Is the ALS a motor neuron disease or a hematopoietic stem cell disease?

Bryukhovetskiy, Andrey; Гривцова, Л. Ю.; Sharma, Hari Shanker

doi:10.1016/bs.pbr.2020.09.005

Cited by 5 publications

(4 citation statements)

References 25 publications

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“…When HSCs interact with cancer cells, their cytoplasm also displays fluorescent stain aggregation and the transfer of specific proteins, accompanied by the epigenetic reprogramming of HSCs (154). The ability of these cells to effectively restore hematopoiesis and immunopoiesis requires further investigation; however, a previous clinical trial demonstrated that oncologic and autoimmune diseases cause significant changes in the molecular phenotype of HSCs, impacting treatment outcomes and the prognosis of patients (156).…”

Section: Hscs and Itmentioning

confidence: 99%

Cell‑based immunotherapy of glioblastoma multiforme (Review)

Bryukhovetskiy

2022

Oncol Lett

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Glioblastoma multiforme (GBM) is the most aggressive and lethal primary glial brain tumor. It has an unfavorable prognosis and relatively ineffective treatment protocols, with the median survival of patients being ~15 months. Tumor resistance to treatment is associated with its cancer stem cells (CSCs). At present, there is no medication or technologies that have the ability to completely eradicate CSCs, and immunotherapy (IT) is only able to prolong the patient's life. The present review aimed to investigate systemic solutions for issues associated with immunosuppression, such as ineffective IT and the creation of optimal conditions for CSCs to fulfill their lethal potential. The present review also investigated the main methods involved in local immunosuppression treatment, and highlighted the associated disadvantages. In addition, novel treatment options and targets for the elimination and regulation of CSCs with adaptive and active IT are discussed. Antagonists of TGF-β inhibitors, immune checkpoints and other targeted medication are also summarized. The role of normal hematopoietic stem cells (HSCs) in the mechanisms underlying systemic immune suppression development in cases of GBM is analyzed, and the potential reprogramming of HSCs during their interaction with cancer cells is discussed. Moreover, the present review emphasizes the importance of the aforementioned interactions in the development of immune tolerance and the inactivation of the immune system in neoplastic processes. The possibility of solving the problem of systemic immunosuppression during transplantation of donor HSCs is discussed. Contents1. Introduction 2. Summary on GBM 3. Treatment resistance factors 4. Immune privilege of the CNS 5. IT and CSC control 6. Adaptive IT 7. Active IT 8. IT and molecular-based therapy 9. HSCs and IT 10. Conclusion Cell-based immunotherapy of glioblastoma multiforme (Review)

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Section: Hscs and Itmentioning

confidence: 99%

Cell‑based immunotherapy of glioblastoma multiforme (Review)

Bryukhovetskiy

2022

Oncol Lett

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“…Amyotrophic lateral sclerosis is the most common subtype of motor neuron disease, with a worldwide prevalence of 4–6 in 100,000 people ( Bucheli et al, 2013 ). It is an incurable, fatal neurodegenerative disorder with an average survival of 2-3 years from diagnosis ( Bryukhovetskiy et al, 2020 ). ALS is characterized by rapid, progressive degeneration of upper and lower motor neurons, resulting in muscle atrophy, gradual paralysis, and death ( Ravits et al, 2007 ).…”

Section: Amyotrophic Lateral Sclerosismentioning

confidence: 99%

Mitochondrial Quality Control Strategies: Potential Therapeutic Targets for Neurodegenerative Diseases?

Liu

2021

Front. Neurosci.

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Many lines of evidence have indicated the therapeutic potential of rescuing mitochondrial integrity by targeting specific mitochondrial quality control pathways in neurodegenerative diseases, such as Parkinson’s disease, Huntington’s disease, and Alzheimer’s disease. In addition to ATP synthesis, mitochondria are critical regulators of ROS production, lipid metabolism, calcium buffering, and cell death. The mitochondrial unfolded protein response, mitochondrial dynamics, and mitophagy are the three main quality control mechanisms responsible for maintaining mitochondrial proteostasis and bioenergetics. The proper functioning of these complex processes is necessary to surveil and restore mitochondrial homeostasis and the healthy pool of mitochondria in cells. Mitochondrial dysfunction occurs early and causally in disease pathogenesis. A significant accumulation of mitochondrial damage resulting from compromised quality control pathways leads to the development of neuropathology. Moreover, genetic or pharmaceutical manipulation targeting the mitochondrial quality control mechanisms can sufficiently rescue mitochondrial integrity and ameliorate disease progression. Thus, therapies that can improve mitochondrial quality control have great promise for the treatment of neurodegenerative diseases. In this review, we summarize recent progress in the field that underscores the essential role of impaired mitochondrial quality control pathways in the pathogenesis of neurodegenerative diseases. We also discuss the translational approaches targeting mitochondrial function, with a focus on the restoration of mitochondrial integrity, including mitochondrial dynamics, mitophagy, and mitochondrial proteostasis.

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“…There is growing scientific and clinical interest in Syr-associated amyotrophic lateral syndrome (also known as ALS mimic syndrome) among all Syr-related disorders in patients with CM1 [4,6,13,29], because it requires a differential diagnosis with idiopathic ALS (also known as Lou Gehrig's disease) [30]. ALS is characterized by damage to the motor neurons of the anterior horns of the spinal cord with the formation of progressive muscle atrophies and fasciculations, which can outpace the development of motor deficit [31].…”

Section: Introductionmentioning

confidence: 99%

Amyotrophic Lateral Sclerosis Mimic Syndrome in a 24-Year-Old Man with Chiari 1 Malformation and Syringomyelia: A Clinical Case

Al-Zamil

Shnayder

Davydova³

et al. 2023

JCM

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Chiari 1 Malformation (CM1) is classically defined as a caudal displacement of the cerebellar tonsils through the foramen magnum into the spinal cord. Modern imaging techniques and experimental studies disclose a different etiology for the development of CM1, but the main etiology factor is a structural defect in the skull as a deformity or partial reduction, which push down the lower part of the brain and cause the cerebellum to compress into the spinal canal. CM1 is classified as a rare disease. CM1 can present with a wide variety of symptoms, also non-specific, with consequent controversies on diagnosis and surgical decision-making, particularly in asymptomatic or minimally symptomatic. Other disorders, such as syringomyelia (Syr), hydrocephalus, and craniocervical instability can be associated at the time of the diagnosis or appear secondarily. Therefore, CM1-related Syr is defined as a single or multiple fluid-filled cavities within the spinal cord and/or the bulb. A rare CM1-related disorder is syndrome of lateral amyotrophic sclerosis (ALS mimic syndrome). We present a unique clinical case of ALS mimic syndrome in a young man with CM1 and a huge singular syringomyelic cyst with a length from segment C2 to Th12. At the same time, the clinical picture showed upper hypotonic-atrophic paraparesis in the absence of motor disorders in the lower extremities. Interestingly, this patient did not have a disorder of superficial and deep types of sensitivity. This made it difficult to diagnose CM1. For a long time, the patient’s symptoms were regarded as a manifestation of ALS, as an independent neurological disease, and not as a related disorder of CM1. Surgical treatment for CM1 was not effective, but it allowed to stabilize the course of CM1-related ALS mimic syndrome over the next two years.

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Is the ALS a motor neuron disease or a hematopoietic stem cell disease?

Cited by 5 publications

References 25 publications

Cell‑based immunotherapy of glioblastoma multiforme (Review)

Cell‑based immunotherapy of glioblastoma multiforme (Review)

Mitochondrial Quality Control Strategies: Potential Therapeutic Targets for Neurodegenerative Diseases?

Amyotrophic Lateral Sclerosis Mimic Syndrome in a 24-Year-Old Man with Chiari 1 Malformation and Syringomyelia: A Clinical Case

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