• Journal of Microbiology and Biotechnology
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• v.17 no.12
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• pp.2027-2032
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• 2007

[ ${\alpha}$ ]-Synuclein is the major component of Lewy bodies and responsible for the amyloid deposits observed in Parkinson's disease. Ordered filamentous aggregate formation of the natively unfolded ${\alpha}$-synuclein was investigated in vitro with the periodic ultrasonication. The ultrasonication induced the fibrillation of ${\alpha}$-synuclein, as the random structure gradually converted into a ${\beta}$-sheet structure. The resulting fibrils obtained at the stationary phase appeared heterogeneous in their size distribution, with the average length and height of $0.28\;{\mu}m{\pm}0.21\;{\mu}m$ and $5.6\;nm{\pm}1.9\;nm$, respectively. After additional extensive ultrasonication in the absence of monomeric ${\alpha}$-synuclein, the equilibrium between the fibril formation and its breakdown shifted to the disintegration of the preexisting fibrils. The resulting fragments served as nucleation centers for the subsequent seed-dependent accelerated fibrillation under a quiescent incubation condition. This self-seeding amplification process depended on the seed formation and subsequent alterations in their properties by the ultrasonication to a state that accretes the monomeric soluble protein more effectively than their reassociation of the seeds back to the original fibrils. Since many neurodegenerative disorders have been considered to be propagated via the seed-dependent amyloidosis, this study would provide a novel aspect of the significance of the seed structure and its properties leading to the acce]erated amyloid formation.

• Journal of Veterinary Clinics
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• v.23 no.2
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• pp.186-189
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• 2006

A 7-month-old female Cocker spaniel dog was examined for chronic anemia. Based on information provided by local clinician the patient had had a 'flu-like' illness three weeks before submission of the sample, had a fever of $40.9^{\circ}C$, and had mild hepatomegaly. This dog had also history of weight loss, vomiting, anorexia, dehydration, lethargy, ascites, polyuria and polydipsia. A blood smear showed non-regenerative anemia. Thoracic radiograph showed irregular shadowing in the left mid-zone. Serum biochemical results showed a hypercalcemia, azotemia, hypercholesterolemia, hyperphosphatemia, hypoalbuminemia, and metabolic acidosis. Results of urinalysis showed proteinuria, slightly acidic with isosthenuria. Histopathologic examination of tissue sections revealed amyloid deposits in multiple sites including kidneys, liver and spleen.

• Journal of the Korean Society of Laryngology, Phoniatrics and Logopedics
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• v.19 no.1
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• pp.47-53
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• 2008

Background and Objectives : Benign neoplasms of the larynx are rare, and papillomas account for approximately 90% of these neoplasms. Other benign neoplasms of the larynx are very rare and form a hetergenous group. We present clinical manifestations of unusual benign neoplasms based on our experiences and review of literatures. Materials and Method : We reviewed retrospectively the clinical records of 14 patients with benign neoplasms of the larynx, excluding papillomas, that were examined in our department during 11-year period from 1995 to 2006. Results : The presenting symptom was most commonly progressive dysphonia. Pathologic diagnosis revealed 5 cases of hemangioma, 3 granular cell tumor, 2 amyloidosis, 2 laryngocele, 1 schwannoma, 1 chondroma. Subsites of the neoplasms were 5 in true vocal cord, 3 in arytenoids, 2 in false vocal cord, 2 in supraglottis, and others were subglottis ; aryepiglottic fold. Treatment was surgical, by a external approach in 1 case of chondroma, and by laryngoscopic approach in other cases. In laryngoscopic approach, carbon dioxide laser was used in 10 cases. Postoperative course was satisfactory. Recurrence was encountered in I case of amyloidosis and revision operation was done 3 times. No recurrence was encountered in other cases. Conclusion : Uncommon benign neoplasms of the larynx require high index of suspicion and histological confirmation. Complete excision with an attempt to maintain normal structures generally results in cure.

• Annals of Clinical Neurophysiology
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• v.10 no.2
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• pp.101-103
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• 2008

Many neurologists, particularly whose subspecialty is in peripheral neurology, may agree that patients with peripheral neuropathy often complain of the "restless legs" symptoms. These symptoms seem to share the typical features of the so-called "restless legs syndrome (RLS)", i.e., unpleasant sensations in the leg/feet, worsening in the evening or at night, and the partial relief of the positive sensory symptoms by the movements such as walking, shaking or rubbing. In fact, a higher incidence of RLS was reported among the neuropathic patients, and peripheral neuropathy was found to be more prevalent in patients with RLS than in general population. Moreover, RLS share many risk factors with peripheral neuropathy such as diabetes, uremia, amyloidosis and cryoglobulinemia, which suggests that peripheral neuropathy may play a pathophysiologic role in the development of RLS.

• Childhood Kidney Diseases
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• v.2 no.1
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• pp.73-76
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• 1998

Behcet syndrome is a multisystem disease complex of unknown cause. It is usually manifested by aphthous oral and genital ulcers, uveitis and skin eruption. Less frequently, CNS involvement, colitis, large vessel vasculitis, and myocarditis occur. Recently, several studies have reported renal manifestations of amyloidosis and focal necrotizing glomerulonephritis in Behcet syndrome. We describe a patient with Behcet syndrome who experienced nephrotic syndrome with focal effacement of epithelial foot process in glomeruli. A brief review of literature ensues.

• Biotechnology and Bioprocess Engineering:BBE
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• v.6 no.4
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• pp.237-243
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• 2001

Substantial progress has been made towards understanding the folding mechanisms of proteins in virto and in vivo even though the general rules governing such folding events remain unknown. This paper reviews current folding models along with experimental approaches used to elucidate the folding pathways. Protein misfolding is discussed in relation to disease states, such as amyloidosis, and the recent findings on the mechanism of converting normally soluble proteins into amyloid fibrils through the formation of intermediates provide an insight into understanding the pathogenesis of amyloid formation and possible cules for the development of therapeutic treatments. Finally, some commonly adopted refolding strategies developed over the part decade are summarized.

• Childhood Kidney Diseases
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• v.19 no.1
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• pp.43-47
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• 2015

Hemolytic anemia and thrombocytopenia are rare clinical manifestations of acute glomerulonephritis. Initially, in all such cases, a diagnosis of hemolytic uremic syndrome, thrombotic thrombocytopenic purpura, systemic lupus erythematosus, and amyloidosis should be ruled out. The presence of hemolytic anemia and thrombocytopenia is rare, but possible, in a case of acute poststreptococcal glomerulonephritis, and may result in delayed diagnosis or misdiagnosis. Correct and timely diagnosis would ensure adequate treatment in such patients. We report of a 22-month-old boy with acute glomerulonephritis coexistent with hemolytic anemia and idiopathic thrombocytopenia.

• Kosin Medical Journal
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• v.33 no.2
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• pp.200-207
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• 2018

Tracheobronchopathia osteoplastica (TO) is a rare benign disease in which the anterior inner wall of the tracheobronchus changes because of abnormal chondrogenesis or ossification, while the posterior wall of the trachea is spared. The etiology is not clearly understood, but may relate with chronic infection, inflammation, and trauma. In some case studies, it has also been reported to be accompanied by other chronic diseases such as atrophic rhinitis and amyloidosis. However, Coexistence of TO and tuberculosis has rarely been reported, and has never been reported in Korea. Here, we report a case of a 70-year-old male patient who complained of hemoptysis and whose case was diagnosed as TO and pulmonary tuberculosis through bronchoscopy with bronchial washing and biopsy.

• Journal of the Korean Magnetic Resonance Society
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• v.24 no.4
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• pp.129-135
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• 2020

In a relatively short period, monoclonal antibodies have made dramatic success as therapeutics for various diseases such as cancers and autoimmune diseases and become an important development items for many pharmaceutical companies. In order to develop antibody drug, it is important to investigate the structural characteristics of both antibody and antigen. NMR studies on antibody are extremely challenging due to big huddles such as a big size of protein and isotope labeling, nevertheless, several studies have been reported in 10 years. Here, we analyzed 95 papers dealing with antibody-related NMR studies reported in recent 10 years. We categorized papers into 3 types: 1) structural characterization of antibody, 2) structural characterization of antigen using antibody, 3) amyloidosis caused by fragment of antibody. This work would shed new light on antibody-related NMR studies.

• Journal of the Korean Magnetic Resonance Society
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• v.27 no.1
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• pp.1-4
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• 2023

Transthyretin (TTR) is an indispensable transporter protein of thyroxine and a retinol molecule in humans. TTR has a stable homo-tetrameric structure in its native state, while upon dissociation into monomers, it becomes aggregation-prone and can form an amyloid fibril. Although the amyloidogenic propensity of TTR has been known and investigated since the late 1990s, the structural information regarding TTR's amyloidogenic species is still elusive. Here, we employed high-pressure nuclear magnetic resonance (HP-NMR) approaches on the monomeric variant of TTR (TTR[F87M/L110M]; M-TTR) and observed that it experiences a two-step transition in response to the pressurized condition. Our study demonstrated that M-TTR in an ambient condition has heterogeneous structural features, which is likely related to the amyloidogenic propensity of TTR.