PMC

Normal pattern of staining for subtypes of type IV collagen. Alpha 1 and alpha 2 type IV collagens are present in all basement membranes. Alpha 3 type IV collagen is present in GBMs and focal distal tubule basement membranes. Alpha 5 type IV collagen is present in GBMs, Bowman's capsule, and focal distal tubule basement membranes (immunofluorescence, FITC-conjugated anti-alpha 1 type IV collagen ×200, Texas Red-conjugated anti-alpha 2 type IV collagen, ×400, FITC-conjugated anti-alpha 3 type IV collagen ×200, FITC-conjugated anti-alpha 5 type IV collagen ×200, FITC-conjugated anti-alpha 5 type IV collagen ×400). GBMs, glomerular basement membranes.

X-linked Alport syndrome pattern of staining for subtypes of type IV collagen in a male patient. Alpha 1 and alpha 2 type IV collagens are present in all basement membranes showing the tissue is adequately preserved for these studies. Alpha 3 and alpha 5 type IV collagens are absent (immunofluorescence FITC-conjugated anti-alpha 1 type IV collagen ×200, Texas Red-conjugated anti-alpha 2 type IV collagen, ×200, FITC-conjugated anti-alpha 3 type IV collagen ×200, FITC-conjugated anti-alpha 5 type IV collagen ×200, FITC-conjugated anti-alpha 5 type IV collagen ×200).

X-linked Alport heterozygous affected female with mosaic pattern of GBM staining for subtypes of type IV collagen. Alpha 1 and alpha 2 type IV collagens are present in all basement membranes showing the tissue is adequately preserved for these studies. Alpha 3 and alpha 5 type IV collagens show mosaic discontinuous staining along GBMs and some Bowman's capsules (immunofluorescence FITC-conjugated anti-alpha 1 type IV collagen ×200, Texas Red-conjugated anti-alpha 2 type IV collagen, ×200, FITC-conjugated anti-alpha 3 type IV collagen ×400, FITC-conjugated anti-alpha 5 type IV collagen ×400, FITC-conjugated anti-alpha 5 type IV collagen ×200). GBM, glomerular basement membrane.

Coexisting protein analysis. Protein gene ontology enrichment analysis (A). The analysis of protein abundance for certain identified proteins. The bluer color, the lower the in relation content of protein (B). AHSG, α2-HS glycoprotein; AIBG, α1-beta glycoprotein; ANXA7, annexin; APOH, beta 2 glycoprotein I; BP, biological process; CC, cellular components; COL1A2, collagen type I alpha 2 chain; COL2A1, collagen type II alpha 1 chain; COL4A1, collagen type IV alpha 1 chain; COL4A2, collagen type IV alpha 2 chain; COL5A1, collagen type V alpha 1 chain; COL5A2, collagen type V alpha 2 chain; COL6A1, collagen type VI alpha 1 chain; COL6A2, collagen type VI alpha 2 chain; COL6A3, collagen type VI alpha 3 chain; COL12A1, collagen type XII alpha 1 chain; COL14A1, collagen type XIV alpha 1 chain; COL24A1, collagen type XIV alpha 1 chain; COL28A1, collagen type XXVII alpha 1 chain; DEP, decellularized epineurium; DCN, decorin; FBN1, fibrillin-1; FN1, fibronectin; FGG, fibrinogen γ; FEP, fresh epineurium; GFAP, glial fibrillary acidic protein; HSPG2, heparan sulfate proteoglycan 2; LAMA1, laminin subunit alpha 1; LAMA2, laminin subunit alpha 2; LAMA5, laminin subunit alpha 5; LAMB1, laminin subunit beta 1; LAMB2, laminin subunit beta 2; LAMC1, laminin subunit gamma 1; LMNA, lamin filament; MF, molecular functions; MPZ, myelin protein; NEFL, neurofilament; PEA15, phosphoprotein enriched in astrocytes 15; TGFB1, transforming growth factor, beta 1; TNXB, tenascin.