Sucrase isomaltase deficiency is a disorder that affects normal carbohydrate digestion and therefore absorption, and leads to sucrose intolerance. Intolerance is described as clinical symptoms that are caused by sugar malabsorption (Scriver et al, 2001), in the case of sucrose intolerance this refers to the inability to digest and absorb sucrose, leading to several abdominal symptoms. Although sucrase isomaltase deficiency is not a fatal condition, it can cause suffers discomfort and therefore changes to the diet need to be made in order to avoid the symptoms caused by the deficiency. Carbohydrate accounts for about half of the energy humans get from food. Carbohydrate digestion and absorption takes across the brush-border member which has an apical and basolateral membrane. Anchored to the brush-border membrane are enzymes that are involved in digesting disaccharides (Silverthorn, 2010). Carbohydrates can only be absorbed as monosaccharaides meaning polysaccharide and disaccharide carbohydrates must first be hydrolysed into monosaccharaides. Carbohydrate digestion begins in the saliva and stomach where alpha-amylase hydrolyses alpha-1, 4 glycosidic bonds between glucose molecules in starch, forming maltotriose, the disaccharide maltose and dextrin’s made of five to ten glucose molecules (Lim, 2007). The disaccharides sucrose and lactose come directly from food. There are four enzymes found on the brush-border membrane responsible for hydrolysing sucrose, lactose and the products of starch break down, into monosaccharaides so that they can be absorbed (Lieberman et al, 2007). These enzymes are known as glycosidases and include; glucoamylase, lactase, trehalase and sucrase isomaltase (Lieberman et al, 2007). Sucrase isomaltase... ... middle of paper ... ... Biol Chem, Volume 281. Lieberman M, Marks A, Smith C. (2007). Marks’ essentials of medical biochemistry a clinical approach. Philadelphia: Lippincott Williams & Wilkins. Pp 316-317. Ouwendijk J, Moolenaar C, Peters WJ, Hollenberg CP, Ginsel LA, Fransen J, et al. (1996). Congenital sucrase-isomaltase deficiency identification of a glutamine to proline substitution that leads to a transport block of sucrase-isomaltase in the pre-golgi compartment. J Clin Invest, Volume 97. Lim M, Roach J. (2007). Metabolism and Nutrition. 3rd ed. Edinburgh: Mosby. Pp 145 Scriver, Charles R, Beaudet, Arthur L, Sly, William S, et al. (2001). The metabolic and molecular bases of inherited disease. 8th ed. London: McGraw-Hill. Pp 1634-1639. Silverthorn DU. (2010). Human physiology: an integrated approach. 5th ed. San Francisco, calif.; London: Pearson/Benjamin Cummings. Pp 703-704.
b) Comprehensive diagnostic chemistry panel with significantly increased amylase (1626 with normal being 300-1100 U/L), total
Glucose is liberated from dietary carbohydrate such as starch or sucrose by hydrolysis within the
Carbohydrates are split into two main groups. The simple sugars and the starches. Simple sugars contain fructose, glucose and lactose. All three sugars can be found in most fruits. The starches are found in bread, rice, some fruits, some vegetables, and potatoes. Carbohydrates are mainly tasked with providing your body energy. However, in some instances where the body lacks enough fibre, the carbohydrates can be broken down to provide a replacement for fibre.
Sucrose- is a disaccharide formed by linking fructose and glucose. It’s also known as simply sugar.
The enzyme pancreas amylase causes the decomposition of starch. The starch during the chemical reaction broken down into disaccharides, lactase, sucrase, and maltase forms of pure sugar. Disaccharides are broken down to monosaccharides. Lactase changed into lactose, then into glucose and galactose sucrase changed sucrose into glucose and fructose these are all forms of sugars. These sugar may not all be utilized by the body. Maltase breaks down maltose 2 form molecules of glucose. Protein -stomach Pepcid and HCI break down proteins. These protein continue during the chemical reaction change to polypeptides. In the small intestines- Trypsin breaks down proteins and polypeptides to dipeptides. Then the dipeptides are changed into chymotrypsin decomposition of proteins and polypeptides to dipeptides. Carboxypeptidase breaks down polypeptides and dipeptides to amino acids. Aminopeptidase disintegrates of polypeptides & dipeptides to amino acids. Dipeptidase dissects of dipeptides to amino acids. Amino acids are more utilized by the digestive process; they are the building blocks of protein. Fats start the chemical digestive process in the mouth, this maybe because that many fats take longer to decompose. Lingual lipase has a minor role in beginning fat digestion. The stomach has an immense amount of chemical reaction going on at one time.
The food digestion plays an undeniably important role in our body system, which is the main way for the human kind to gain nutrients and energy in order to growth, repairs the body cells, and carry out the daily routine (National Institutes of Health, 2013). The foods and drinks that people consume are required to be turned into the smaller nutrient-molecules before the blood absorbs and carries the various nutrients such as carbohydrates, proteins, fats, vitamins and minerals to the body cells (National Institutes of Health, 2013). According to National Institutes of Health (2013), the decomposition of food nutrients are completed through the digestive system which form by the gastrointestinal (GI) tract, also defined as digestive tract, and along liver, pancreas and gallbladder as well. The GI tract is made up of a series of hollow organs with the connection from mouth to anus, which consisting buccal cavity (mouth), esophagus, stomach, small intestine, and large intestine. Based on Batrisya (2013), the food digestive process are classified into four stages, that are ingestion, digestion, absorption, and egestion or elimination (refer to Figure 1 in Appendix 1).
Hers, H. G. "a- Glucosidase Deficiency in Generalized Glycogen-Storage Disease (Pompe's Disease)." Biochem J (1963): 11-16.
Mouth- Digestion begins in the mouth. Physical actions, such as chewing, breaks food into small parts so it can be easily digested. Next, salivary glands secrete an enzyme called saliva to mix with food to start the breaking down of carbohydrates (WebMD (2).) From the mouth, food travels to the pharynx, or throat, by swallowing,
Enzymes are proteins that help speed up chemical reactions. Some enzymes associated with carbohydrate breakdown are sucrose, lactose, maltase, and amylase. They aid in turning carbohydrate into glucose. The body uses glucose for energy, especially within the cells. Lipids or fats must be emulsified by bile found in the liver, and will be broken down into fatty acids and glycerol, and the body is able to absorb them. The enzyme associated with lipid breakdown is lipase. Protein that is ingested use the enzymes proteases and peptidases to help break up the protein and make amino acids in the
The sugar molecule lactose is found in dairy products and people with Lactose Intolerance are unable to digest lactose because the enzyme lactase is unable to split the sugar molecule into glucose. Lactase is an enzyme that breaks lactose down into galactose and glucose. Lactase functions best between 21 and 48 degrees Celsius (or 70 and 120 degrees Fahrenheit). Cooler temperatures will slow down lactase’s function, whereas high temperatures can denature it or lactase will lose its shape. If lactase is rendered nonfunctional because of temperature or pH extremes, the breakdown of lactose stops. Lactose intolerance occurs when this breakdown fails due to insufficient or ineffective lactase. The experiment will stimulate the process
Fernandes, J. Saudubray, J. van den Berghe, G. Inborn Metabolic Dieases, 4th ed. Germany, Springer Medizin Verlag Heidelberg 2000, 2006; pp 234-240.
Inborn errors of metabolism include phenylketonuria (rise of phenylalanine levels and adverse metabolic products), tyrosinaemia and albinism (relating with tyrosine), alkaptonuria (impair phenylalanine and tyrosine metabolism), homocystiburia (affects methionine metabolism), maple syrup urine disease (effects
...apter 362. Glycogen Storage Diseases and Other Inherited Disorders of Carbohydrate Metabolism. In D.L. Longo, A.S. Fauci, D.L. Kasper, S.L. Hauser, J.L. Jameson, J. Loscalzo (Eds), Harrison's Principles of Internal Medicine, 18e. Retrieved January 21, 2012 from http://www.accessmedicine.com/content.aspx?aID=9144477.
A dehydration synthesis reaction forms a bond between carbon atoms in two monosaccharides, sandwiching an oxygen atom between them and releasing a water molecule.” (https://dlc.dcccd.edu/biology1-3/carbohydrates). Carbohydrates are composed of carbon, hydrogen, and oxygen in a ratio of one carbon atom to two hydrogen atoms to one oxygen atom. Carbohydrates exist as monosaccharides, disaccharides, and polysaccharides. A monosaccharide is a monomer of a carbohydrate. A monosaccharide is a single sugar that contains carbon, hydrogen, and oxygen. When two monosaccharides join, they form a double sugar called a disaccharide. “This image shows disaccharides joined by a glycosidic bond. A glycosidic bond to the anomeric carbon can be either a or B. For example maltose, is the disaccharide gained by enzyme-catalyzed hydrolysis of starch, consists of two D-glucopyranose units joined by a 1,4’-a-glycoside bond. The “prime” indicates that C-4 is not the same ring as C-1. Unlike the other disaccharides, sucrose is not a reducing sugar and does not exhibit mutarotation because the glycosidic
All carbohydrates are made up of units of sugar (also called saccharide units). Carbohydrates that contain only one sugar unit (monosaccharides) or two sugar units (disaccharides) are referred to as simple sugars. Simple sugars are sweet in taste and are broken down quickly in the body to release energy. Two of the most common monosaccharides are glucose and fructose. Glucose is the primary form of sugar stored in the human body for ener...