Intrathecal Antibody Synthesis in Autoimmune Nodopathy.

Background: Autoimmune nodopathy (AN) is caused by autoantibodies targeting the nodes of Ranvier or paranodes. AN frequently affects cranial nerves and spinal nerve roots and may accompany central demyelination, all of which belong to the intrathecal compartment. We aimed to ascertain the frequency of intrathecal antibody synthesis and blood-CSF barrier (BCSFB) dysfunction in AN and their clinical correlates.

Regio- and Stereoselective Ribose Modification in Truncated 2,8-Disubstituted Adenosine Scaffold to Develop Highly Potent A Adenosine Receptor Antagonists.

Previous molecular dynamics simulations of hAAR with and structure-activity relationship (SAR) analyses prompted us to synthesize diol-, deoxy-, fluoro-, and methoxy-derivatives () in a regio- and stereoselective manner, modifying the ribose moiety to enhance potency and selectivity at hAAR. SAR analysis revealed that the presence of at least one hydroxyl group at either the R or R position is preferred for hAAR binding, and inversion of the R hydroxyl group significantly reduced binding affinity at hAAR. Alteration of the hydroxyl groups in the ribose moiety showed that hAAR favored diol derivatives, and the following trend was observed at hAAR: , diol ≈ deoxy < fluoro < methoxy.

Frequency-comb-referenced multiwavelength interferometry for high-precision and high-speed 3D measurement in heterogeneous semiconductor packaging.

As Moore's law approaches its physical limits, the semiconductor industry has begun to focus on improving I/O density and power efficiency through 2.5D/3D packaging. Heterogeneous integration, which combines integrated circuit blocks from different linewidth processes into a single package, is central to these developments.

Estimating the Prevalence of Autosomal Recessive Neuromuscular Diseases in the Korean Population.

Background: Genetic neuromuscular diseases (NMDs) are a heterogeneous group of conditions that primarily affect the peripheral nerves, muscles, and neuromuscular junctions. This study was performed to identify pathogenic or likely pathogenic variants (PLPVs), calculate carrier frequencies, and predict the genetic prevalence of autosomal recessive-NMDs (AR-NMDs) in a Korean population.

Methods: In total, 267 genes were associated with AR-NMDs.

Structure-Activity Relationship Analysis of 2-Aryl-8-alkynyl Adenine and Nucleoside Scaffolds as A Adenosine Receptor Ligands.

Numerous adenosine receptor (AR) ligands have been synthesized over many years, but the development of 2,8-disubstituted nucleoside derivatives was limited due to the lack of efficient synthetic methods. Leveraging Pd catalyst-controlled regioselective coupling, we embarked on structure-activity relationship (SAR) analyses at the C8 position. Our SAR studies revealed that an aliphatic alkyne chain of specific length, such as 1-hexyne, at the C8 position is ideally suited for hAAR interaction.

Outcomes of immunosuppressive therapy discontinuation in patients with myelin oligodendrocyte glycoprotein antibody-associated disease.

Background: Research on the optimal duration of immunosuppressive therapy (IST) and the outcome upon its discontinuation in myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) remains limited.

Objective: To evaluate the outcomes following IST discontinuation in MOGAD.

Methods: This multicenter retrospective study collected data from 333 MOGAD patients in Korea.

Early prediction of refractory myasthenia gravis based on response to treatment within the first year of diagnosis.

Background: The long lead time required to diagnose refractory myasthenia gravis (MG) can prevent early attempts at new treatments. Herein, we aimed to predict refractory MG by comparing treatment responses during the early stages between patients with refractory and non-refractory MG.

Methods: We retrospectively investigated the medical records of patients with acetylcholine receptor antibody-positive generalized MG.

In Situ Transfer of Laser-Induced Graphene Electronics for Multifunctional Smart Windows.

The ascent of internet of things (IoT) technology has increased the demand for glass electronics. However, the production of glass electronics necessitates complicated processes, including conductive materials coating and chemical vapor deposition, which entail the use of additional chemicals. Consequently, this raises environmental apprehensions concerning chemical and electronic waste.

Effect of the Administration of Cautionary Drugs on the Risk of Worsening Myasthenia Gravis: A Retrospective Matched Case-Control Study.

Purpose: Although some medications trigger the worsening of myasthenia gravis (MG), their clinical influence on patients with MG has not been significantly evaluated. We aimed to investigate whether the risk of clinical worsening of MG increases after administering cautionary drugs in patients with MG.

Materials And Methods: This retrospective case-control study was based on the medical records of patients diagnosed with MG between 2007 and 2020.

Surface Engineering of Perovskite Solar Cells via the Dry-Vacuum Process: Deposition of Lead Halides and Alkylammonium Halides.

Perovskite solar cells (PSCs) have demonstrated remarkably rapid efficiency improvements mainly through spin-coating-based solution processes. While these processes offer numerous advantages, there are also several limitations, prompting research into alternative fabrication methodologies for PSCs. Meanwhile, surface engineering has been identified as one of the most critical factors for enhancing the efficiency and stability of PSCs.

Development and Application of a Cell-Based Assay for Detecting Anti-Agrin Antibodies Associated With Myasthenia Gravis.

Background And Purpose: Anti-agrin antibodies (agrin Abs) have recently been identified in patients with myasthenia gravis (MG), sometimes in conjunction with antibodies (Abs) to the acetylcholine receptor (AChR), muscle-specific tyrosine kinase (MuSK), or low-density lipoprotein receptor-related protein 4. This study aimed to develop an in-house cell-based assay (CBA) for detecting agrin Abs, and to test its application to serum samples collected from individuals diagnosed with MG.

Methods: Agrin complementary DNA as cloned into a pCMV6-AC-GFP vector, which was subsequently transfected into human embryonic kidney 293T (HEK293T) cells.

Design, synthesis, and biological evaluation of 5'-deoxy (N)-methanocarbanucleoside derivatives as A adenosine receptor ligands.

Based on the potent and selective antagonism exhibited by truncated North (N)-methanocarba adenosine analogs, we synthesized a series of 5'-deoxy (N)-methanocarba nucleosides to explore their structure-activity relationships (SAR). The stereoselective synthesis of the North cyclopropyl-fused alcohol was achieved from d-ribose using ring-closing metathesis, oxidative rearrangement, and cyclopropanation as key steps. Mitsunobu reactions were employed to condense nucleobases with glycosyl donors, followed by N functionalization with various amines.

Assessment of concurrent neoplasms and a paraneoplastic association in MOGAD.

Cases of myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease (MOGAD) co-occurring with neoplasms have been reported. In this international, retrospective cohort study in South Korea and the USA, 16 of 445 (3.6%) patients with MOGAD had concurrent neoplasm within 2 years of MOGAD onset, resulting in a standardized incidence ratio for neoplasm of 3.

Platelet-derived HMGB1 induces NETosis, exacerbating brain damage in the photothrombotic stroke model.

Following cerebral ischemia, neutrophil extracellular traps (NETs) contribute significantly to brain damage by exacerbating delayed immune cell infiltration and vascular injury. They are detected both in brain tissue and within blood vessels. Danger-associated molecular pattern (DAMP) molecules have been implicated in inducing NETosis after cerebral ischemia.

Synthesis and Biological Evaluation of 5'-Deoxy-adenosine Derivatives as A Adenosine Receptor Ligands.

The A Adenosine Receptor (AAR) is an important therapeutic target due to its role in inflammation and immune response regulation. Herein, we synthesized and evaluated 5'-deoxy-adenosine derivatives with oxygen at the 4'-position, comparing them to previously studied 4'-thionucleosides. Compound exhibited the highest binding affinity ( = 5.

Profiling of Anti-Signal-Recognition Particle Antibodies and Clinical Characteristics in South Korean Patients With Immune-Mediated Necrotizing Myopathy.

Background And Purpose: This study evaluated the diagnostic utility of an anti-signal-recognition particle 54 (anti-SRP54) antibody-based enzyme-linked immunosorbent assay (ELISA) as well as the clinical, serological, and pathological characteristics of patients with SRP immune-mediated necrotizing myopathy (IMNM).

Methods: We evaluated 87 patients with idiopathic inflammatory myopathy and 107 healthy participants between January 2002 and December 2023. The sensitivity and specificity of the ELISA for anti-SRP54 antibodies were assessed, and the clinical profiles of patients with anti-SRP54 antibodies were determined.

Neuroprotective effect of L-DOPA-induced interleukin-13 on striatonigral degeneration in cerebral ischemia.

Levodopa (L-DOPA) treatment is a clinically effective strategy for improving motor function in patients with ischemic stroke. However, the mechanisms by which modulating the dopamine system relieves the pathology of the ischemic brain remain unclear. Emerging evidence from an experimental mouse model of ischemic stroke, established by middle cerebral artery occlusion (MCAO), suggested that L-DOPA has the potential to modulate the inflammatory and immune response that occurs during a stroke.

Synthesis and biological evaluation of sugar-modified truncated carbanucleosides as A and A adenosine receptor ligands to explore conformational effect to the receptors.

This study investigated the impact of conformation on the binding affinity of carbanucleosides to A and A adenosine receptors (ARs). A series of nucleosides, including saturated, unsaturated, North (N)-methano, and South (S)-methanocarbanucleosides was prepared, and their binding affinities to AAR and AAR were assessed. Biological evaluations revealed that all synthesized (S)-methanocarbanucleosides had negligible binding to both receptors, and most (N)-methanocarbanucleosides exhibited high binding affinities.

Exploring C2 and N Substituent Effects on Truncated 4'-Thioadenosine Derivatives as Dual A and A Adenosine Receptor Ligands.

Based on high binding affinity of truncated 2-hexynyl-4'-thioadenosine (3 a) at both A adenosine receptor (AR) and A AR, we explored structure-activity relationship (SAR) of the C2-substitution by altering chain length of the 2-hexynyl moiety, thereby evaluating the hydrophobic pocket size. A series of truncated N-substituted 4'-thioadenosine derivatives with C2-alkynyl substitution were successfully synthesized from D-mannose, using a palladium-catalyzed Sonogashira coupling reaction as the key step, whose structures were confirmed by the X-ray crystal structure of 4 h. As the size of the alkynyl group at the C2-position increased, the binding affinity improved; however, when the substituted group was larger than hexynyl, the binding affinity decreased.

Validation and proposal of case definitions for identifying patients with myasthenia gravis.

Background And Purpose: Public medical insurance data are increasingly used to study myasthenia gravis (MG); however, a validated case definition is lacking. We assessed the clinical characteristics of patients identified according to previously used case definitions.

Methods: Patients with diagnosis code G70.