Morphological diagnosis of Alport syndrome and thin basement membrane nephropathy by low vacuum scanning electron microscopy
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- OKADA Shinichi
- Division of Pediatrics and Perinatology, Faculty of Medicine, Tottori University
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- INAGA Sumire
- Division of Genomic Morphology, Faculty of Medicine, Tottori University
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- KITAMOTO Koichi
- Division of Pediatrics and Perinatology, Faculty of Medicine, Tottori University
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- KAWABA Yasuo
- Division of Pediatrics and Perinatology, Faculty of Medicine, Tottori University
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- NAKANE Hironobu
- Division of Genomic Morphology, Faculty of Medicine, Tottori University
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- NAGURO Tomonori
- Division of Genomic Morphology, Faculty of Medicine, Tottori University
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- KAIDOH Toshiyuki
- Division of Genomic Morphology, Faculty of Medicine, Tottori University
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- KANZAKI Susumu
- Division of Pediatrics and Perinatology, Faculty of Medicine, Tottori University
書誌事項
- タイトル別名
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- <b>Morphological diagnosis of Alport syndrome and thin basement membrane nephropathy by low vacuum scanning electron micr</b><b>oscopy </b>
この論文をさがす
抄録
Alport syndrome (AS) and thin basement membrane nephropathy (TBMN) are genetic disorders caused by mutations of the type IV collagen genes COL4A3, COL4A4, and/or COL4A5. We here aimed to investigate the three-dimensional ultrastructure of the glomerular basement membrane (GBM) in order to introduce a novel method of diagnosing AS and TBMN. The subjects were 4 patients with AS and 6 patients with TBMN. Conventional renal biopsy paraffin sections from AS and TBMN patients were stained with periodic acid methenamine silver (PAM) and observed directly under low vacuum scanning electron microscopy (LVSEM). The PAM-positive GBMs were clearly visible under LVSEM through the overlying cellular components. The GBMs showed characteristic coarse meshwork appearances in AS, and thin and sheet-like appearances in TBMN. At the cut side view of the capillary wall, the GBMs in AS appeared as fibrous inclusions between a podocyte and an endothelial cell, while the GBMs in TBMN showed thin linear appearances. These different findings of GBMs between AS and TBMN were easily observed under LVSEM. Thus, we conclude that three-dimensional morphological evaluation by LVSEM using conventional renal biopsy paraffin sections will likely be useful for the diagnosis of AS and TBMN, including for retrospective investigations.
収録刊行物
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- Biomedical Research
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Biomedical Research 35 (5), 345-350, 2014
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