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Related Concept Videos

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Type IV collagen is a 400 nm long, network-forming collagen that acts as a barrier between the epithelial and endothelial cells. Type IV collagen  forms the backbone of the basement membrane by scaffolding with laminin, entactin, proteoglycans, and fibronectin. Apart from rendering structural support to the basement membrane, it also helps entail signaling potentials necessary for both pathological and physiological functions.
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Fibril-associated collagens are a type of collagens present in the extracellular matrix with interrupted triple helices or FACIT (Fibril-associated collagens interrupted triple-helices). FACIT help connect and attach the collagen fibrils with each other as well as with other proteins of the extracellular matrix.
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Fibrous proteins are either long and narrow proteins or assemble to form long and thin structures. They contain repetitive units and usually consist of either alpha helices or beta sheets and, in rare cases, a mix of both. The amino acids in the primary structure often consist of repeating amino acid sequences. The role of fibrous proteins is primarily structural. Many are located in the extracellular matrix and are present in connective tissues to impart strength and joint mobility. They are...
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In vitro Synthesis of Native, Fibrous Long Spacing and Segmental Long Spacing Collagen
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Collagenofibrotic glomerulopathy.

Anila Abraham Kurien1, Christopher P Larsen2, L Nicholas Cossey3

  • 1Center for Renal and Urological Pathology , Chennai, Tamil Nadu , India.

Clinical Kidney Journal
|September 29, 2015
PubMed
Summary
This summary is machine-generated.

Collagenofibrotic glomerulopathy, a rare kidney disease, involves type III collagen deposition. This study highlights its sporadic occurrence in adult Indian populations, presenting the largest case series to date.

Keywords:
collagenofibrotic glomerulopathyprimary glomerular fibrosistype III collagen glomerulopathy

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Area of Science:

  • Nephrology
  • Renal Pathology
  • Glomerular Diseases

Background:

  • Collagenofibrotic glomerulopathy is a rare renal disease characterized by glomerular type III collagen deposition.
  • The etiology of this condition remains largely unknown, with limited case series available in medical literature.

Purpose of the Study:

  • To describe the clinical, pathological, and etiological features of collagenofibrotic glomerulopathy.
  • To present the largest case series of collagenofibrotic glomerulopathy in an adult population.

Main Methods:

  • Prospective collection of renal biopsies diagnosed with collagenofibrotic glomerulopathy from 2012 to 2015.
  • Analysis of clinical data, serologic testing, immunofluorescence, and electron microscopy findings from eight adult patients.

Main Results:

  • All patients presented with nephrotic syndrome; five had hypertension. Mean serum creatinine was 146.5 µmol/L.
  • Histopathology revealed diffuse mesangial expansion and PAS-negative material. Electron microscopy showed collagen bundles in the mesangium and subendothelial space.
  • Serologic tests and complement levels were normal; no familial history or associated conditions like nail-patella syndrome were observed.

Conclusions:

  • Collagenofibrotic glomerulopathy appears to be a sporadic, non-familial disease more prevalent in adult Indian populations.
  • This study represents the largest case series of collagenofibrotic glomerulopathy in adults, contributing valuable data to the understanding of this rare condition.