[Amyloid and amyloidoses]

C Röcken1, M Eriksson

  • 1Institut für Pathologie, Charité-Universitätsmedizin Berlin, Charitéplatz 1, 10117, Berlin, Deutschland. christoph.roecken@charite.de

Der Pathologe
|March 26, 2009
PubMed

Insights

Amyloidosis diagnosis relies on Congo red staining and polarization microscopy. Accurate classification of amyloid proteins is crucial for assessing prognosis and guiding patient treatment strategies.

Area of Science:

  • Biochemistry
  • Pathology
  • Genetics

Context:

  • Amyloidosis involves the pathological aggregation of proteins into cross-beta-sheet fibrils.
  • Over 29 distinct amyloid proteins are known, causing diverse cerebral and extracerebral diseases.
  • Diagnosis traditionally involves Congo red staining and polarization microscopy, considered the gold standard.

Purpose:

  • To highlight the diagnostic gold standard for amyloidosis.
  • To emphasize the necessity of amyloid protein classification.
  • To underscore the role of molecular biology in identifying hereditary amyloid diseases.

Summary:

  • Amyloid deposition causes amyloidoses, manifesting as systemic or localized diseases.
  • Polarization microscopy of Congo red-stained tissues is the gold standard for diagnosis.
  • Molecular analyses increasingly aid in classifying amyloid types, including hereditary forms discovered in Germany.

Impact:

  • Correct amyloid classification is vital for accurate prognosis.
  • Informed classification enables personalized patient treatment planning.
  • Advances in molecular biology are enhancing the understanding and diagnosis of hereditary amyloidosis.

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...
Alzheimer Disease ll: Pathophysiology01:23

Alzheimer Disease ll: Pathophysiology

Alzheimer disease involves structural changes in the brain that begin long before symptoms appear. The most distinctive features are extracellular neuritic plaques and intracellular neurofibrillary tangles.Neuritic plaques form in the cerebral cortex and around blood vessels. These plaques contain a dense core of beta-amyloid (Aβ)—a toxic protein fragment that clumps outside neurons. The core is surrounded by damaged neuronal extensions, as well as reactive astrocytes and microglia. Abnormal...
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,...
Alzheimer Disease l: Introduction01:29

Alzheimer Disease l: Introduction

Alzheimer disease is a chronic, progressive, and irreversible neurodegenerative disorder and the most common cause of dementia in older adults. It leads to gradual neuronal loss, causing cognitive decline, behavioral changes, and loss of functional independence.Risk Factors and EtiologyThe disease is multifactorial. Age is the strongest risk factor, with prevalence doubling every 5 years after age 65. Genetic factors include mutations in genes such as APP, PSEN1, and PSEN2, which are associated...
Alzheimer's Disease: Overview01:26

Alzheimer's Disease: Overview

Alzheimer's Disease (AD) is a continually advancing neurodegenerative disorder, distinguished by escalating memory loss, cognitive dysfunction, and dementia. The disease unfolds in three stages: preclinical, mild cognitive impairment (MCI), and dementia. Its onset is insidious, and the progression gradual, with the cause not well explained by other disorders.
The clinical diagnosis of AD hinges on the presence of memory and other cognitive impairments. Biomarkers, such as changes in Aβ and tau...