Related Experiment Video

Updated: May 3, 2026

Implementing Patch Clamp and Live Fluorescence Microscopy to Monitor Functional Properties of Freshly Isolated PKD Epithelium
08:46

Implementing Patch Clamp and Live Fluorescence Microscopy to Monitor Functional Properties of Freshly Isolated PKD Epithelium

Published on: September 1, 2015

9.2K

Excessive activation of the alternative complement pathway in autosomal dominant polycystic kidney disease

Z Su1, X Wang, X Gao

  • 1Kidney Institute, Department of Nephrology, Shanghai Changzheng Hospital, Second Military Medical University, Shanghai, China.

Journal of Internal Medicine
|February 6, 2014
PubMed

Insights

The complement system, specifically the alternative pathway, is implicated in autosomal dominant polycystic kidney disease (ADPKD) progression. Targeting this pathway with agents like rosmarinic acid (RMA) shows therapeutic potential for ADPKD.

Area of Science:

  • Nephrology
  • Immunology
  • Molecular Biology

Background:

  • The complement system plays a role in immune complex-mediated kidney diseases.
  • Its specific involvement in autosomal dominant polycystic kidney disease (ADPKD) pathogenesis is not well understood.

Purpose of the Study:

  • To investigate the role of the complement system in ADPKD.
  • To explore the therapeutic potential of targeting the complement system in ADPKD.

Main Methods:

  • Urine glycoproteome screening identified complement factors.
  • Immunostaining and Western blot analyzed protein expression in kidney tissues.
  • Pharmacological inhibition of the complement system using rosmarinic acid (RMA) in ADPKD mouse and rat models.

Main Results:

  • Elevated levels of complement factor B (CFB) and complement component 9 (C9) were observed in ADPKD patients.
  • CFB and C9 expression was higher in ADPKD kidneys compared to other chronic kidney diseases.
  • RMA treatment significantly reduced kidney dysfunction, cystogenesis, inflammation, and fibrosis in animal models.

Conclusions:

  • Excessive alternative complement pathway activation is linked to ADPKD progression.
  • Therapeutic strategies targeting the complement system may offer a novel treatment for ADPKD.
Abstract

Keywords:
autosomal dominant polycystic kidney diseasecomplementcomplement inhibitorglycoproteomicsurine

More Related Videos

Use of Ultra-high Field MRI in Small Rodent Models of Polycystic Kidney Disease for In Vivo Phenotyping and Drug Monitoring07:35

Use of Ultra-high Field MRI in Small Rodent Models of Polycystic Kidney Disease for In Vivo Phenotyping and Drug Monitoring

Published on: June 23, 2015

10.9K
Evaluation of Zebrafish Kidney Function Using a Fluorescent Clearance Assay08:13

Evaluation of Zebrafish Kidney Function Using a Fluorescent Clearance Assay

Published on: February 20, 2015

12.9K

Related Concept Videos

Chronic Kidney Disease I: Introduction01:25

Chronic Kidney Disease I: Introduction

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...
1.4K
Complement System01:27

Complement System

The complement system is a group of approximately 20 plasma proteins that strengthen the body's defenses against infections through opsonization, inflammation, and cell lysis. Opsonization involves coating pathogens with complement proteins, making them more recognizable and facilitating phagocyte engulfment. Certain complement proteins induce inflammation that attracts immune cells to the site of infection. Cell lysis involves the destruction of pathogens through the formation of a...
10.6K
Renal Corpuscle01:20

Renal Corpuscle

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...
8.4K
Diabetic Nephropathy01:28

Diabetic Nephropathy

Definition Diabetic nephropathy is a chronic kidney complication that results from prolonged hyperglycemia.Prevalence It is the most common cause of chronic kidney disease (CKD) and end-stage renal disease (ESRD) worldwide, affecting up to half of individuals with diabetes.Pathophysiology • Sustained hyperglycemia triggers multiple hemodynamic and metabolic changes in the kidney. • Early in the disease, increased renal blood flow and glomerular hyperfiltration...
32
Acute Kidney Injury II: Pathophysiology01:29

Acute Kidney Injury II: Pathophysiology

Acute kidney injury (AKI) causes are categorized into three primary categories based on the location of the injury: prerenal, intrarenal (or intrinsic), and postrenal causes. This classification guides clinical management and illustrates how different pathways can impair kidney function.Etiology and Pathophysiology of Acute Kidney Injury1. Prerenal causesEtiology: Prerenal Acute Kidney Injury, the most common type, occurs when reduced blood flow to the kidneys decreases filtration capacity...
2.3K
Nephrotic Syndrome I : Introduction01:24

Nephrotic Syndrome I : Introduction

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...
1.2K