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Related Concept Videos

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...
Smooth Endoplasmic Reticulum01:21

Smooth Endoplasmic Reticulum

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...
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,...
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 fluid...

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Related Experiment Video

Updated: Jun 4, 2026

Genetic Analysis of Hereditary Transthyretin Ala97Ser Related Amyloidosis
06:33

Genetic Analysis of Hereditary Transthyretin Ala97Ser Related Amyloidosis

Published on: June 9, 2018

Renal amyloidosis in children.

Yelda Bilginer1, Tekin Akpolat, Seza Ozen

  • 1Hacettepe University Faculty of Medicine, Pediatric Nephrology and Rheumatology Unit, Ankara, Turkey.

Pediatric Nephrology (Berlin, Germany)
|March 2, 2011
PubMed
Summary

Renal amyloidosis in children, often caused by autoinflammatory diseases like familial Mediterranean fever, leads to kidney damage. Early diagnosis via histopathology and treating the underlying cause, such as with colchicine, are crucial for management.

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Rapid Generation of Amyloid from Native Proteins In vitro
05:48

Rapid Generation of Amyloid from Native Proteins In vitro

Published on: December 5, 2013

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Last Updated: Jun 4, 2026

Genetic Analysis of Hereditary Transthyretin Ala97Ser Related Amyloidosis
06:33

Genetic Analysis of Hereditary Transthyretin Ala97Ser Related Amyloidosis

Published on: June 9, 2018

Rapid Generation of Amyloid from Native Proteins In vitro
05:48

Rapid Generation of Amyloid from Native Proteins In vitro

Published on: December 5, 2013

Area of Science:

  • Nephrology
  • Immunology
  • Genetics

Background:

  • Renal amyloidosis involves amyloid fibril deposition, potentially causing proteinuria, nephrotic syndrome, and chronic renal failure in children.
  • Historically linked to chronic infections, secondary amyloidosis in children is now predominantly caused by autoinflammatory diseases.
  • Familial Mediterranean fever (FMF) is the most common worldwide autoinflammatory cause, characterized by recurrent inflammation and elevated acute-phase reactants.

Purpose of the Study:

  • To summarize the causes, diagnosis, and treatment of renal amyloidosis in pediatric patients.
  • To highlight the shift in etiology towards autoinflammatory diseases, particularly FMF and cryopyrin-associated periodic syndromes (CAPS).
  • To underscore the importance of managing the underlying autoinflammatory condition for amyloidosis treatment.

Main Methods:

  • Diagnosis relies on characteristic histopathological findings of amyloid deposition.
  • Identifying and managing the primary autoinflammatory disease is the cornerstone of treatment.
  • Colchicine is the primary therapy for familial Mediterranean fever-associated amyloidosis.

Main Results:

  • Autoinflammatory diseases, especially FMF, are the leading causes of secondary amyloidosis in children.
  • Cryopyrin-associated periodic syndromes (CAPS) are another significant cause, with a strong predilection for amyloidosis if untreated.
  • Effective treatment of the underlying autoinflammatory condition is essential to halt or slow amyloid progression.

Conclusions:

  • Renal amyloidosis in children necessitates prompt diagnosis and management of underlying autoinflammatory conditions.
  • Targeting the primary disease, such as FMF or CAPS, is key to preventing further renal damage.
  • Emerging therapies for amyloidosis itself are under development, offering future treatment possibilities.