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LET'S TALK LIFE-SCIENCE BIOCHEMISTRY

Suraj Prakash Sharma | Ekta Chotia

GLUCONEOGENESIS
67

10.12.    Fructose intolerance

Fructose intolerance is due to mutation in aldolase B gene. In this condition, fructose is still phosphorylated by fructokinase. The product fructose-1-phosphate, is not processed further, and therefore the phosphate tied up in it cant be reused. Since phosphate is required for the regeneration of ATP from ADP, this means that ATP will be lacking too, which will sooner or later damage or even destroy the cell. The disease is characterized by potentially severe liver failure.

Fructosuria is increased in fructose concentration in Urine. This is due to  defect in the gene encoding fructokinase. In this disease fructose levels are increased both in the blood and the urine. Since fructose is not phosphorylated, no phosphate depletion occurs, and the liver cells do not occur any damage. The disease is therefore quite benign.

10.13.    The Leloir pathway for galactose utilization

This is a galactose utilization cycle mainly occur in liver.

Step:

  1. Galactokinase transfer the phosphate group to galactose and convert galactose into galactose-1-phosphate.
  2. Galactose-1-phosphate uridyltransferase catalyses the exchange reaction in which the galactose-1-phosphate undergoes an exchange reaction with UDP-glucose and releases glucose-1-phosphate and UDP-galactose.

10.14.    Lactose intolerance

It is due to deficiency of lactase enzyme in the small intestine. Lactose is cleaved by lactase enzyme in small intestine. If lactose is not cleaved, it cannot be absorbed by brush border epithelial of small instestine. It enters into large intestine where lactose is metabolised by intestinal bacteria like E.coli. Escherichia coli has a pathway called mixed acid fermentation which produce acids( formic acid) and gas. Formic acid lyases converts formic acid into H2 and CO2. Treatment consists in emission of lactose in the diet.

10.15.    Galactosemia means “galactose in the blood”

                Galactosemia is due to defect in the following enzymes

10.16.    Sorbitol

Sorbitol is not a sugar, since it lacks a keto or aldehyde group.

It is normally a minor component of dietary carbohydrates. Sorbitol  is also formed by metabolism from glucose in the polyol pathway. Sorbitol converted into fructose. NADPH is used in the first step and NAD+ is  used in the second.

Aldose reductase can reduce galactose into galactitol. Elevated level of galactose in the blood causes galactitol to accumulate in the lens and causes cataract. Conversion of glucose to fructose via the polyol pathway occurs in the seminal Vesicles in male. Fructose is found in the sperm fluid. Sperm cells require fructose to sustain their motility

10.17.    Mannose

Mannose is the C2 epimer of glucose. Mannose is a substrate for hexokinase which converts it into Mannose 6-phosphate. An enzyme similar to phosphoglucose isomerase, phosphomannose isomerase isomerizes mannose 6-phosphate into fructose-6-phosphate. Fructose 6-phosphate is the substrate for PFK-1.

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