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Alport syndrome (AS) is an inherited disorder that affects kidney glomeruli of both men and women - AQA - A-Level Biology - Question 8 - 2020 - Paper 1
Question 8
Alport syndrome (AS) is an inherited disorder that affects kidney glomeruli of both men and women. Affected individuals have proteinuria (high quantities of protein ... show full transcript
Worked Solution & Example Answer:Alport syndrome (AS) is an inherited disorder that affects kidney glomeruli of both men and women - AQA - A-Level Biology - Question 8 - 2020 - Paper 1
Step 1
Suggest how AS could cause proteinuria.
Answer
Alport syndrome affects the integrity of the kidney glomeruli, where the filtration of blood occurs. This impairment can lead to the malfunctioning of the glomerular filtration barrier. As a result, larger proteins that should normally be retained in the bloodstream may pass into the urine, leading to proteinuria. Specifically, the disorder could damage or reduce the presence of protein channels or carriers in the basement membrane, which are essential for maintaining selective permeability.
Step 2
Step 3
Evaluate the use of stem cells to treat AS in humans.
Answer
The use of stem cells for treating Alport syndrome presents a promising approach. Stem cells have the potential to differentiate into various cell types, including those found in the kidney glomeruli. If successfully transplanted, these stem cells could replace the damaged cells, thereby restoring normal kidney function and reducing protein levels in urine.
However, there are several factors to consider. It is crucial to assess whether the stem cells can effectively integrate into the existing renal architecture and function properly. Additionally, the immune response to the transplanted cells must be monitored, as rejection could impede treatment success. The evidence from mouse studies indicates that the quantity of protein in the urine reduced significantly, suggesting a potential benefit. Nevertheless, it is important to note that results in animal models do not always translate to humans.
Long-term effects and the overall efficacy of this treatment would require further investigation, including controlled clinical trials.
Step 4
Suggest how the transplanted stem cells reduce proteinuria.
Answer
The transplanted stem cells could reduce proteinuria by differentiating into glomerular cells that replace the damaged ones in the kidney. This replenishment can restore the glomerular filtration barrier's integrity, allowing it to effectively retain proteins that are typically too large to pass into the urine. Moreover, these stem cells may also secrete factors that promote repair and regeneration of the renal tissue, which further contributes to lower protein levels in the urine.