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Related Concept Videos

Nephrotic Syndrome I : Introduction01:24

Nephrotic Syndrome I : Introduction

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Nephrotic Syndrome is a chronic kidney disorder defined by clinical findings such as severe proteinuria, hypoalbuminemia, hyperlipidemia, and edema. These symptoms result from damage to the glomeruli, the kidney’s filtering units, increasing their permeability to proteins.Definition and Meaning:Proteinuria, defined as the loss of more than 3.5 grams of protein per day in adults, is a crucial feature of nephrotic syndrome. This condition is often accompanied by edema, the accumulation of...
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The Proteasome02:18

The Proteasome

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Eukaryotic cells can degrade proteins through several pathways. One of the most important amongst these is the ubiquitin-proteasome pathway. It helps the cell eliminate the misfolded, damaged, or unwarranted cytoplasmic proteins in a highly specific manner.
In this pathway, the target proteins are first tagged with small proteins called ubiquitin. A series of enzymes carry out the ubiquitination of the target proteins - E1 (ubiquitin-activating enzyme), E2 (ubiquitin-conjugating enzyme), and E3...
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The Proteasome01:13

The Proteasome

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Eukaryotic cells can degrade proteins through several pathways. One of the most important among these is the ubiquitin-proteasome pathway. It helps the cell eliminate the misfolded, damaged, or unwarranted cytoplasmic proteins in a highly specific manner.
In this pathway, the target proteins are first tagged with small proteins called ubiquitin. This involves participation of a series of enzymes including— E1 (ubiquitin-activating enzyme), E2 (ubiquitin-conjugating enzyme), and E3...
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Renal Corpuscle01:20

Renal Corpuscle

8.9K
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...
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Nephrotic Syndrome II : Assessment and Medical Management01:26

Nephrotic Syndrome II : Assessment and Medical Management

320
IntroductionNephrotic syndrome is a kidney disorder marked by excessive protein loss in the urine, leading to various systemic complications. This condition often results from damage to the glomeruli—the kidney's filtering units—causing proteinuria, low blood protein levels, and fluid retention. Understanding the assessment, diagnosis, and management of nephrotic syndrome is essential for effective treatment and prevention of further kidney damage.AssessmentPatient History: Document...
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Chronic Kidney Disease I: Introduction01:25

Chronic Kidney Disease I: Introduction

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Chronic Kidney Disease (CKD) arises when the kidneys progressively lose their ability to function, ultimately leading to end-stage renal disease. At this advanced stage, the kidneys can no longer filter waste or maintain essential body functions, requiring renal replacement therapy (RRT) through dialysis or a kidney transplant for survival.Early-stage chronic kidney disease and detection challengesIn CKD's early stages, symptoms often remain absent because healthy nephrons compensate for...
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Related Experiment Video

Updated: Mar 31, 2026

Glomerular Outgrowth as an Ex Vivo Assay to Analyze Pathways Involved in Parietal Epithelial Cell Activation
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Glomerular Outgrowth as an Ex Vivo Assay to Analyze Pathways Involved in Parietal Epithelial Cell Activation

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Dysregulated Protease Homeostasis Defines Primary FSGS.

Johannes Schmidt1, Nina Sopel2, Carmen Rist2

  • 1Department of Preclinical Development and Validation, Fraunhofer Institute for Cell Therapy and Immunology IZI, Leipzig, Germany.

Kidney360
|March 30, 2026
PubMed
Summary
This summary is machine-generated.

Reduced serum serpin family A member 1 (SERPINA1) is a key biomarker distinguishing primary FSGS from other kidney diseases. This finding in FSGS patients and zebrafish models highlights SERPINA1

Keywords:
FSGSbiomarkersnephrotic syndromeproteomics

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An Efficient Sieving Method to Isolate Intact Glomeruli from Adult Rat Kidney
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Area of Science:

  • Nephrology
  • Proteomics
  • Molecular Biology

Background:

  • Primary focal segmental glomerulosclerosis (pFSGS) lacks specific biomarkers for diagnosis and outcome prediction.
  • Circulating permeability factors are implicated in pFSGS pathogenesis.
  • Current research aims to identify unique protein signatures in pFSGS serum.

Purpose of the Study:

  • To identify disease-specific protein signatures in the serum of pFSGS patients using liquid chromatography-mass spectrometry-based proteomics.
  • To investigate the functional role of identified dysregulated proteins in kidney disease models.

Main Methods:

  • Serum proteomics analysis via liquid chromatography-mass spectrometry on samples from pFSGS patients, other proteinuric diseases, and healthy controls.
  • In vitro studies using immortalized human podocytes and in vivo experiments with zebrafish larvae (knockdown studies).
  • Immunofluorescent staining on zebrafish and human kidney biopsies to validate findings.

Main Results:

  • Proteomics identified 27 proteins specifically dysregulated in pFSGS, including proteases and protease inhibitors.
  • Reduced serum serpin family A member 1 (SERPINA1) was specific to pFSGS, unlike increased urinary SERPINA1 in nephrotic syndromes.
  • SERPINA1 knockdown in zebrafish induced proteinuria, edema, and loss of podocyte markers, supporting its role in glomerular injury.

Conclusions:

  • Reduced circulating SERPINA1 is a potential biomarker distinguishing pFSGS.
  • SERPINA1's decrease may disrupt the protease-antiprotease balance, contributing to podocyte injury in pFSGS.
  • SERPINA1 may have compartment-specific functions in glomerular disease pathogenesis.