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Α-Galactosidase
α-Galactosidase ( EC 3.2.1.22, α-GAL, α-GAL A; systematic name α-D-galactoside galactohydrolase) is a glycoside hydrolase enzyme that catalyses the following reaction: : Hydrolysis of terminal, non-reducing α-D-galactose residues in α-D-galactosides, including galactose oligosaccharides, galactomannans and galactolipids It catalyzes many catabolic processes, including cleavage of glycoproteins, glycolipids, and polysaccharides. The enzyme is encoded by the ''GLA'' gene. Function This enzyme is a homodimeric glycoprotein that hydrolyses the terminal α-galactosyl moieties from glycolipids and glycoproteins. It predominantly hydrolyzes ceramide trihexoside, and it can catalyze the hydrolysis of melibiose into galactose and glucose. Reaction mechanism Applications α-Galactosidase from ''Aspergillus niger'' is the active ingredient in Beano, a dietary supplement for bloating and flatulence. Recombinant α-galactosidase made by baker's yeast is approved in Eur ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
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 ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Enzyme
An enzyme () is a protein that acts as a biological catalyst by accelerating chemical reactions. The molecules upon which enzymes may act are called substrate (chemistry), substrates, and the enzyme converts the substrates into different molecules known as product (chemistry), products. Almost all metabolism, metabolic processes in the cell (biology), cell need enzyme catalysis in order to occur at rates fast enough to sustain life. Metabolic pathways depend upon enzymes to catalyze individual steps. The study of enzymes is called ''enzymology'' and the field of pseudoenzyme, pseudoenzyme analysis recognizes that during evolution, some enzymes have lost the ability to carry out biological catalysis, which is often reflected in their amino acid sequences and unusual 'pseudocatalytic' properties. Enzymes are known to catalyze more than 5,000 biochemical reaction types. Other biocatalysts include Ribozyme, catalytic RNA molecules, also called ribozymes. They are sometimes descr ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
GLA (gene)
Galactosidase alpha is an enzyme that in humans is encoded by the ''GLA'' gene. Two recombinant forms of human α-galactosidase are called agalsidase alpha (INN) and agalsidase beta (INN). A mold-derived form is the primary ingredient in gas relief supplements. Disease relevance Fabry disease Signs and Symptoms Defects in human α-GAL result in Fabry disease, a rare lysosomal storage disorder and sphingolipidosis that results from a failure to catabolize α-D-galactosyl glycolipid moieties. Characteristic features include episodes of pain in hands and feet ( acroparesthesia), dark red spots on skin ( angiokeratoma), decreased sweating (hypohidrosis), decreased vision (corneal opacity), gastrointestinal problems, hearing loss, tinnitus, etc.. Complications may be life-threatening and may include progressive kidney damage, heart attack, and stroke. This disease may have late onset and only affect the heart or kidneys. Fabry disease is an X-linked disease, affecting 1 i ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Ceramide
Ceramides are a family of waxy lipid molecules. A ceramide is composed of sphingosine and a fatty acid joined by an amide bond. Ceramides are found in high concentrations within the cell membrane of Eukaryote, eukaryotic cells, since they are component lipids that make up sphingomyelin, one of the major lipids in the lipid bilayer. Contrary to previous assumptions that ceramides and other sphingolipids found in cell membrane were purely supporting structural elements, ceramide can participate in a variety of cellular lipid signaling, signaling: examples include regulating cell differentiation, differentiation, cell proliferation, proliferation, and programmed cell death (PCD) of Cell (biology), cells. The word ''ceramide'' comes from the Latin ''cera'' (wax) and ''amide''. Ceramide is a component of vernix caseosa, the waxy or cheese-like white substance found coating the skin of newborn human infants. Pathways for ceramide synthesis There are three major pathways of ceramide ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Melibiose
Melibiose is a reducing disaccharide formed by an α-1,6 linkage between galactose and glucose (D-Gal-(α1→6)-D-Glc). It differs from lactose in the chirality of the carbon where the galactose ring is closed and that the galactose is linked to a different point on the glucose moiety. It can be formed by invertase-mediated hydrolysis of raffinose, which produces melibiose and fructose. Melibiose can be broken down into its component saccharides, glucose and galactose, by the enzyme alpha-galactosidase, such as MEL1 from ''Saccharomyces pastorianus'' (lager yeast). Melibiose cannot be used by ''Saccharomyces cerevisiae ''Saccharomyces cerevisiae'' () (brewer's yeast or baker's yeast) is a species of yeast (single-celled fungal microorganisms). The species has been instrumental in winemaking, baking, and brewing since ancient times. It is believed to have be ...'' (ale yeast), so this is one test to differentiate between the two yeast species. References {{reflist Disa ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Reaction Mechanism For Human α-GAL
Reaction may refer to a process or to a response to an action, event, or exposure. Physics and chemistry *Chemical reaction *Nuclear reaction *Reaction (physics), as defined by Newton's third law * Chain reaction (other) Biology and medicine *Adverse drug reaction *Allergic reaction *Reflex, neural reaction *Hypersensitivity, immune reaction *Intolerance (other) * Light reaction (other) Psychology *Emotional, reaction * Reactivity *Proactivity, opposite of reactive behaviour *Reactive attachment disorder Politics and culture *Reactionary, a political tendency *Reaction video *Commentary (other) Proper names and titles * ''Reaction'' (album), a 1986 album by American R&B singer Rebbie Jackson ** "Reaction" (song), the title song from the Rebbie Jackson album *"Reaction", a single by Dead Letter Circus *''Reactions'', a 2018 album by The Mods *ReAction GUI, a GUI toolkit used on AmigaOS * Reaction.life, a political news and commentary web ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Beano (dietary Supplement)
Beano is an enzyme-based dietary supplement that is used to reduce gas in the digestive tract, thereby improving digestion and reducing bloating, discomfort, and flatulence caused by gas. It contains the enzyme alpha-galactosidase (α-GAL). It was introduced as a liquid, but that has been discontinued and it is now available only as tablets and strawberry-flavored "Meltaways". Beano is marketed and distributed by Prestige Consumer Healthcare, Inc. Mechanism of action Beano contains the enzyme α-Galactosidase, specifically one derived from the fungus ''Aspergillus niger''. The enzyme works in the digestive tract to break down the complex or branching sugars (polysaccharides and oligosaccharides) in foods such as legumes (beans and peanuts) and cruciferous vegetables (cauliflower, broccoli, cabbage, and brussels sprouts, among others). The enzyme breaks those complex sugars into simple sugars, making these foods somewhat more digestible. The polysaccharides and oligosacchari ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Migalastat
Migalastat, sold under the brand name Galafold, is a medication for the treatment of Fabry disease, a rare genetic disorder. It was developed by Amicus Therapeutics. The US Food and Drug Administration (FDA) granted it orphan drug status in 2004, and the European Commission followed in 2006. The European Medicines Agency's Committee for Medicinal Products for Human Use (CHMP) granted the drug a marketing approval under the name Galafold in May 2016. The U.S. Food and Drug Administration (FDA) considers it to be a first-in-class medication. Medical uses Migalastat is used for the long-term treatment of Fabry disease in adults and adolescents aged 16 or older with an amenable mutation of the enzyme alpha-galactosidase A (α-GalA). An "amenable" mutation is one that leads to Protein folding, misfolding of the enzyme, but otherwise would not significantly impair its function. Based on an ''in vitro'' test, Amicus Therapeutics has published a list of 269 amenable and nearly 600 non- ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
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 ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
Enzymes
An enzyme () is a protein that acts as a biological catalyst by accelerating chemical reactions. The molecules upon which enzymes may act are called substrates, and the enzyme converts the substrates into different molecules known as products. Almost all metabolic processes in the cell need enzyme catalysis in order to occur at rates fast enough to sustain life. Metabolic pathways depend upon enzymes to catalyze individual steps. The study of enzymes is called ''enzymology'' and the field of pseudoenzyme analysis recognizes that during evolution, some enzymes have lost the ability to carry out biological catalysis, which is often reflected in their amino acid sequences and unusual 'pseudocatalytic' properties. Enzymes are known to catalyze more than 5,000 biochemical reaction types. Other biocatalysts include catalytic RNA molecules, also called ribozymes. They are sometimes described as a ''type'' of enzyme rather than being ''like'' an enzyme, but even in the d ... [...More Info...] [...Related Items...] OR: [Wikipedia] [Google] [Baidu] |
