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Related Experiment Video

Updated: Sep 30, 2025

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Basement membrane defects in CD151-associated glomerular disease.

Richard W Naylor1, Elizabeth Watson2, Samantha Williamson3

  • 1Wellcome Centre for Cell-Matrix Research, Division of Cell-Matrix Biology and Regenerative Medicine, School of Biological Sciences, Faculty of Biology Medicine and Health, The University of Manchester, Manchester Academic Health Science Centre, Manchester, M13 9PT, UK.

Pediatric Nephrology (Berlin, Germany)
|March 12, 2022
PubMed
Summary
This summary is machine-generated.

A novel CD151 gene variant causes nephrotic syndrome in a child with nail dystrophy and urinary tract infections. This finding highlights CD151

Keywords:
CD151Glomerular Basement MembraneKidney diseaseMER2 PodocyteProteinuria

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Area of Science:

  • Nephrology
  • Genetics
  • Molecular Biology

Background:

  • CD151, a tetraspanin family member, is crucial for podocyte adhesion to the glomerular basement membrane (GBM) via interaction with integrin ɑ3β1.
  • Mouse models lacking Cd151 exhibit glomerular dysfunction, including proteinuria and focal glomerulosclerosis.
  • CD151 is not routinely screened in patients with nephrotic-range proteinuria, necessitating further investigation into its role in human kidney disease.

Observation:

  • A child with nail dystrophy and recurrent urinary tract infections presented with nephrotic-range proteinuria.
  • Targeted next-generation sequencing (NGS) identified a novel, homozygous truncating variant in the CD151 gene.
  • Kidney biopsy revealed GBM thickening and podocyte effacement, with reduced CD151 expression.

Findings:

  • The identified CD151 variant was confirmed as disease-causing using CRISPR-Cas9 in zebrafish, where cd151 depletion induced proteinuria.
  • The zebrafish proteinuria model was rescued by wild-type CD151 mRNA, but not by the variant mRNA.
  • Patient red blood cells showed no immunoreactivity to anti-CD151/MER2 antibodies, consistent with the genetic findings.

Implications:

  • This study identifies a novel CD151 variant associated with nephrotic-range proteinuria and microscopic hematuria in a pediatric patient.
  • It provides further evidence for CD151's critical role in glomerular disease pathogenesis.
  • The findings underscore the importance of considering CD151 in the genetic workup of unexplained nephrotic syndrome and highlight a pipeline for functional variant analysis.