Α-N-acetylgalactosaminidase
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Α-N-acetylgalactosaminidase
α-''N''-acetylgalactosaminidase () is a glycoside hydrolase from bacteria and animals, also known as nagalase. The human gene that codes for this enzyme is NAGA. Mutations in this gene and the deficiency in α-''N''-acetylgalactosaminidase activity have been identified as the cause of Schindler disease. Enzymes with this activity is useful for converting type A blood to type O, giving it the name of A-zyme. Bacterial enzymes in the GH109 family, including the A-zyme from ''Elizabethkingia meningoseptica ''Elizabethkingia meningoseptica'' is a Gram-negative, rod-shaped bacterium widely distributed in nature (e.g. fresh water, salt water, or soil). It may be normally present in fish and frogs; it may be isolated from chronic infectious states, as ...'', runs this reaction efficiently. Nagalase catalyzes the deglycosylation of the Gc protein also known as vitamin D3 binding protein rendering it incapable of being converted to the regulatory protein, Gc Macrophage Activating ...
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Glycoside Hydrolase
In biochemistry, glycoside hydrolases (also called glycosidases or glycosyl hydrolases) are a class of enzymes which catalysis, catalyze the hydrolysis of glycosidic bonds in polysaccharide, complex sugars. They are extremely common enzymes, with roles in nature including degradation of biomass such as cellulose (cellulase), hemicellulose, and starch (amylase), in anti-bacterial defense strategies (e.g., lysozyme), in pathogenesis mechanisms (e.g., viral neuraminidases) and in normal cellular function (e.g., trimming alpha-Mannosidase, mannosidases involved in N-linked glycoprotein, ''N''-linked glycoprotein biosynthesis). Together with glycosyltransferases, glycosidases form the major catalytic machinery for the synthesis and breakage of glycosidic bonds. Occurrence and importance Glycoside hydrolases are found in essentially all domains of life. In prokaryotes, they are found both as intracellular and extracellular enzymes that are largely involved in nutrient acquisitio ...
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NAGA (gene)
Alpha-N-acetylgalactosaminidase is an enzyme that in humans is encoded by the ''NAGA'' gene. NAGA encodes the lysosomal enzyme alpha-N-acetylgalactosaminidase, which cleaves alpha-N-acetylgalactosaminyl moieties from glycoconjugates In molecular biology and biochemistry, glycoconjugates are the classification family for carbohydrates – referred to as glycans – which are covalently linked with chemical species such as proteins, peptides, lipids, and other compounds. Gly .... Mutations in NAGA have been identified as the cause of Schindler disease types I and II (type II also known as Kanzaki disease). References Further reading * * * * * * * * * * * * * * * * * * * External links * Human proteins {{gene-22-stub ...
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Schindler Disease
Schindler disease, also known as Kanzaki disease and alpha-N-acetylgalactosaminidase deficiency, is a rare disease found in humans. This lysosomal storage disorder is caused by a deficiency in the enzyme alpha-NAGA ( alpha-N-acetylgalactosaminidase), attributable to mutations in the NAGA gene on chromosome 22, which leads to excessive lysosomal accumulation of glycoproteins. A deficiency of the alpha-NAGA enzyme leads to an accumulation of glycosphingolipids throughout the body. This accumulation of sugars gives rise to the clinical features associated with this disorder. Schindler disease is an autosomal recessive disorder, meaning that one must inherit an abnormal allele from both parents in order to have the disease. Types There are three main types of the disease each with its own distinctive symptoms. ''Type I'': infantile form, infants will develop normally until about a year old. At this time, the affected infant will begin to lose previously acquired skills involving ...
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Type A Blood
Type may refer to: Science and technology Computing * Typing, producing text via a keyboard, typewriter, etc. * Data type, collection of values used for computations. * File type * TYPE (DOS command), a command to display contents of a file. * Type (Unix), a command in POSIX shells that gives information about commands. * Type safety, the extent to which a programming language discourages or prevents type errors. * Type system, defines a programming language's response to data types. Mathematics * Type (model theory) * Type theory, basis for the study of type systems * Arity or type, the number of operands a function takes * Type, any proposition or set in the intuitionistic type theory * Type, of an entire function ** Exponential type Biology * Type (biology), which fixes a scientific name to a taxon * Dog type, categorization by use or function of domestic dogs Lettering * Type is a design concept for lettering used in typography which helped bring about modern textual printi ...
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Elizabethkingia Meningoseptica
''Elizabethkingia meningoseptica'' is a Gram-negative, rod-shaped bacterium widely distributed in nature (e.g. fresh water, salt water, or soil). It may be normally present in fish and frogs; it may be isolated from chronic infectious states, as in the sputum of cystic fibrosis patients. In 1959, American bacteriologist Elizabeth O. King (who isolated '' Kingella kingae'' in 1960) was studying unclassified bacteria associated with pediatric meningitis at the Centers for Disease Control and Prevention in Atlanta, when she isolated an organism (CDC group IIa) that she named ''Flavobacterium meningosepticum'' (''Flavobacterium'' means "the yellow bacillus" in Latin; ''meningosepticum'' likewise means "associated with meningitis and sepsis"). In 1994, it was reclassified in the genus ''Chryseobacterium'' and renamed ''Chryseobacterium meningosepticum''(''chryseos'' = "golden" in Greek, so ''Chryseobacterium'' means a golden/yellow rod similar to ''Flavobacterium''). In 2005, a 16S r ...
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EC 3
EC3 can refer to: People * Ethan Carter III (EC3) (born 1983), American professional wrestler Places * EC3, a district in the London EC postcode area Groups, organizations, companies * European Cybercrime Centre * EarthCheck, formerly EC3 Global; international tourism advisory group Transportation * BJEV ''EC3'', a Chinese electric vehicle * KUR ''EC3 class'', a class of steam locomotive * EC-3 radar, Italian WWII radar Other uses * Dolby Digital Plus, also known as EC-3 * Hydrolase enzymes (EC 3); see List of EC numbers (EC 3) See also * ECCC (other) {{Letter-NumberCombDisambig ...
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