Related Experiment Video

Updated: Jun 1, 2026

Ex Vivo OCT-Based Multimodal Imaging of Human Donor Eyes for Research into Age-Related Macular Degeneration
10:14

Ex Vivo OCT-Based Multimodal Imaging of Human Donor Eyes for Research into Age-Related Macular Degeneration

Published on: May 26, 2023

Dense deposit disease

Richard J H Smith1, Claire L Harris, Matthew C Pickering

  • 1Department of Internal Medicine, Division of Nephrology, Carver College of Medicine, University of Iowa, 21151 PFP, 200 Hawkins Drive, Iowa City, IA 52242, USA. Richard-smith@uiowa.edu

Molecular Immunology
|May 24, 2011
PubMed

Insights

Dense deposit disease (DDD) is a rare kidney disease caused by complement dysregulation. Further genetic and complement studies are crucial for developing targeted therapies and improving patient outcomes.

Area of Science:

  • Nephrology
  • Immunology
  • Genetics

Background:

  • Dense deposit disease (DDD) is a rare orphan disease affecting children and young adults.
  • Its pathophysiology involves fluid-phase dysregulation of the alternative complement pathway.
  • The precise roles of genetics and autoantibodies like C3 nephritic factors require further elucidation.

Purpose of the Study:

  • To investigate the genetic and complement factors in Dense Deposit Disease (DDD).
  • To identify prognostic factors for kidney and transplant survival.
  • To understand risk factors associated with disease progression and allograft loss.

Main Methods:

  • Detailed genetic analysis of DDD patients.
  • Comprehensive complement system studies.
  • Analysis of clinical outcomes including renal failure and transplant survival.

Main Results:

  • Current understanding points to complement pathway dysregulation as the cause of DDD.
  • No mechanism-directed therapies are currently available.
  • Half of diagnosed patients progress to end-stage renal failure within 10 years.
  • Half of kidney transplants in DDD patients fail due to disease recurrence.

Conclusions:

  • Further genetic and complement studies are essential for advancing DDD management.
  • Identifying protective and risk factors can guide therapeutic strategies.
  • Increased healthcare provider awareness and accessible resources are needed to optimize patient care.

More Related Videos

Skin Biopsy for Diagnosing Discoid Lupus Erythematosus05:44

Skin Biopsy for Diagnosing Discoid Lupus Erythematosus

Published on: June 10, 2025

LipidUNet-Machine Learning-Based Method of Characterization and Quantification of Lipid Deposits Using iPSC-Derived Retinal Pigment Epithelium06:16

LipidUNet-Machine Learning-Based Method of Characterization and Quantification of Lipid Deposits Using iPSC-Derived Retinal Pigment Epithelium

Published on: July 28, 2023

Related Concept Videos

Degenerative Disc Disease ll: Pathophysiology01:23

Degenerative Disc Disease ll: Pathophysiology

The symptoms of degenerative disc disease arise from a combination of mechanical compression, vascular compromise, and biochemical inflammation, which together disrupt nerve function and produce pain.Mechanical CompressionDisc degeneration reduces height and elasticity, predisposing to herniation of the nucleus pulposus, a major cause of radicular pain. Herniations may be protrusion (bulging with intact annulus), extrusion (nucleus extends beyond disc but remains connected), or sequestration...
Cardiomyopathy IV: Restrictive Cardiomyopathy01:29

Cardiomyopathy IV: Restrictive Cardiomyopathy

Restrictive cardiomyopathy (RCM) is a rare heart muscle disease characterized by impaired ventricular filling due to stiffened ventricular walls, leading to significant diastolic dysfunction.EtiologyRestrictive cardiomyopathy can arise from both inherited and acquired diseases, many of which are systemic. It is categorized into four main types: infiltrative, storage, non-infiltrative, and endomyocardial diseases.Infiltrative diseases, such as amyloidosis, lead to RCM by depositing amyloid...
Amyloid Fibrils03:03

Amyloid Fibrils

Amyloid fibrils are aggregates of misfolded proteins.  Under most circumstances, misfolded proteins are either refolded by chaperone proteins or degraded by the proteasome. However, in the case of a mutation or a disease, these proteins can accumulate to form large clusters and often further assemble to form elongated fibers, called fibrils. 
Amyloid deposits were observed as early as 1639 in the liver and the spleen.   In 1854, Rudolph Virchow performed iodine staining, normally used to...
Amyloid Fibrils03:03

Amyloid Fibrils

Amyloid fibrils are aggregates of misfolded proteins.  Under most circumstances, misfolded proteins are either refolded by chaperone proteins or degraded by the proteasome. However, in the case of a mutation or a disease, these proteins can accumulate to form large clusters and often further assemble to form elongated fibers, called fibrils. 
Amyloid deposits were observed as early as 1639 in the liver and the spleen.   In 1854, Rudolph Virchow performed iodine staining, normally used to...
Degenerative Disc Disease I: Introduction01:27

Degenerative Disc Disease I: Introduction

Degenerative disc disease is a chronic condition in which intervertebral discs gradually lose structure and function. It is not infectious or autoimmune; rather, it results from age-related biochemical and mechanical changes, influenced by genetic, metabolic, and environmental factors.Structure and Function of DiscsThe spine contains 23 intervertebral discs that absorb load, distribute forces, maintain spacing, and allow flexibility. Each disc consists of a nucleus pulposus, a gel-like core...
Lysosomal Hydrolases01:22

Lysosomal Hydrolases

Lysosomes are the site for the degradation of macromolecules and biological polymers released during membrane trafficking events such as secretory, endocytic, autophagic, and phagocytic pathways. The membrane-enclosed area of the lysosome, called the lumen, contains hydrolytic enzymes active in an acidic environment. These acid hydrolases are functional at a pH between 4.5 and 5 and are involved in cellular processes such as cell signaling, energy metabolism, restoration of the plasma membrane,...