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Comparative Proteomic Analysis of Whole Kidney, Medulla, and Cortical Tubules in Diabetic Pathogenesis of Kidney Injury in Mice
Published on: May 2, 2025
[Kidney and hemoglobinopathy]
Philippe Rémy1, Vincent Audard1, Frédéric Galactéros1
1Service de néphrologie-dialyse-transplantation, hôpital Henri-Mondor, 51, avenue du Maréchal-de-Lattre-de-Tassigny, 94010 Créteil cedex, France.
Insights
Sickle-cell disease (SCD) significantly increases the risk of chronic kidney disease due to hemoglobin S polymerization. This review explores the causes and varied kidney problems in SCD patients.
Area of Science:
- Nephrology
- Hematology
- Genetics
Background:
- Sickle-cell disease (SCD) is a common, severe monogenic disorder globally.
- It stems from a mutation in the beta-globin gene, producing hemoglobin S (HbS).
- HbS polymerization causes red blood cell rigidity and vaso-occlusive episodes, central to SCD pathogenesis.
Purpose of the Study:
- To review the pathogenesis of sickle cell nephropathy.
- To outline the spectrum of renal manifestations in SCD patients.
Main Methods:
- Literature review focusing on SCD and renal involvement.
- Analysis of pathophysiological mechanisms underlying sickle cell nephropathy.
Main Results:
- SCD is linked to a high frequency of chronic kidney disease.
- Renal manifestations in SCD are diverse and age-dependent.
- Endothelial dysfunction from chronic hemolysis is a key factor in kidney damage.
Conclusions:
- Understanding SCD pathogenesis is crucial for managing kidney complications.
- The broad spectrum of renal diseases in SCD requires comprehensive clinical attention.
Abstract:
Sickle-cell disease (SCD), one of the most common severe monogenic disorders into the world, is associated with an increased frequency of chronic kidney disease. SCD is caused by a point mutation in the gene encoding β globin gene which leads to the formation of hemoglobin S that polymerises after deoxygenation. HbS polymerisation is associated with erythrocyte rigidity and vaso-occlusive episodes that play a central role into SCD pathogenesis. The spectrum of renal diseases during SCD is broad and includes various renal manifestations which become more apparent with increasing age. Underlying pathophysiological processes involved in sickle cell nephropathy are multifactorial but endothelial dysfunction related to chronic hemolysis is a key factor contributing to renal involvement. Our review focuses on the pathogenesis and on the spectrum of renal manifestations occurring in SCD patients.
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