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Disaccharidases are glycoside hydrolases, enzymes that break down certain types of sugars called disaccharides into simpler sugars called monosaccharides.In the human body, disaccharidases are made mostly in an area of the small intestine's wall called the brush border, making them members of the group of "brush border enzymes".
Excessive flatus and abdominal bloating may reflect excessive gas production due to fermentation of unabsorbed carbohydrate, especially among patients with a primary or secondary disaccharidase deficiency, such as lactose intolerance or sucrose intolerance. Malabsorption of dietary nutrients and excessive fluid secretion by inflamed small ...
A deficiency is responsible for sucrose intolerance.Congenital sucrase-isomaltase deficiency (CSID), also called genetic sucrase-isomaltase deficiency (GSID), and sucrose intolerance, is a genetic, intestinal disorder that is caused by a reduction or absence of sucrase and isomaltase [13] Explanations for GSID include:
With sucrose intolerance, the result of consuming sucrose is excess gas production and often diarrhea and malabsorption. Lactose intolerance is a similar condition that reflects an individual's inability to hydrolyze the disaccharide lactose. Sucrase is secreted by the tips of the villi of the epithelium in the small intestine.
The two organs most commonly affected are the liver and the skeletal muscle. Glycogen storage diseases that affect the liver typically cause hepatomegaly and hypoglycemia; those that affect skeletal muscle cause exercise intolerance, progressive weakness and cramping. [1] Glucose-6-phosphate isomerase deficiency affects step 2 of glycolysis.
Sucrose intolerance can also be caused by irritable bowel syndrome, aging, or small intestine disease (secondary sucrose intolerance). There are specific tests used to help determine if a person has sucrose intolerance. The most accurate test is the enzyme activity determination, which is done by biopsying the small intestine.
[10] [11] [12] It is one of the more serious glycogen storage diseases affecting muscle tissue. [13] AMD is categorized into three separate types based on the age of onset of symptoms in the affected individual. Infantile (Type a), childhood (Type b), and adulthood (Type c). The type of AMD is determined by the type of gene mutation localized ...
β-Fructofuranosidase is an enzyme that catalyzes the hydrolysis (breakdown) of the table sugar sucrose into fructose and glucose. [1] [2] Alternative names for β-fructofuranosidase EC 3.2.1.26 include invertase, saccharase, glucosucrase, β-fructosidase, invertin, fructosylinvertase, alkaline invertase, and acid invertase.