Questions regarding Cystic Fibrosis. See details?
-What specific part of the cell/structure allows the mucus secretions to be thick and viscous rather than fluid (i.e., is it a membrane channel, organelle or something else)? Please be specific and describe the step-by-step mechanism whereby this altered structure/part of the cell causes the secretions to contain less water.
-Why does Alvin have salt crystals forming on his skin? Explain the mechanism for this.
-What is the accepted treatment for children with cystic fibrosis? Make sure to list at least three and explain why they work (i.e., what is the purpose of each individual treatment).
-List and explain the mechanism of at least two experimental treatments that are currently being tried to help patients with cystic fibrosis.
-Briefly describe how gene therapy may help cure a genetically inherited disease like cystic fibrosis.
link to website with more info (like who alvin is) http://www.sciencecases.org/cf/cf.asp
If you can’t answer all the questions (only some) that’s better than nothing.
Tagged with: Cystic Fibrosis • Organelle • Salt Crystals
Filed under: Cystic Fibrosis
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it may be here, and if not you could add anyway. spread the word. find a cure,
I just found this new information you may want to research this. A lot more on the mucus question you asked.
Summary Many of the symptoms of cystic fibrosis are not explained by the current disease mechanisms. Therefore, the authors conducted an extensive literature review and present a new model of cystic fibrosis pathology, which is the culmination of this research. Understanding that the cystic fibrosis transmembrane conductance regulator (CFTR) is responsible for glutathione (GSH) transport, the authors hypothesize that mutations of the CFTR, which create abnormal GSH transport, will lead to aberrations of GSH levels in both the intracellular as well as the extracellular milieu. These alterations in normal cellular GSH levels affect the redox state of the cell, thereby affecting the intracellular stress protein, metallothionein. The authors describe how this disruption of the redox state caused by excess cellular GSH, will naturally prevent the delivery of zinc as a cofactor for various enzymatic processes, and how these disruptions in normal redox may cause alterations in both humoral and cell-mediated immunity. Moreover, the symptom of thick sticky mucus in these patients might be explained through the understanding that oversulfation of mucus is a direct result of elevated cellular GSH and cysteine. The issues of hyperinflammation, altered pH and the imbalance of fatty acids that are typical in cystic fibrosis are addressed—all of which may also be linked to disruptions in GSH homeostasis. Additionally, this new model of cystic fibrosis pathology, clarifies the relationship between the CFTR and the multi-drug resistance proteins, and the lack of cell-mediated immunity by predicting that the substrate of these proteins is a glutathione adduct of thiocyanate. Finally, a new therapeutic strategy by using isothiocyanates to rectify the GSH imbalance and restore the immune system is suggested for the treatment of cystic fibrosis patients. More on this here
The salt situation on the other hand is a problem with the way the body processes salt. Sodium-chloride (Salt) is divided in part into each respective element (sodium and chloride). The sodium is passed around the cells whereas the chloride is absorbed into cirtain cells where it is to be later excreted and recombined with the sodium. The problem with CF is that the cells that are supposed to be “chloride excreting”, don’t excrete the chloride. Consequently the salt excreted from the mucus membranes, sweat glands and possibly other organs has a sodium and chloride imbalance, (high sodium and low chloride). From my grade school chemistry recollection, sodium is somewhat unstable (reactive) without its counterpart (most commonly, chloride or iodine) to bond to. That is why the sweat from CF patients tastes different, saltier. The gene therapy and exact experimental mechanism questions you are looking for stretch my understanding so i will not attempt to answer those. Try a pubmed search, use the words “hamster cystic fibrosis” in your search. Lots of genetic info there.
Hopefully this helps though?
Best Regards
Edric Zaruba