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Related Concept Videos

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Amyloid Fibrils

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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. 
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Renal Corpuscle01:20

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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
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Nephrons01:10

Nephrons

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The kidneys are intricate organs with millions of working units known as nephrons. Each nephron features two major structures: the renal corpuscle, which facilitates blood plasma filtration, and the renal tubule, which handles the glomerular filtrate. Blood supply is directly linked to the nephrons. The renal corpuscle consists of the glomerulus, a capillary network, and the Bowman's capsule, a double-walled epithelial structure that encases the glomerulus. The filtering of blood plasma...
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Glomerular Filtration01:15

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The filtration membrane in the renal system is a highly specialized structure essential for filtering blood. It consists of glomerular capillaries and podocytes, forming a selective barrier that permits the passage of water and small solutes while restricting most plasma proteins and blood cells.
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Glomerular Filtration: Net Filtration Pressure01:26

Glomerular Filtration: Net Filtration Pressure

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Glomerular filtration, a key process in the kidneys, is regulated by three main pressures: Glomerular blood hydrostatic pressure (GBHP), Capsular hydrostatic pressure (CHP), and Blood colloid osmotic pressure (BCOP).
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Fibronectins Connect Cells with ECM01:25

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Fibronectin is an adhesive glycoprotein present in the extracellular matrix of embryogenic and adult tissue. These molecules primarily aid in regulating cell motility and attachment. A fibronectin molecule is composed of two identical polypeptide chains attached to each other by a pair of disulfide bonds at the C-terminal.
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An Immunohistopathologic Study to Profile the Folate Receptor Beta Macrophage and Vascular Immune Microenvironment in Giant Cell Arteritis
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[Fibrillary glomerulonephritis].

C Dumas De La Roque1, I Brocheriou2, A Mirouse1

  • 1Département de médecine interne et immunologie clinique, hôpital de la Pitié-Salpêtrière, 75013 Paris, France; Centre de référence maladies auto-immunes systémiques rares, Paris, France; Sorbonne université, Paris, France.

La Revue De Medecine Interne
|May 16, 2024
PubMed
Summary
This summary is machine-generated.

Fibrillary glomerulonephritis (FGN), a rare kidney disease, is now diagnosed using the highly accurate anti-DNAJB9 antibody. Early diagnosis and treatment are crucial due to its guarded prognosis and risk of end-stage renal failure.

Keywords:
DNAJB9Fibrillary glomerulonephritisGlomérulonéphrite fibrillaireNephrotic syndromeSyndrome néphrotique

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Area of Science:

  • Nephrology
  • Immunohistochemistry
  • Glomerular Diseases

Context:

  • Fibrillary glomerulonephritis (FGN) is a rare glomerular disease (<1% of renal biopsies) presenting in middle-aged adults.
  • Clinical manifestations include renal failure, proteinuria, hematuria, and hypertension.
  • Histological findings reveal Congo red-negative fibrillary deposits.

Purpose:

  • To highlight the diagnostic advancements in FGN.
  • To discuss the current understanding of FGN's pathophysiology, associations, and treatment.
  • To emphasize the guarded renal prognosis associated with FGN.

Summary:

  • Diagnosis of FGN has been revolutionized by immunohistochemistry using the anti-DNAJB9 antibody, offering high sensitivity (98%) and specificity (99%).
  • Electron microscopy, previously the gold standard, is now superseded by this molecular diagnostic approach.
  • The association of FGN with other conditions like HCV, autoimmune diseases, or neoplasia remains under investigation.

Impact:

  • The anti-DNAJB9 antibody significantly improves the diagnostic accuracy and efficiency for FGN.
  • Understanding FGN's pathophysiology is crucial for developing targeted therapies.
  • Despite treatment, 50% of patients progress to end-stage renal failure within 2-4 years, underscoring the need for further research and improved management strategies.