A number of mouse models for human cystic fibrosis (CF) exist. Each of these mou

Question

A number of mouse models for human cystic fibrosis (CF) exist. Each of these mouse strains is transgenic and bears a different specific CFTR gene mutation. The mutations are the same as those seen in the varieties of human CF. These transgenic CF mice are being used to study the range of different phenotypes that characterize CF in humans. They are also used as models to test potential CF drugs. Unfortunately, most transgenic mouse CF strains do not show one of the most characteristic symptoms of human CF, that of lung congestion.

Can you think of a reason why mouse CF strains do not display this symptom of human CF?

Select the two correct answers.

1. Mice tend not to suffer from airway bacterial infections that severely influence human pathology.

2. These transgenic CF mice havemutated CFTR and the results cannot be used for characterizing human symptoms.

3. The distribution of cell types, CFTR presence, and chloride/sodium handling differ in the upper and lower airways of mice and humans.

4. The human genome has too many differences compared to the mouse genome and their regulations are based on different principles.

Explanation / Answer

3. The distribution of cell types, CFTR presence, and chloride/sodium handling differ in the upper and lower airways of mice and humans.

4. The human genome has too many differences compared to the mouse genome and their regulations are based on different principles.

Research studies have shown that there are large number of genes having expression levels that are different in mouse and humans. The findings are reported in the mouse ENCODE (Encylopaedia of DNA elements).

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