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A genetic defect in one of these enzymes will cause a disaccharide ... "Radiological diagnosis of disaccharidase deficiency". Lancet. 2 (7455): 139–143. doi:10.1016 ...
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 ...
Sucrose intolerance or genetic sucrase-isomaltase deficiency (GSID) is the condition in which sucrase-isomaltase, an enzyme needed for proper metabolism of sucrose (sugar) and starch (e.g., grains), is not produced or the enzyme produced is either partially functional or non-functional in the small intestine.
Inborn errors of metabolism form a large class of genetic diseases involving congenital disorders of enzyme activities. [1] The majority are due to defects of single genes that code for enzymes that facilitate conversion of various substances into others ().
Breaking apart a double sugar into its two monosaccharides is accomplished by hydrolysis with the help of a type of enzyme called a disaccharidase. As building the larger sugar ejects a water molecule, breaking it down consumes a water molecule. These reactions are vital in metabolism.
Acid maltase deficiency (AMD) also known as Pompe disease was first described by Dutch pathologist JC Pompe in 1932. [ 10 ] [ 11 ] AMD is a non sex linked autosomal recessive condition in which excessive accumulation of glycogen build up within lysosome vacuoles in nearly all types of cells all over the body.
Causes of vitamin B12 deficiency. There are a few reasons why someone might develop a vitamin B12 deficiency. Following a vegan diet for years is a big one, says Deborah Cohen, ...
Sucrose is a non-reducing sugar, so will not test positive with Benedict's solution.To test for sucrose, the sample is treated with sucrase. The sucrose is hydrolysed into glucose and fructose, with glucose being a reducing sugar, which in turn tests positive with Benedict's solution.