نتایج جستجو برای: hexosaminidase b

تعداد نتایج: 899988  

Background Tay-Sachs disease is an autosomal-recessive lysosomal storage metabolic disorder. The typical symptoms of the disease include ataxia, muscle weakness, and mental disorders. The severity of the clinical symptom relies on the enzymatic activity of residual Hexosaminidase-A. Case Presentation</...

2006
Noboru Tomiya Someet Narang Jung Park Badarulhisam Abdul-Rahman One Choi Sundeep Singh Jun Hiratake Kanzo Sakata Michael J. Betenbaugh Karen B. Palter Yuan C. Lee

PURIFICATION, CHARACTERIZATION, AND CLONING OF A SPODOPTERA FRUGIPERDA SF9 β-N-ACETYLHEXOSAMINIDASE THAT HYDROLYZES TERMINAL N-ACETYLGLUCOSAMINE ON N-GLYCAN CORE Noboru Tomiya, Someet Narang, Jung Park, Badarulhisam Abdul-Rahman, One Choi, Sundeep Singh, Jun Hiratake, Kanzo Sakata, Michael J. Betenbaugh, Karen B. Palter, Yuan C. Lee Department of Biology, Department of Chemical and Biomolecular...

Journal: :Immunochemistry 1975
Y Ben-Yoseph B Geiger R Arnon

Abstraet--N-Acetyl-hexosaminidase (Hex) exists in various human tissues in two major isozymic forms--Hex A and Hex B. The main difference between the two forms is the lability of Hex A to both heat and acid as compared to the stability of Hex B. When heated to 50°C for 2-3 hr Hex A loses its entire enzymatic activity. In the present study we demonstrate that specific antiserum stabilizes Hex A ...

Journal: :Clinical chemistry 1988
E Branum L Cummins M Bartilson M Hopper S Pruett J F O'Brien

We compared acid citrate-dextrose (ACD-B) and heparin to determine which anticoagulant better preserves leukocytes for lysosomal enzyme assays if processing was done immediately or delayed for 24 h or more. Twenty normal subjects had blood drawn into tubes containing either ACD-B or heparin. The leukocytes were isolated by sedimentation in dextran (50 g/L) less than 2, 24, 48, and 72 h later. T...

Journal: :Science 2004
Dapeng Zhou Jochen Mattner Carlos Cantu Nicolas Schrantz Ning Yin Ying Gao Yuval Sagiv Kelly Hudspeth Yun-Ping Wu Tadashi Yamashita Susann Teneberg Dacheng Wang Richard L Proia Steven B Levery Paul B Savage Luc Teyton Albert Bendelac

NKT cells represent a distinct lineage of T cells that coexpress a conserved alphabeta T cell receptor (TCR) and natural killer (NK) receptors. Although the TCR of NKT cells is characteristically autoreactive to CD1d, a lipid-presenting molecule, endogenous ligands for these cells have not been identified. We show that a lysosomal glycosphingolipid of previously unknown function, isoglobotrihex...

Journal: :Proceedings of the National Academy of Sciences of the United States of America 2014
Shih-Hsin Kan Mika Aoyagi-Scharber Steven Q Le Jon Vincelette Kazuhiro Ohmi Sherry Bullens Daniel J Wendt Terri M Christianson Pascale M N Tiger Jillian R Brown Roger Lawrence Bryan K Yip John Holtzinger Anil Bagri Danielle Crippen-Harmon Kristen N Vondrak Zhi Chen Chuck M Hague Josh C Woloszynek Diana S Cheung Katherine A Webster Evan G Adintori Melanie J Lo Wesley Wong Paul A Fitzpatrick Jonathan H LeBowitz Brett E Crawford Stuart Bunting Patricia I Dickson Elizabeth F Neufeld

Mucopolysaccharidosis type IIIB (MPS IIIB, Sanfilippo syndrome type B) is a lysosomal storage disease characterized by profound intellectual disability, dementia, and a lifespan of about two decades. The cause is mutation in the gene encoding α-N-acetylglucosaminidase (NAGLU), deficiency of NAGLU, and accumulation of heparan sulfate. Impediments to enzyme replacement therapy are the absence of ...

Journal: :International journal of molecular medicine 2016
Jae-Myung Yoo Ju Hye Yang Hye Jin Yang Won-Kyung Cho Jin Yeul Ma

Arctium lappa fruit has been used in traditional medicine, and it is known to exert beneficial effects, such as antioxidant, anti-inflammatory and anticancer effects. However, the effects of the Arctium lappa fruit on the allergic response remain unknown. In this study, we evaluated the anti-allergic effects of Arctium lappa fruit extract (AFE) and its fermented form (F-AFE) using immunoglobuli...

2013
Napoleon Waszkiewicz Beata Zalewska-Szajda Sylwia Chojnowska Sławomir Dariusz Szajda Anna Zalewska Beata Konarzewska Agata Szulc Aleksandra Wojtulewska-Supron Alina Kępka Małgorzata Knaś Jerzy Robert Ładny Robert Milewski Krzysztof Zwierz

BACKGROUND Severe periodontitis leading to tooth loss is found in 5-15% of most populations worldwide. AIM The applicability of salivary β -hexosaminidase (β-HEX A%, percentage of β-HEX A isoenzyme to total β-HEX) and β-HEX B% (β-HEX B/β-HEX) indexes was investigated as a possible marker of periodontitis. METHODS Thirty three alcohol-dependent smokers (AS) and 32 healthy controls (C) were e...

Journal: :Arthritis Research & Therapy 2009
Mária Pásztói György Nagy Pál Géher Tamás Lakatos Kálmán Tóth Károly Wellinger Péter Pócza Bence György Marianna C Holub Ágnes Kittel Krisztina Pálóczy Mercédesz Mazán Péter Nyirkos András Falus Edit I Buzas

INTRODUCTION Similar to matrix metalloproteinases, glycosidases also play a major role in cartilage degradation. Carbohydrate cleavage products, generated by these latter enzymes, are released from degrading cartilage during arthritis. Some of the cleavage products (such as hyaluronate oligosaccharides) have been shown to bind to Toll-like receptors and provide endogenous danger signals, while ...

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