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Related Concept Videos

Amyloid Fibrils03:03

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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,...
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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.
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Related Experiment Video

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Detecting Amyloid-β Accumulation via Immunofluorescent Staining in a Mouse Model of Alzheimer's Disease
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[AA amyloidosis].

Katia Stankovic Stojanovic1, Sophie Georgin-Lavialle1, Gilles Grateau1

  • 1Service de médecine interne, centre de référence des amyloses d'origine inflammatoire et de la fièvre méditerranéenne familiale, hôpital Tenon, 4, rue de la Chine, 75020 Paris, France; Département hospitalo-universitaire inflammation immunopathologie biothérapie (DHU I2B), faculté de médecine, université Pierre-et-Marie-Curie, 4, rue de la Chine, 75020 Paris, France.

Nephrologie & Therapeutique
|May 3, 2017
PubMed
Summary
This summary is machine-generated.

AA amyloidosis, a type of systemic amyloidosis, is decreasing in Western countries. Chronic inflammation drives this condition, impacting kidneys and gut, with treatments focusing on inflammation control.

Keywords:
AA amyloidosisAmyloseImmunohistochemistryImmunohistochimieNephropathyNéphropathieProtéine AAASerum amyloid A protein

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Rapid Generation of Amyloid from Native Proteins In vitro
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Area of Science:

  • Nephrology
  • Rheumatology
  • Immunology

Background:

  • AA amyloidosis is a systemic disease, alongside AL and ATTR amyloidosis, with decreasing incidence in Western nations.
  • Chronic inflammatory conditions like rheumatoid arthritis and autoinflammatory diseases are primary drivers of AA amyloidosis.
  • Castleman's disease is a curable cause, while genetic and environmental factors also contribute to amyloidosis development.

Purpose of the Study:

  • To review the current understanding of AA amyloidosis, including its causes, clinical manifestations, diagnosis, and treatment.
  • To highlight the role of chronic inflammation in the pathogenesis of AA amyloidosis.
  • To discuss emerging therapeutic strategies targeting amyloid formation.

Main Methods:

  • Literature review of AA amyloidosis, focusing on incidence, causes, clinical presentation, diagnostic methods, and treatment options.
  • Analysis of diagnostic criteria, emphasizing the importance of immunohistochemistry for accurate diagnosis.
  • Evaluation of current and investigational treatment strategies, including anti-inflammatory approaches and amyloid formation inhibitors.

Main Results:

  • AA amyloidosis incidence is declining, primarily linked to chronic inflammatory diseases.
  • The initial clinical sign is often chronic glomerular nephropathy, detectable via urinalysis and serum creatinine.
  • Immunohistochemistry is crucial for confirming AA amyloidosis and preventing misdiagnosis; prognosis is poor with gut involvement.

Conclusions:

  • Effective management of AA amyloidosis hinges on controlling the underlying chronic inflammatory response.
  • Targeted therapies inhibiting amyloid formation, such as those against serum amyloid P component, show promise.
  • Further research into novel treatments is essential for improving long-term outcomes in AA amyloidosis patients.