Related Experiment Video
Updated: Jul 1, 2025
10:14
An Efficient Sieving Method to Isolate Intact Glomeruli from Adult Rat Kidney
Published on: November 1, 2018
13.7K
Characterization of glomerular basement membrane components within pediatric glomerular diseases
Dan Chen1, Xindi Zhou1, Chun Gan1
1Department of Nephrology, Children's Hospital of Chongqing Medical University, National Clinical Research Center for Child Health and Disorders, Ministry of Education Key Laboratory of Child Development and Disorders, Chongqing Key Laboratory of Pediatric Metabolism and Inflammatory Diseases, Chongqing, P.R. China.
Clinical Kidney Journal
|March 8, 2024
Summary
Pediatric glomerular diseases show altered expression of key glomerular basement membrane (GBM) proteins. Idiopathic nephrotic syndrome features increased Collagen IV and Laminin 5α, while other conditions exhibit reduced Laminin β2γ1 and Integrin α3β1.
Area of Science:
- Nephrology
- Molecular Biology
- Pathology
Background:
- Glomerular basement membrane (GBM) integrity is crucial for kidney function.
- Dysregulation of GBM components is implicated in pediatric glomerular diseases.
- Systematic characterization of GBM proteins in these conditions is needed.
Purpose of the Study:
- To investigate pathological and molecular changes in key GBM components in pediatric glomerular diseases.
- To correlate these changes with disease type and glomerular function.
Main Methods:
- Systematic analysis of Collagen IV α3α4α5, Laminin α5β2γ1, and Integrin α3β1.
- Focus on prevalent pediatric glomerular diseases including idiopathic nephrotic syndrome (INS), IgA nephropathy (IgAN), IgA vasculitis nephritis (IgAVN), and lupus nephritis (LN).
Main Results:
- Upregulation of Collagen IV α3α4α5 (COL4A3/4/5) and Laminin 5α in INS (Minimal Change Disease, Focal Segmental Glomerulosclerosis).
- Reduction of COL4A3/4/5 and Laminin 5α in IgAN, IgAVN, and LN.
- Decreased expression of Laminin β2γ1 and Integrin α3β1 across various primary and secondary childhood glomerular diseases.
Conclusions:
- Distinct expression patterns of GBM proteins characterize different pediatric glomerular diseases.
- Reduced Laminin β2γ1 and Integrin α3β1 suggest a common GBM structural alteration in childhood glomerular diseases.
- Understanding these molecular changes may inform diagnostic and therapeutic strategies.
Related Concept Videos
01:20Renal Corpuscle
2.1K
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.
Glomerulus: Structure and Function
The glomerulus is a tiny, intricate network of capillaries located at the beginning of the nephron. It's enveloped by the Bowman's capsule and receives its blood supply from an afferent arteriole, which divides into numerous...
Glomerulus: Structure and Function
The glomerulus is a tiny, intricate network of capillaries located at the beginning of the nephron. It's enveloped by the Bowman's capsule and receives its blood supply from an afferent arteriole, which divides into numerous...
2.1K
01:15Glomerular Filtration
1.4K
The filtration membrane in the renal system is a highly specialized structure essential for filtering blood. It consists of glomerular capillaries and podocytes, forming a selective barrier that permits the passage of water and small solutes while restricting most plasma proteins and blood cells.
Components of the Filtration Membrane
The filtration process involves three key layers: the glomerular endothelial cells, the basement membrane, and the podocyte-formed filtration slits.
Components of the Filtration Membrane
The filtration process involves three key layers: the glomerular endothelial cells, the basement membrane, and the podocyte-formed filtration slits.
1.4K
01:10Nephrons
2.4K
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...
2.4K
01:28Glomerular Filtration Rate and its Regulation
2.7K
The Glomerular Filtration Rate (GFR) is a measure of kidney function, reflecting the volume of filtrate formed per minute in the kidneys. On average, GFR is approximately 125 mL/min in males and 105 mL/min in females. Maintaining a relatively constant GFR is essential for the kidneys to effectively regulate body fluid homeostasis and maintain extracellular stability.
GFR regulation involves two primary intrinsic controls: the myogenic and tubuloglomerular feedback mechanisms.
The myogenic...
GFR regulation involves two primary intrinsic controls: the myogenic and tubuloglomerular feedback mechanisms.
The myogenic...
2.7K
01:24Renal Tubule and Collecting Duct
969
The renal tubule is divided into three parts: the proximal convoluted tubule (PCT), the Loop of Henle (LOH), and the distal convoluted tubule (DCT).
Proximal Convoluted Tubule (PCT):
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...
Proximal Convoluted Tubule (PCT):
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...
969
01:21Smooth Endoplasmic Reticulum
5.7K
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.
The ER provides optimal conditions for synthesizing steroid hormones and lipids, such as phospholipids and triglycerides. Traditionally, lipid metabolism was considered to be a smooth ER function. However, there is no direct evidence to prove that rough ER is completely excluded from lipid...
The ER provides optimal conditions for synthesizing steroid hormones and lipids, such as phospholipids and triglycerides. Traditionally, lipid metabolism was considered to be a smooth ER function. However, there is no direct evidence to prove that rough ER is completely excluded from lipid...
5.7K