Progress in the role and mechanism of TDP-43 DOI
Rongbing Li

New discovery., Год журнала: 2024, Номер unknown, С. 1 - 8

Опубликована: Сен. 20, 2024

Background: TAR DNA-binding protein 43 kDa (TDP-43) has been shown to play an important role in the development of neurodegenerative diseases, but mechanism is still under study. Methods: By utilizing “TDP43”, “disease”, and “mechanism” as keywords, 200 related studies were retrieved downloaded from Pubmed database, including 60 articles. We summarized progress understanding TDP-43 over past two years, focusing on disease systems classification upstream downstream, connection, improvement, formation. Results: TDP-43, when abnormally aggregated, phosphorylated, or mislocalized, plays a key pathological diseases. Additionally, its impact normal reproductive cell formation, development, quantity, activity, well insulin secretion activation intestinal epithelial necrosis, should not be overlooked. Mechanistically, we identified relationship between expression factors, Enterovirus D68 (EV-D68), Heterogeneous Nuclear Ribonucleoprotein D (HNRNPD AUF1), Endoplasmic Reticulum Protein 57 (ERp57), Progranulin (PGRN), downstream factors such Meiotic Recombination Spo11 (Spo11), AMP-Activated Kinase (AMPK), Double-Strand-Break Repair Rad21 Homolog (Rad21L), IκB (IKK), TDP-43. Conclusion: neurodegeneration, which, phosphorylation, EV-d68, HNRNPD.

Язык: Английский

Interferons in myositis: Pathogenesis and therapy DOI Open Access
Nozomu Tawara

Clinical and Experimental Neuroimmunology, Год журнала: 2025, Номер unknown

Опубликована: Янв. 26, 2025

Abstract Idiopathic inflammatory myopathies (IIMs), or myositis, are a heterogeneous group of autoimmune diseases affecting skeletal muscle and other organs. Recent research has revealed the important role interferons (IFNs) in pathogenesis IIMs. This review summarizes three types IFNs their functions immune system, focusing on association with different IIM subtypes. Dermatomyositis (DM) is strongly associated Type I IFNs. In contrast, inclusion body myositis (IBM), polymyositis mitochondrial pathology (PM‐mito), anti‐synthetase syndrome (ASyS) predominantly II IFN. explores molecular mechanisms underlying these associations impact function. addition, potential IFN signaling pathway as therapeutic target for IIMs will be discussed. Several clinical trials currently underway planned that using JAK inhibitors monoclonal antibodies against have shown promise treating DM, particularly refractory cases. However, more needed to fully understand efficacy safety profiles The concludes by highlighting importance ongoing this area new targeted therapies

Язык: Английский

Процитировано

0

Elevated Serum MCP‐2 and TARC Associated With Increased Risk of Death in Guamanian ALS Patients DOI Creative Commons
Risana N. Chowdhury,

Mahad Azam,

Saud Azam

и другие.

European Journal of Neurology, Год журнала: 2025, Номер 32(3)

Опубликована: Фев. 25, 2025

This study explores the relationship between inflammation and longevity in a high-incidence focus of amyotrophic lateral sclerosis (ALS) post-WWII Guam. Characteristics this include sudden appearance disease high numbers unusually long lifespan (without medical interventions) seen some cases. We used bio-banked specimens to evaluate serum immunoregulators survival time. evaluated sera from 69 Guam ALS cases collected within 2 years symptom onset by NIH researchers 1950 1983 for 11 via ELISA (CRP, eotaxin-1, RANTES, IL-6, IL-8, IL-10, IFN-γ, IP-10, MCP-1, MCP-2 TARC). Factor analysis identified two factors responsible ~68% variation data. estimated Cox proportional hazards models identify associated with time death. Each 10-unit increase factor cytokines (MCP-2 TARC) was 38% risk death (HR: 1.38; 95% CI: 1.19, 1.65; p: 0.00). Like sporadic worldwide, is shortened Guamanian ALS; more specifically, our findings suggest levels TARC at may predict duration. Further investigation needed determine role these prognosis as targets diagnostic therapeutic interventions.

Язык: Английский

Процитировано

0

Progress in the role and mechanism of TDP-43 DOI
Rongbing Li

New discovery., Год журнала: 2024, Номер unknown, С. 1 - 8

Опубликована: Сен. 20, 2024

Background: TAR DNA-binding protein 43 kDa (TDP-43) has been shown to play an important role in the development of neurodegenerative diseases, but mechanism is still under study. Methods: By utilizing “TDP43”, “disease”, and “mechanism” as keywords, 200 related studies were retrieved downloaded from Pubmed database, including 60 articles. We summarized progress understanding TDP-43 over past two years, focusing on disease systems classification upstream downstream, connection, improvement, formation. Results: TDP-43, when abnormally aggregated, phosphorylated, or mislocalized, plays a key pathological diseases. Additionally, its impact normal reproductive cell formation, development, quantity, activity, well insulin secretion activation intestinal epithelial necrosis, should not be overlooked. Mechanistically, we identified relationship between expression factors, Enterovirus D68 (EV-D68), Heterogeneous Nuclear Ribonucleoprotein D (HNRNPD AUF1), Endoplasmic Reticulum Protein 57 (ERp57), Progranulin (PGRN), downstream factors such Meiotic Recombination Spo11 (Spo11), AMP-Activated Kinase (AMPK), Double-Strand-Break Repair Rad21 Homolog (Rad21L), IκB (IKK), TDP-43. Conclusion: neurodegeneration, which, phosphorylation, EV-d68, HNRNPD.

Язык: Английский

Процитировано

0