Impaired GABAergic Regulation and Developmental Immaturity in Interneurons Derived from the Medial Ganglionic Eminence in the Tuberous Sclerosis Complex

Hippocampal Neurons - The Destructive Force Behind Epilepsy and Mental Disorders Background Introduction Tuberous Sclerosis Complex (TSC) is a complex multi-system genetic disorder that manifests with lesions in the brain, skin, heart, kidneys, and other organs as the patient ages. Clinically, TSC presents with symptoms such as epilepsy and develop...

Physiological aging and inflammation-induced cellular senescence may contribute to oligodendroglial dysfunction in MS

Background Introduction In this paper, the researchers discuss the impact of aging on the functionality of all cell types in the central nervous system (CNS) and its role in neurological diseases such as multiple sclerosis (MS). However, the molecular mechanisms underlying these age-related changes and their contribution to diseases remain poorly u...

Aberrant CHCHD2-Associated Mitochondriopathy in Kii ALS/PDC Astrocytes

Study Report on Abnormal CHCHD2-Related Mitochondrial Pathology in Astrocytes in Kii ALS/PDC I. Research Background Amyotrophic lateral sclerosis/Parkinsonism-dementia complex (ALS/PDC) is a rare and complex neurodegenerative disorder primarily observed in Western Pacific islands such as Japan, Guam, and Papua New Guinea. Patients with this disease...

Disentangling the Heterogeneity of Multiple Sclerosis Through Identification of Independent Neuropathological Dimensions

Research Background Multiple Sclerosis (MS) is a common disease involving lesions in the central nervous system, characterized mainly by demyelination of neuronal axons and neuronal damage. The heterogeneity of the disease is extremely high, meaning that different patients exhibit varying symptoms and pathological mechanisms, which greatly complica...

Co-registration of MALDI-MSI and histology demonstrates gangliosides co-localize with amyloid beta plaques in Alzheimer’s disease

Co-registration of MALDI-MSI and histology demonstrates gangliosides co-localize with amyloid beta plaques in Alzheimer’s disease

Co-localization of Gangliosides with Amyloid Beta Plaques in Alzheimer’s Disease Detected by MALDI-MSI and Histology Alzheimer’s Disease (AD) is a progressive neurodegenerative disease characterized by cognitive impairment and behavioral changes. Historically, AD research has focused on misfolded proteins, but with advancements in mass spectrometry...

Annexin A11 Aggregation in FTLD-TDP Type C and Related Neurodegenerative Disease Proteinopathies

Study on Annexin A11 Aggregation and TDP-43 Proteinopathies in Neurodegenerative Diseases In this research report published in Acta Neuropathologica, researchers led by John L. Robinson from the University of Pennsylvania investigate the aggregation phenomena of Annexin A11 in neurodegenerative diseases, particularly in FTLD-TDP Type C, which is as...

Alteration of Gene Expression and Protein Solubility of the PI5-Phosphatase SHIP2 are Correlated with Alzheimer’s Disease Pathology Progression

Changes in Gene Expression and Protein Insolubility in Alzheimer’s Disease Progression Background Introduction As the most common type of dementia, Alzheimer’s Disease (AD) is well-known for its two main neuropathological features: amyloid plaques and neurofibrillary tangles (NFTs). Amyloid plaques consist of β-amyloid (Aβ) peptide segments generat...

Inter-alpha-trypsin inhibitor heavy chain h3 is a potential biomarker for disease activity in myasthenia gravis

Research Background Myasthenia Gravis (MG) is a chronic antibody-mediated autoimmune disease that primarily affects synaptic transmission at the neuromuscular junction. Approximately 85% of MG patients are antibody-mediated targeting acetylcholine receptors (AChR). The clinical features of this disease include muscle weakness, especially fatigue-in...

Characterization of NEB Pathogenic Variants in Patients Reveals Novel Nemaline Myopathy Disease Mechanisms and Omecamtiv Mecarbil Force Effects

Scientific Report: Analysis of NEB Pathogenic Variants Reveals Novel Mechanisms of Nemaline Myopathy and the Mechanical Effects of Omecamtiv Mecarbil Background and Motivation Nemaline Myopathy (NEM) is a rare and heterogeneous genetic disorder primarily characterized by hypotonia and muscle weakness. Pathologically, the disease is caused by the di...

BTK Inhibition Limits Microglia-Perpetuated CNS Inflammation and Promotes Myelin Repair

Research Report: Effect of Bruton Tyrosine Kinase Inhibitor Evobrutinib on Myelin Repair and CNS Inflammation in Multiple Sclerosis Background Introduction Multiple Sclerosis (MS) is a demyelinating disease of the Central Nervous System (CNS) where the myelin sheath of patients is damaged by inflammation. Traditionally, MS is considered to be prima...