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Related Concept Videos

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The kidneys are intricate organs with millions of working units known as nephrons. Each nephron features two major structures: the renal corpuscle, which facilitates blood plasma filtration, and the renal tubule, which handles the glomerular filtrate. Blood supply is directly linked to the nephrons. The renal corpuscle consists of the glomerulus, a capillary network, and the Bowman's capsule, a double-walled epithelial structure that encases the glomerulus. The filtering of blood plasma...
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The renal tubule is divided into three parts: the proximal convoluted tubule (PCT), the Loop of Henle (LOH), and the distal convoluted tubule (DCT).
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The PCT is the initial segment of the renal tubule, extending from the Bowman's capsule that encloses the glomerulus. Its convoluted structure and microvilli-lined cells increase the surface area for reabsorption. The PCT reabsorbs glucose, amino acids, sodium, and water from the filtrate, ensuring essential...
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The glomerulus and Bowman's capsule are two essential components of the nephron, which is the functional unit of the kidney. These microscopic structures play a critical role in the process of blood filtration to produce urine.
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Smooth Endoplasmic Reticulum01:21

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Smooth endoplasmic reticulum or smooth ER is a sub-organelle with specialized functions in animal cells and plant cells. It is often associated with the tubule morphology of the endoplasmic reticulum.
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Microtubules in Signaling

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The primary cilium, made up of microtubules, acts as antennae on the cell surfaces for relaying external stimuli into the cells. These fine hair-like structures are present, generally one per cell. These are non-motile cilia in a 9+0 microtubules arrangement, where the central pair of microtubules are absent. The primary cilia arise from the basal body embedded in the cell membrane. Intraflagellar transport (IFT) carries requisite proteins from the cytoplasm to the cilium because the primary...
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Translation

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Lesson: Translation
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Updated: May 10, 2025

Evaluation of Zebrafish Kidney Function Using a Fluorescent Clearance Assay
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Renal ciliopathies.

Laura A Devlin1, Rebecca M Dewhurst1, Praveen D Sudhindar1

  • 1Translational and Clinical Research Institute, Faculty of Medical Sciences, Newcastle University, Newcastle upon Tyne, United Kingdom.

Current Topics in Developmental Biology
|April 20, 2025
PubMed
Summary
This summary is machine-generated.

Primary cilia are vital kidney organelles. Dysfunctional cilia cause renal ciliopathies, including polycystic kidney disease, impacting signalling pathways and cellular metabolism, with ongoing research into new therapies.

Keywords:
Ciliary-specific therapeuticsGenetic therapeuticsHedgehog signallingJoubert syndromeNephronophthisisPolycystic kidney diseasePrimary ciliaWnt signalling

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Area of Science:

  • Nephrology
  • Cell Biology
  • Genetics

Background:

  • Primary cilia are crucial cellular organelles involved in numerous signaling pathways.
  • Their structure and protein complexes are key to kidney function.
  • Primary cilia play a significant role in maintaining kidney homeostasis.

Purpose of the Study:

  • To provide a comprehensive overview of primary cilia function in the kidney.
  • To highlight the link between primary cilia and renal ciliopathies.
  • To explore the molecular mechanisms and therapeutic strategies for these diseases.

Main Methods:

  • Review of existing literature on primary cilia structure, function, and associated diseases.
  • Analysis of in vitro and in vivo models of renal ciliopathies.
  • Examination of signaling pathways, inflammation, and metabolism in disease pathogenesis.

Main Results:

  • Primary cilia dysfunction is central to various renal ciliopathies, including polycystic kidney disease, nephronophthisis, Joubert syndrome, and Bardet-Biedl syndrome.
  • Key molecular mechanisms involve disrupted Wnt and Hedgehog signaling, inflammation, and altered cellular metabolism.
  • Diverse therapeutic approaches are being investigated, from current treatments to novel preclinical and clinical strategies.

Conclusions:

  • Primary cilia are indispensable for kidney health, and their defects lead to severe renal ciliopathies.
  • Understanding the molecular underpinnings of these diseases is crucial for developing effective treatments.
  • Ongoing research offers hope for improved therapeutic interventions for patients with renal ciliopathies.