J. Mol. ; Su, V.C.H. 1) Chloride ions are pumped into the epithelial cell causing a concentration gradient and the CFTR channel protein opens 2) Chloride ions diffuse into the mucus through the CFTR protein 3) This closes the ENaC 4) The build up of chloride ions creates an electrical gradient so sodium ions diffuse into the mucus between the gaps in epithelial cells https://doi.org/10.3390/ijms24054635, Recchia D, Stelitano G, Stamilla A, Gutierrez DL, Degiacomi G, Chiarelli LR, Pasca MR. Mycobacterium abscessus Infections in Cystic Fibrosis Individuals: A Review on Therapeutic Options. Cystic Fibrosis Transmembrane Concordance Regulator (gated channel protein). * The basic structure of an amino acid 2.15 (i): Know the meaning of the terms: gene, allele, genotype, phenotype, recessive, dominant, codominance, homozygote and heterozygote * Simple and facilitated diffusion as methods of passive transport Cystic fibrosis. movement of particles from an area of high concentration to an area of lower concentration Fick's law the rate of diffusion is inversely proportional to the thickness of the gas exchange surface surfactant a complex mixture of phospholipids and proteins that reduces surface tension at the air-liquid interface of the alveolus dipeptide ; et al. This is achieved through the wide variety of tasks in the PowerPoints and accompanying worksheets which include exam-style questions with clear answers, discussion points, differentiated tasks and quick quiz competitions. The 20 lesson PowerPoints and accompanying resources contain a wide range of activities which cover the following topic 2 specification points: In CF, the inherited CF gene directs the body's epithelial cells to produce a defective form of a protein called CFTR. ; Waldbrook, M.; Mullaly, S.C.; Volkmer, R.; Hancock, R.E. * Understand the uses and implications of genetic screening and prenatal testing IBO was not involved in the production of, and does not endorse, the resources created by Save My Exams. The mucus becomes thick, sticky, and hard to move. CF therapy has been considerably improved in recent years. ; Otto, M. Different drugs for bad bugs: Antivirulence strategies in the age of antibiotic resistance. If you have cystic fibrosis, this mucus is excessively thick and sticky and cannot perform this role properly. * The primary, secondary, tertiary and quaternary structure of proteins Fast expert advice. Kortright, K.E. 2.6 (ii): Understand the roles of the DNA template (antisense) strand in transcription, codons on messenger RNA and anticodons on transfer RNA ; Colmant, A.; Rodriguez-Villalobos, H. Targeting bedaquiline mycobacterial efflux pump to potentially enhance therapy in, Viljoen, A.; Raynaud, C.; Johansen, M.D. [6]. * The process of DNA replication - can lead to designer babies Feature papers represent the most advanced research with significant potential for high impact in the field. Cystic fibrosis is a genetic disorder of cell membranes caused by a recessive allele of the CFTR ( C ystic F ibrosis T ransmembrane Conductance R egulator) gene located on chromosome 7 This gene codes for the production of chloride ion channels required for secretion of sweat, mucus and digestive juices Living on the edge: Inhibition of host cell apoptosis by. How do family and peers affect decisions about clothing? Antivirulence compounds: A future direction to overcome antibiotic resistance? It affects about 1 person in 2000. * The involvement of ATP and carrier proteins in active transport, endocytosis and exocytosis Medical applications and toxicities of gallium compounds. Thakur, A.; Mikkelsen, H.; Jungersen, G. Intracellular pathogens: Host immunity and microbial persistence strategies. I am constantly thinking of new ways to engage a student with a topic and try to implement that in the design of the lessons. One mutation causes a change in the primary structure of CFTR, a membrane transport protein. ; Gaillard, J.L. 2.15 (i): Understand the uses of genetic screening, including the identification of carriers, pre-implantation genetic diagnosis (PGD) and prenatal testing, including amniocentesis and chorionic villus sampling Cystic Fibrosis Mutation Database. No problem. ; Shukla, S.; Chung, Y.H. Millar, B.C. ; Coler, R.N. ; Bindokas, V.; et al. 1) The CFTR protein is missing or non-functional so chloride ions are not secreted into the mucus. It is a recessive genetic trait. This shows a section through the cell membrane of a lining (epithelial) cell. Physics Aqa Empa 2018 Paper corgov de. CFTR regulates phagosome acidification in macrophages and alters bactericidal activity. 2.15 (iii): Understand sex linkage on the X chromosome, including red-green colour blindness in humans A full range of published resources is available to support the delivery of our Biology qualifications. Sci. 2. Chan, B.K. 2.11: Understand the nature of the genetic code Cystic fibrosis (CF) is the number-one life-shortening inherited disease of young people. basement membrane These authors equally contributed to the work. Non-disulfide-Bridge Peptide 5.5 from the Scorpion, das Neves, R.C. For more information, please refer to ; Li, C.; Szeto, F.L. at 7-9 weeks Membranes, proteins, DNA and gene expression represent some of the most important structures, molecules and processes involved in this subject and a deep understanding of their role in living organisms is important for a student's success. Your rating is required to reflect your happiness. Ribet, D.; Cossart, P. How bacterial pathogens colonize their hosts and invade deeper tissues. 2.15 i) Understand the uses of genetic screening, including the identification of . * The nature of the genetic code Locate the section on student dress in your school handbook. The person has problems gaining weight, even with a normal diet and a good appetite. ; Coutinho, P.; Mendonca, A.G.; Correia, I.J. Phage Therapy: A Renewed Approach to Combat Antibiotic-Resistant Bacteria. It affects organs in the body, particularly the lungs and digestive system, which become clogged with sticky mucus, making it difficult to breathe and digest food. Paper should be a substantial original Article that involves several techniques or approaches, provides an outlook for As a result, delta F508 never reaches the cell membrane. * The role of channel and carrier proteins in membrane transport 1-2% miscarriage risk M. abscessus is naturally resistant to many antibiotics such as rifamycin, tetracyclines and β-lactams. ; Goncalves, J.C.; Trentini, M.M. in 2013 reported an MIC of 25 g/mL against, More specific research analyses 22 pure compounds extracted from different parts of 4 medicinal plants (, All the reported studies indicate that plants and fungi are still nowadays a source with a huge potential for discovering new molecules that are effective in fighting, In conclusion, this review provides an overview of the latest findings on innovative therapies against, For some of the strategies described, positive results have been obtained at the early pre-clinical stages, and it is hoped that these will form the basis for developing these strategies and confirming their efficacy in vivo so that they can reach the clinical trial stage. Miller, C.; McMullin, B.; Ghaffari, A.; Stenzler, A.; Pick, N.; Roscoe, D.; Ghahary, A.; Road, J.; Av-Gay, Y. Gaseous nitric oxide bactericidal activity retained during intermittent high-dose short duration exposure. Unbeatable support throughout the qualification. Visit our dedicated information section to learn more about MDPI. ; Huang, K.H. Cherkasov, A.; Hilpert, K.; Jenssen, H.; Fjell, C.D. 3.12: Understand how cells become specialised through differential gene expression, producing active mRNA leading to synthesis of proteins, which in turn control cell processes or determine cell structure in animals and plants, including the lac operon ; Essi, L.; de Campos, M.M.A. - cells from the chorionic villi collect through the abdomen with a needle or through the vagina with a catheter, Non-Invasive Prenatal Diagnosis The following specification points are covered by the lessons within this bundle: ; Anggard, E.E. ; Turner, P.E. ; das Neves, R.C. Hours and hours of planning have gone into each and every lesson that's included in this bundle to ensure that the students are engaged and motivated whilst the detailed content of topic 2 of the Edexcel International A-level Biology specification is covered. Class Effect on CFTR protein IV has a faulty opening to the pore VI synthesised but breaks down quickly 3.14 (i): Phenotype is an interaction between genotype and the environment Smaller fleas ad infinitum: Therapeutic bacteriophage redux. Something went wrong, please try again later. 1996-2023 MDPI (Basel, Switzerland) unless otherwise stated. ; Herrmann, J.L. Use of bacteriophages in the treatment of colistin-only-sensitive, Schooley, R.T.; Biswas, B.; Gill, J.J.; Hernandez-Morales, A.; Lancaster, J.; Lessor, L.; Barr, J.J.; Reed, S.L. ; Aksamit, T.; Carvalho, A.C.C. ; Brown-Elliott, B.A. Brogden, K.A. Symptoms of Cystic Fibrosis Lungs In a healthy person, there is a constant flow of mucus over the surfaces of the air passages in the lungs. 2.8: Know that a gene is a sequence of bases on a DNA molecule that codes for a sequence of amino acids in a polypeptide chain Yaacoby-Bianu, K.; Gur, M.; Toukan, Y.; Nir, V.; Hakim, F.; Geffen, Y.; Bentur, L. Compassionate Nitric Oxide adjuvant treatment of persistent. ; Nick, J.A. 1.1.2 The Importance of Water in Transport, 1.1.3 Mammalian Heart: Structure & Function, 1.1.4 Blood Vessels: Structure & Function, 2.1 Gas Exchange, Cell Membranes & Transport, 2.1.1 Properties of Gas Exchange Surfaces, 2.1.5 Practical: Investigating Membrane Structure & Permeability, 2.3.6 Globular Proteins: Structure & Function, 2.3.7 Fibrous Proteins: Structure & Function, 2.3.10 Practical: Investigating the Effect Enzyme and Substrate Concentrations, 3.2.3 Practical: Identifying Mitosis in Plant Cells, 3.3.4 Meiosis: Source of Genetic Variation, 4.1.2 Measuring Biodiversity Within a Habitat, 4.1.3 Comparing Biodiversity Between Habitats, 4.2.3 Importance of Water & Inorganic Ions to Plants, 4.2.4 Starch & Cellulose: Structure & Function, 4.2.6 Practical: Identifying Tissue Types Within Stems, 4.2.7 Practical: Determining the Tensile Strength of Plant Fibres, 5.1.4 Practical: Determining Distribution & Abundance, 5.1.7 Calculating the Efficiency of Biomass & Energy Transfers, 5.3.2 Evidence for the Causes of Climate Change, 5.3.7 Practical: Temperature & Development of Organisms, 5.3.8 Climate Change & the Scientific Community, 5.3.9 Carbon Cycle & Reduction of Atmospheric Carbon Dioxide, 5.4.3 Evidence of Evolution & the Scientific Community, 6.1.2 Micro-organisms: Decomposition & Nutrient Recycling, 6.2.9 Evolutionary Race Between Pathogens & Host, 7.2.6 Practical: Investigating the Rate of Respiration, 7.4.1 Variations in Breathing Rate & Heart Rate, 7.4.2 Practical: Investigating the Effects of Exercise, 7.4.4 Modern Medicine & Participation in Sports, 8.1.3 Nervous System: Response to a Stimulus, 8.2.4 The Role of Animal Models in Research, 8.2.6 Imbalances in Brain Chemicals Leading to Diseases, 8.2.10 Nature vs Nurture in Brain Development. Immunocompromised individuals and cystic fibrosis patients are especially vulnerable to serious infections by these bacteria, motivating the search for compounds with antimicrobial activity against the Bcc. ; Aksamit, T.; Brown-Elliott, B.A. * The meaning of key genetic terms * The basic structure of mononucleotides Again, the students are guided through the events that lead to the depletion of the apical surface liquid and the cilia being unable to move the viscous mucus. 2.7: Understand the nature of the genetic code Chalmers, J.D. 2.14: Understand how the expression of a gene mutation in people with cystic fibrosis impairs the functioning of the gaseous exchange, digestive and reproductive systems 6.4: Know how DNA can be amplified using the polymerase chain reaction (PCR) These proteins transport molecules such as sugars, peptides, inorganic phosphate, chloride, and metal cations across the cellular membrane. squamous (alveoli and capillaries) and columnar (small intestine), appearance of multiple layers due to nuclei when there is only one, apical membrane - cilia and mucus https://www.mdpi.com/openaccess. interesting to readers, or important in the respective research area. If you want to see what we offer before purchasing, we have a free membership with sample revision materials. 30 terms. * A gene is a sequence of bases on DNA that codes for the amino acid sequence of a polypeptide Combined Science Biology Chemistry Physics AQA. US Cystic Fibrosis Foundation and European Cystic Fibrosis Society. Gibson, L.E. ; Cheung, G.Y.C. Real-world safety of CFTR modulators in the treatment of cystic fibrosis: A systematic review. ; Ogbonna, D.; Ferro, B.E. * Sex linkage on the X chromosome Our customer service team will review your report and will be in touch. If you would like to sample the quality of these lessons, then download the rapid gas exchange, osmosis, DNA & RNA, genetic code, genetic terms and cystic fibrosis lessons as these have been uploaded for free. A Science teacher by trade, I've also been known to be found teaching Maths and PE! Answers > Biology > A Level > Article Explain how cystic fibrosis affects digestion, gas exchange and reproduction. - Genetic discrimination from employers and life insurance companies, Preimplantation genetic diagnosis In fact, alongside symptom therapy, personalized therapies are now starting to treat the basic defect in some CFTR mutations [, Thanks to scientific research, new therapeutic approaches have indeed emerged that can correct the defects underlying CF. ; Ortega-Rivera, O.A. For all these reasons, the identification of new therapeutic strategies that can support or refine the scarce antibiotic options available today is an imperative issue to be addressed. However, strange as it may seem, my real love is designing resources that can be used by other teachers to maximise the experience of the students. Bacteriophage therapy for infections in CF. -Stressful and expensive Naomi graduated from the University of Oxford with a degree in Biological Sciences. Although the majority of the lesson is described with reference to the gaseous exchange system, the impaired functioning of the digestive system in terms of the blockage of the pancreas and liver secretions is considered and discussed and the students are challenged on their understanding through a range of exam-style questions. I am constantly thinking of new ways to engage a student with a topic and try to implement that in the design of the lessons. The mucus becomes thick, sticky, and hard to move. What are the treatment for cystic fibrosis? CFTR transports chloride ions (Cl-) ions across the membranes of cells in the lungs, liver, pancreas, digestive tract, reproductive tract, and skin. ; Young, L.; Baldwin, S.L. ; Iaccarino, J.M. ; Huang, T.T. ; Riabova, O.; Makarov, V.; Pasca, M.R. Use of artificial intelligence in the design of small peptide antibiotics effective against a broad spectrum of highly antibiotic-resistant superbugs. Thanks very much for sharing this It is a great help. They have been successfully used to treat a plethora of diseases, from cancer to infections and as putative wound dressing [, Recently, those tools have been also investigated as putative weapons against, One example is gallium-based nanoparticles, which showed significant results in blocking, The efficacy of liposome encapsulated rifampicin compared to free rifampicin, in an ex vivo, Nanoniosomes are vesicles with a composition similar to that of the cell membrane (named niosomes) that have been loaded with antibiotics or lignin-silver nanoparticles. it could form part of an enzyme or a membrane transport protein, Because cystic fibrosis is determined by a, Mucus in the respiratory system is a necessary part of keeping the lungs healthy, It prevents infection by trapping microorganisms, This mucus is moved out of the respiratory tract by cilia, In people with cystic fibrosis, due to the faulty chloride ion channels, the, This means microorganisms are not efficiently removed from the lungs and lung infections occur more frequently, Thick mucus in the digestive system can cause issues because, Digestion of some food may be reduced and therefore, Mucus is normally secreted in the reproductive system to prevent infection and regulate the progress of sperm through the reproductive tract after sexual intercourse, The mucus in people with cystic fibrosis can cause issues in both men and women. ; Waldbrook, M. ; Mullaly, S.C. ; Volkmer, R. ; Hancock, R.E regulates! 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