Quantitative analysis of type IV collagen subchains in the glomerular basement membrane of patients with Alport syndrome with confocal microscopy | Zendy

Jian Su | Zendy; Zhihong Liu | Zendy; Caihong Zeng | Zendy; Wei-Gong | Zendy; Huiping Chen | Zendy; LI Leishi | Zendy

Open Access

Quantitative analysis of type IV collagen subchains in the glomerular basement membrane of patients with Alport syndrome with confocal microscopy

Author(s) -

Jian Su,

Zhihong Liu,

Caihong Zeng,

Wei-Gong,

Huiping Chen,

LI Leishi

Publication year - 2006

Publication title -

nephrology dialysis transplantation

Language(s) - English

Resource type - Journals

SCImago Journal Rank - 1.654

H-Index - 168

eISSN - 1460-2385

pISSN - 0931-0509

DOI - 10.1093/ndt/gfl090

Subject(s) - alport syndrome , type iv collagen , glomerular basement membrane , basement membrane , immunofluorescence , pathology , medicine , confocal microscopy , nephropathy , staining , glomerulonephritis , antibody , microbiology and biotechnology , endocrinology , kidney , extracellular matrix , biology , laminin , immunology , diabetes mellitus

Alport syndrome (AS) is an inherited nephropathy characterized by glomerular basement membrane (GBM) abnormalities due to mutations in the type IV collagen genes. Through immunofluorescence analysis, the absence of alpha3(IV), alpha4(IV) and alpha5(IV) chains within the GBM has been shown in the majority of AS cases. In some atypical AS cases, however, staining of the GBM with antibodies against the alpha3(IV), alpha4(IV) and alpha5(IV) chains appeared normal. In this study, we studied these atypical AS cases by quantitative analysis of the expression of type IV collagen subchains in GBM.

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