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Ultrastructural immunocytochemistry of collagenous and non‐collagenous proteins in fast‐frozen, freeze‐substituted, and low‐temperature‐embedded renal tissue in Alport syndrome
Author(s) -
Onetti Muda Andrea,
Rahimi Siavash,
Renieri Alessandra,
Rizzoni Gianfranco,
Massella Laura,
Faraggiana Tullio
Publication year - 1997
Publication title -
the journal of pathology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.964
H-Index - 184
eISSN - 1096-9896
pISSN - 0022-3417
DOI - 10.1002/(sici)1096-9896(199708)182:4<465::aid-path465>3.0.co;2-v
Subject(s) - basement membrane , alport syndrome , laminin , type iv collagen , glomerular basement membrane , pathology , immunocytochemistry , lamina densa , renal glomerulus , ultrastructure , chemistry , glomerulus , anatomy , biology , glomerulonephritis , extracellular matrix , kidney , microbiology and biotechnology , endocrinology , medicine
This paper describes the ultrastructural immunolocalization of the α2 chain of collagen IV, laminin, and the amino terminal propeptide of collagen I (N‐Pro I) in glomeruli of rapidly frozen, freeze‐substituted, and low‐temperature‐embedded renal biopsy specimens from two cases of Alport disease and from normal kidneys. The α2 chain of collagen IV is present in the whole thickness of the basement membrane in glomeruli of Alport patients, while it is limited to the subendothelial portion of the basement membrane of normal glomeruli. Laminin has the same distribution in both normal and Alport glomeruli, but is apparently more concentrated along the basement membrane of normal glomeruli. N‐Pro I is localized in mesangial areas and in the basement membrane in Alport cases, while it is not detected in normal glomeruli. These data suggest complex rearrangements of major constituents of the glomerular basement membrane network and demonstrate early deposition of fibrillary collagen proteins in the matrix before the appearance of banded collagen fibres. This finding could be an indicator of early evolution towards glomerulosclerosis. © 1997 John Wiley & Sons, Ltd.