec 2022 Viewed: 104

Our staff editors continue to share exciting, interesting, and thought-provoking reading material in the recommended articles series.

This week, we would like to share several latest articles on Amyotrophic Lateral Sclerosis.

Title: Multidisciplinary clinic contributes to the decreasing trend in the number of emergency hospitalizations for amyotrophic lateral sclerosis in Japan
Authors: Tatsuki Sugisawa, Harumi Morioka, Takehisa Hirayama, Osamu Kano, Satoru Ebihara
Type: Research Article
Abstract:
Background
Multidisciplinary care is recommended for amyotrophic lateral sclerosis (ALS). We opened the first multidisciplinary care "ALS clinic" in Japan in February 2017. This study aimed to clarify the impact of multidisciplinary care on the number and incidence rate of emergency hospitalizations, as well as the survival rate of patients with ALS.

Methods
We studied the medical charts of patients with ALS who visited our hospital between March 1, 2014, and February 29, 2020, in a retrospective study. All patients were divided into two groups: a General Neurology Clinic group (GNC) and an ALS Clinic group (AC), based on the duration of the first visit to our hospital.

Results
The survey participants included 90 patients with ALS (32 in the GNC vs 58 in the AC). The mean follow-up duration was 276 ± 257 days in the GNC and 307 ± 267 days in the AC. The number of emergency hospitalizations was 11 in the GNC and nine in the AC. The number of patients with two or more emergency hospitalizations was decreased in the AC (3 in the GNC vs 0 in the AC), which was statistically significantly different (p = 0.04). The survival rate was significantly different between the two groups (p = 0.01).

Conclusions
Our results suggest that intervention through ALS multidisciplinary care in the hospital setting effectively controls emergency hospitalizations and improves the survival rate in patients with ALS. Multidisciplinary care is recommended since various medical treatments are required as the condition progresses.
Access this article: https://doi.org/10.1016/j.jocn.2022.10.011


Title: Therapeutic and diagnostic potential of extracellular vesicles in amyotrophic lateral sclerosis
Authors: Taylor J. Ellison, Steven L. Stice, Yao Yao
Type: Review
Abstract:
As a late-onset and fatal neurodegenerative disease that affects upper and lower motor neurons, amyotrophic lateral sclerosis (ALS) is a difficult disease to diagnose and treat, needing further research on mechanisms of pathogenesis. In addition due to difficulties in delivering therapeutics past the blood brain barrier (BBB) to target disease-relevant cells in the central nervous system (CNS), diseases like ALS are particularly difficult to manage. However, recent advances in nanomedicine have provided new opportunities. Extracellular vesicles (EVs) are small, spherical, nanosized particles that have shown various applicable properties in the treatment and diagnosis of ALS through inherent characteristics and drug delivery capabilities. Extracellular vesicles with unique cargos determined by cells of origin possess neural protecting qualities and can cross the BBB and deliver endogenous and/or engineered cargo to specific neural cell targets. In this review, we discuss the potential use of EVs as therapeutics, drug delivery systems, and biomarkers for ALS.
Access this article: https://doi.org/10.1016/j.vesic.2022.100019


Title: Protective effect of human umbilical cord mesenchymal stem cell derived conditioned medium in a mutant TDP-43 induced motoneuron-like cellular model of ALS
Authors: Jiaqi Lan, Yujun Zhou, Hongyue Wang, Jingshu Tang, Yuying Kang, Peishen Wang, Xuebin Liu, Ying Peng
Type: Research Article
Abstract:
Amyotrophic lateral sclerosis (ALS) is a multi-factor neurodegenerative disease, characterized by the loss of motor neurons. TAR DNA-binding protein 43 (TDP-43) mutation, accumulation and aggregation, as well as oxidative stress are recognized as major pathological denominators and biochemical markers for ALS. Recently, human umbilical cord mesenchymal stem cell-derived conditioned medium (UC-CM) has been introduced to treat ALS patients. However, there is no research for the protective effect of UC-CM on the TDP-43 model of ALS. In this study, we evaluated the potential neuroprotective effect of UC-CM on a cellular ALS model expressing TDP-43mutant M337V, as well as its underlying mechanism. We found that 24 h UC-CM treatment could protect M337V expressing motor neurons by increasing cell viability and reducing LDH leakage. Furthermore, the aggregation of M337V, generation of ROS, malondialdehyde (MDA), 4-hydroxynonenal (4-HNE), protein carbonyl and 8-OHdG were also reduced by UC-CM, indicating that UC-CM protected cells by reducing oxidative damage. Moreover, UC-CM significantly increased the expression of nuclear Nrf2 and its downstream enzyme HO1. The Nrf2 translocation inhibitor ML385 could inhibit the effect of UC-CM on the cell viability and aggregate of M337V. Our results suggest that UC-CM protect cells against M337V expression by its strong antioxidative effect via Nrf-2/HO-1 axis activation.
Access this article: https://doi.org/10.1016/j.brainresbull.2022.12.008


Title: XCL1, a serum biomarker in neurological diseases; HTLV-1-associated myelopathy and multiple sclerosis
Authors: Morteza Saeidi, Zohreh Vahidi, Mohammad Ali Nahayati, Majid Khadem Rezaiyan, Fariba Zemorshidi, Maryam Mahdifar, Fatemeh Hafezi, Saeedeh Mehraban Moghadam, Effat Saghi, Ensieh Akbarpour, Reza Boostani, Houshang Rafatpanah
Type: Research Article
Abstract:
The XCL1-XCR1 axis has a potential role in the recruitment of immune cells to the site of inflammation. The present study aimed to examine the relation of XCL1 serum levels with Multiple sclerosis (MS) and HTLV-1-associated myelopathy (HAM), as chronic inflammatory diseases of the central nervous system (CNS). DNA was extracted to evaluate HTLV-1 proviral load (PVL) using real-time PCR. Serum levels of XCL1 was determined by using an ELISA assay. The serum level of XCL1 was significantly higher in patients with HAM than that of asymptomatic carriers (ACs) and healthy controls (HCs) (p < 0.001 and p < 0.0001, respectively) and was also higher in MS patients compared to HCs (p < 0.0001). Moreover, the concentration of XCL1 serum level was significantly different between the ACs and HCs group (p < 0.0001). In conclusion, increased expression of XCL1 might contribute to the migration of autoreactive T cells to the central nervous system and play a critical role in the development and pathogenesis of inflammatory neurological diseases including HAM and MS.
Access this article: https://doi.org/10.1016/j.micpath.2022.105962


Title: NMNAT2: An important metabolic enzyme affecting the disease progression
Authors: Wentao Li, Mengxiang Gao, Chunhui Hu, Xiuwen Chen, Yanhong Zhou
Type: Review
Abstract:
Nicotinamide mononucleotide adenylyltransferase 2 (NMNAT2) is an evolutionarily conserved nicotinamide adenine dinucleotide (NAD+) synthase located in the cytoplasm and Golgi apparatus. NMNAT2 has an important role in neurodegenerative diseases, malignant tumors, and other diseases that seriously endanger human health. NMNAT2 exerts a neuroprotective function through its NAD synthase activity and chaperone function. Among them, the NMNAT2-NAD+-Sterile alpha and Toll/interleukin-1 receptor motif-containing 1 (SARM1) axis is closely related to Wallerian degeneration. Physical injury or pathological stimulation will cause a decrease in NMNAT2, which activates SARM1, leading to axonal degeneration and the occurrence of amyotrophic lateral sclerosis (ALS), Alzheimer's disease, peripheral neuropathy, and other neurodegenerative diseases. In addition, NMNAT2 exerts a cancer-promoting role in solid tumors, including colorectal cancer, lung cancer, ovarian cancer, and glioma, and is closely related to tumor occurrence and development. This paper reviews the chromosomal and subcellular localization of NMNAT2 and its basic biological functions. We also summarize the NMNAT2-related signal transduction pathway and the role of NMNAT2 in diseases. We aimed to provide a new perspective to comprehensively understand the relationship between NMNAT2 and its associated diseases.
Access this article: https://doi.org/10.1016/j.biopha.2022.114143


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