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Biochemistry

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Biochemical Implications in Metabolic Disorders
Kim Wertz
Western Governors University
Biochemistry
208.5.4-01-208.5.5-07
WGU
August 7, 2013

Biochemical Implications in Metabolic Disorders Enzymes play an important role and are involved in the process of fructose breakdown. The enzymes are catalysts, meaning, they work to lower activation energy without using the reaction.
Sucrose is plain table sugar and it is broken down into glucose and fructose that needs to be broken apart. This process known as glycolysis will break down glucose-producing pyruvate and then it goes into the citric acid cycle to produce ATP. Fructose will break down first, forming fructose 1-phosphate by the enzyme, fructokinase. Aldolase B then will convert the fructose 1-phosphate into DHAP and glyceraldehyde as they enter into the glycolysis pathway. When there is a deficit or a mutation of Aldolase B gene, then hereditary fructose intolerance will occur. Individuals are usually asymptomatic until they ingest sucrose, fructose or sorbitol. When the fructose is ingested, there is a block of the Aldolase B, which will cause an increase of the F1P. This is an autosomal recessive condition from mutation of the Aldolase gene. As of 1991, there have been eight structural defects found in the Aldolase B gene, as documented by Wikipedia. Mutant alleles result in many different mutations; one being base pair substitutes, small deletions, and areas of the splicing regions of the Aldolase B gene. The two by products of F1P can then metabolize and produce glucose, uric acid, lactate and glycogen. Without this process, the body is unable to break down fructose leading to the Cori Cycle. When the body is missing Aldolase B, it will be unable to change energy storage of glycogen into glucose. The body will experience a fall in sugar levels and a

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