Glycans in Sera of Amyotrophic Lateral Sclerosis Patients and Their Role in Killing Neuronal Cells

Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease caused by degeneration of upper and lower motor neurons. To date, glycosylation patterns of glycoproteins in fluids of ALS patients have not been described. Moreover, the aberrant glycosylation related to the pathogenesis of other neurodegenerative diseases encouraged us to explore the glycome of ALS patient sera. We found high levels of sialylated glycans and low levels of core fucosylated glycans in serum-derived N-glycans of patients with ALS, compared to healthy volunteer sera. Based on these results, we analyzed the IgG Fc N297-glycans, as IgG are major serum glycoproteins affected by sialylation or core fucosylation and are found in the motor cortex of ALS patients. The analyses revealed a distinct glycan, A2BG2, in IgG derived from ALS patient sera (ALS-IgG). This glycan increases the affinity of IgG to CD16 on effector cells, consequently enhancing Antibody-Dependent Cellular Cytotoxicity (ADCC). Therefore, we explore whether the Fc-N297-glycans of IgG may be involved in ALS disease. Immunostaining of brain and spinal cord tissues revealed over-expression of CD16 and co-localization of intact ALS-IgG with CD16 and in brain with activated microglia of G93A-SOD1 mice. Intact ALS-IgG enhanced effector cell activation and ADCC reaction in comparison to sugar-depleted or control IgG. ALS-IgG were localized in the synapse between brain microglia and neurons of G93A-SOD1 mice, manifesting a promising in vivo ADCC reaction. Therefore, glycans of ALS-IgG may serve as a biomarker for the disease and may be involved in neuronal damage

Erratum to: Changes in patterns of uveitis at a tertiary referral center in Northern Italy: analysis of 990 consecutive cases

Erratum to: Changes in patterns of uveitis at a tertiary referral center in Northern Italy: analysis of 990 consecutive case

Post-intervention Status in Patients With Refractory Myasthenia Gravis Treated With Eculizumab During REGAIN and Its Open-Label Extension

OBJECTIVE: To evaluate whether eculizumab helps patients with anti-acetylcholine receptor-positive (AChR+) refractory generalized myasthenia gravis (gMG) achieve the Myasthenia Gravis Foundation of America (MGFA) post-intervention status of minimal manifestations (MM), we assessed patients' status throughout REGAIN (Safety and Efficacy of Eculizumab in AChR+ Refractory Generalized Myasthenia Gravis) and its open-label extension. METHODS: Patients who completed the REGAIN randomized controlled trial and continued into the open-label extension were included in this tertiary endpoint analysis. Patients were assessed for the MGFA post-intervention status of improved, unchanged, worse, MM, and pharmacologic remission at defined time points during REGAIN and through week 130 of the open-label study. RESULTS: A total of 117 patients completed REGAIN and continued into the open-label study (eculizumab/eculizumab: 56; placebo/eculizumab: 61). At week 26 of REGAIN, more eculizumab-treated patients than placebo-treated patients achieved a status of improved (60.7% vs 41.7%) or MM (25.0% vs 13.3%; common OR: 2.3; 95% CI: 1.1-4.5). After 130 weeks of eculizumab treatment, 88.0% of patients achieved improved status and 57.3% of patients achieved MM status. The safety profile of eculizumab was consistent with its known profile and no new safety signals were detected. CONCLUSION: Eculizumab led to rapid and sustained achievement of MM in patients with AChR+ refractory gMG. These findings support the use of eculizumab in this previously difficult-to-treat patient population. CLINICALTRIALSGOV IDENTIFIER: REGAIN, NCT01997229; REGAIN open-label extension, NCT02301624. CLASSIFICATION OF EVIDENCE: This study provides Class II evidence that, after 26 weeks of eculizumab treatment, 25.0% of adults with AChR+ refractory gMG achieved MM, compared with 13.3% who received placebo

Influence of surface preparation in resin/enamel interface: A SEM study.

37th Annual Meeting of the International-Association-for-Dental-Research -- SEP 05-08, 2001 -- ROME, ITALYWOS: 000180217902057…Int Assoc Dent Re

Effect of ohmic treatment on quality characteristic of meat: A review

WOS: 000314735100012PubMed ID: 23273448Ohmic cooking, a well-known electro-heating technique, provides an alternative method for cooking meat products due to its ability for rapid heat generation. Ohmic heating uses the resistance of meat products to convert the electric energy into heat. The rate of heat generation depends on the voltage gradient applied and the electrical conductivity of the meat product. The advantages of ohmic cooking over conventional heating include shorter processing times, higher yields, and less power consumption while still maintaining the colour and nutritional value of meat products. In recent years, ohmic cooking has increasingly drawn interest from the meat industry as a method to ensure the quality and the safety of meat products. The present paper reviews the effects of ohmic cooking on the physical, chemical, sensory, and microbiological quality characteristics and toxicological properties of meat and meat products. (c) 2012 Elsevier Ltd. All rights reserved.The Scientific and Technological Research Council of Turkey (TUBITAK)Turkiye Bilimsel ve Teknolojik Arastirma Kurumu (TUBITAK) [1100068]The authors are grateful for the financial support provided for the project entitled "Setting up and performance evaluation of ohmic-infrared heating system, and the investigation of its use in koefte cooking by considering its effects on some quality attributes" under project no: 1100068 by The Scientific and Technological Research Council of Turkey (TUBITAK)

Vestibular impairment in Charcot-Marie-Tooth disease

5th Congress of the European-Academy-of-Neurology (EAN) -- JUN 29-JUL 02, 2019 -- Oslo, NORWAYWOS: 00047448100029