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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. All GSID patients lack fully ...
Sucrase-isomaltase is a bifunctional glucosidase (sugar-digesting enzyme) located on the brush border of the small intestine, encoded by the human gene SI. It is a dual-function enzyme with two GH31 domains, one serving as the isomaltase , the other as a sucrose alpha-glucosidase .
Sacrosidase (trade name Sucraid) is a medication used to replace sucrase in people lacking this enzyme. [1] It is available as an oral solution. Sucraid is approved by the U.S. Food and Drug Administration (FDA) for the therapy of the genetically determined sucrase deficiency that is part of the Congenital Sucrase-Isomaltase Deficiency (CSID).
One form, sucrase-isomaltase, is secreted in the small intestine on the brush border. [1] The enzyme invertase, which occurs more commonly in plants, fungi and bacteria, also hydrolyzes sucrose (and other fructosides) but by a different mechanism: it is a fructosidase, whereas sucrase is a glucosidase. [2]
Inborn errors of metabolism are often referred to as congenital metabolic diseases or inherited metabolic disorders. [2] Another term used to describe these disorders is "enzymopathies". This term was created following the study of biodynamic enzymology , a science based on the study of the enzymes and their products.
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HFI is caused by a deficiency of aldolase B. [5] A deficiency of aldolase B results in an accumulation of fructose-1-phosphate, and trapping of phosphate (fructokinase requires adenosine triphosphate (ATP)). The downstream effects of this enzyme block are the inhibition of glucose production and reduced regeneration of ATP. [5]
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