Explain the molecular basis for cystic fibrosis you learned in lecture for chapt

Question

Explain the molecular basis for cystic fibrosis you learned in lecture for chapter 15. Name two proteins found in the tight junctions Actin-binding proteins bind to actin and can modify its properties. You purify a protein, Cap1, that seems to bind and cap one end of an actin filament, although you do not know whetherit binds the plus end or the minus end. To determine which end of the actin filament your protein binds to, you decide to examine the effe fCapil on actin polymerization by measuring the kinetics of actin filament formation in the presence and the sence of Cap protein. You obtain the following results (see figure below). Do you think Capl binds the pl dor the minus end of actin? Explain your reasoning. KINETICS OF ACTINASSEMBLY j 10000 actin alone 7* 000 5 E actin Cap 2500 protein o o so s o OOO time (seconds

Explanation / Answer

D-1. Molecular basis of Cystic fibrosis:

Cystic fibrosis is a common dangerous autosomal recessive disorder occurred due to a defective gene which produces a deformed form of   cystic fibrosis transmembrane conductance regulator (CFTR) protein. The most common mutation is the deletion of a phenylalanine amino acid at position 508, due to deletion of three corresponding nucleotides. This dleltion occurred in the NBD 1 domain of CFTR proein. Mutant poteina re destroyed quickly by body thus pelple with Cystic fibrosis suffered a lack of this protein. CFTR is a chloride ion channel receptor which is highl;y important in creating sweat, digestive juice, mucus etc. CFTR has two domains each of which linked with a regulatory domain. Due to the deletion CFTR became inactive thus the secretion occurs through CFTR protein became thick.

D-2. Claudin and Occuldin are two proteins present in tight junctions. Tight junction is actually a network of claudin. These proteins allow passage of molecules and ions through the intercellular space. They also restrict the movement of integral membrane proteins between the basolateral and apical sides of cell. Thus helps to maintain the asymmetry between apical and basal sides.

D-3. Please clearly mention the lower experimental data part. It’s not understandable.

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