Hey Biochemians! During this course Mr. Matthew gave us some questions to be handed up for marks where we had to look up about real life topics that are related to our course. I have attached some of the questions and my answers because I think after learning all of this we should have some idea as to why we are learning this and what is the link between this course and our very own lives. Have a good read and I urge you to do your own research on these topics. They are really cool!!

1.Describe the pathophysiology of the following diseases:

(i) Tay-Sachs disease

(ii) Leigh’s disease

(iii) Pompe’s disease

Ria’s Answer

A) The pathophysiological mechanism responsible for the development of Tay Sach’s disease is the insufficient activity of the enzyme Hexosaminidase A. This disease is said to be a lysosomal storage disease and is necessary in the breakdown of phospholipids. Hexosaminidase A is important in the breakdown of fatty acid derivatives which are known as gangliosides. As a result of the defect in the functionality of the enzyme there is a buildup of GM2-ganglioside in the lysosomes of neuronal tissue as well as a buildup of lipids in the brain and interference to other neurological and biological processes.

B) The pathophysiology of Leigh’s disease involves the failure of the oxidative mechanism which takes place in the mitochondria of a developing brain resulting in a range of molecular abnormalities. The mitochondrion is important in the conversion of energy in glucose to fatty acids by use of ATP which is described as the energy driving force of a cell. Mutations occurring in the mitochondria results in a lack of energy thus affecting two major areas: central nervous system and motor function. Mutations or deficiencies in pyruvate dehydrogenase can both be responsible for this disease.

C) The fundamental pathophysiological defect in Pompe’s disease is the abnormality in the storage of glycogen. This metabolic disorder is characterized by the acid alpha-glycosidase deficiency which leads to lysosomal glycogen storage. As a result of the defect in the glycogen storage, mobility and respiratory problems occur. This disease begins from the accumulation of the lysosomal glycogen and as the acidity activity decreases below the critical widespread distribution to other tissues and cells can occur.


2. The sweetness of honey gradually decreases at a high temperature. Also, high fructose corn syrup (a commercial product in which much of the glucose in corn syrup is converted to fructose) is used for sweetening cold but not hot drinks. Account for these observations?

Ria’s Answer

High fructose corn syrup is used in the sweetening of cold drinks in oppose to hot drinks because at higher temperatures the equilibrium will shift from the pyranose form to the furanose form. The pyranose form is much sweeter than the furanose form hence at higher temperatures, less sweet solution as the fructose is in the furanose form.


3. Aquorea victoria employs quite an interesting defense mechanism that uses a green fluorescent protein (GFP) together with another protein aequorin. Briefly explain this mechanism.

Ria’s Answer

Aequorin and GFP are both used by Aquorea Victoria as a defense mechanism to frighten predators. When in the presence of oxygen, active aequorin is bounded to coelenterazin which is then converted to coelenteramide as a result of the binding of Ca 2+ aequorin. Now a blue light (469nm) is emitted due to the activated coelenteramide returning to ground state. The green fluorescent protein is activated by the blue light emitter and now green light is emitted (508 nm).


4. Why are the muscles of diving mammals such as the whale, seal, and porpoise brown?

Ria’s Answer

Muscles of diving mammals are brown as they are rich in a protein known is Myoglobin. Myoglobin aids in the storage of oxygen and facilitates oxidative phosphorylation in these mammals. This over richness in Myoglobin allows them to remain underwater for long periods of time.


5.The food product gelatin is derived from collagen; it has little nutritional value as a protein. Why?

Ria’s Answer

Gelatin which is described as processed collagen as consists of repeating sequences of glycine-proline-hydroxyproline throughout its structure.  Gelatin is described as an empty protein as it lacks the essential amino acid tryptophan which is one of 10 essential amino acids vital to the body. Tryptophan is useful in preventing niacin deficiency and raising serotonin levels.

Please note that these answers were sourced from various resources.




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