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Pathological features of proteinuric nephropathy resembling Alport syndrome in a young Pyrenean Mountain dog.

Sugahara G, Naito I, Miyagawa Y, Komiyama T, Takemura N, Kobayashi R, Mineshige T, Kamiie J, Shirota K - J. Vet. Med. Sci. (2015)

Bottom Line: Ultrastructural examination revealed multilaminar splitting and fragmentation of the glomerular basement membrane (GBM) and diffuse podocyte foot process effacement.Immunofluorescent staining for α(IV) chains revealed presence of α5(IV) and complete absence of α3(IV) and α4(IV) chains in the GBM.Immunohistochemistry also revealed decreased and altered expression of nephrin and podocin in the glomeruli compared with normal canine glomeruli.

View Article: PubMed Central - PubMed

Affiliation: Laboratory of Veterinary Pathology, School of Veterinary Medicine, Azabu University, 1-17-71 Fuchinobe, Chuo-ku, Sagamihara, Kanagawa 252-5201, Japan.

ABSTRACT
The renal biopsy tissue from a 9-month-old, male Pyrenean Mountain dog with renal disorder and severe proteinuria was examined. Ultrastructural examination revealed multilaminar splitting and fragmentation of the glomerular basement membrane (GBM) and diffuse podocyte foot process effacement. Immunofluorescent staining for α(IV) chains revealed presence of α5(IV) and complete absence of α3(IV) and α4(IV) chains in the GBM. Immunohistochemistry also revealed decreased and altered expression of nephrin and podocin in the glomeruli compared with normal canine glomeruli. These results suggested that the glomerular disease of the present case might be consistent with canine hereditary nephropathy resembling human Alport syndrome caused by genetic defect of type IV collagen, and indicated possible contribution of podocyte injury to severe proteinuria in this case.

No MeSH data available.


Related in: MedlinePlus

Transmission electron microscopic features of the biopsy specimen. The lamina densa ofthe GBM is irregularly and globally thickened with multilaminar splitting andfragmentation (arrows). Severe foot process effacement is also observed (arrowheads).CL, capillary lumen; US, urinary space; Po, podocyte. TEM. Bar, 2µm.
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fig_002: Transmission electron microscopic features of the biopsy specimen. The lamina densa ofthe GBM is irregularly and globally thickened with multilaminar splitting andfragmentation (arrows). Severe foot process effacement is also observed (arrowheads).CL, capillary lumen; US, urinary space; Po, podocyte. TEM. Bar, 2µm.

Mentions: The ultrastructural appearance of the GBM included distinctive morphological features of HN,namely, irregular and global thickening of the GBM with multilaminar splitting andfragmentation of the lamina densa (Fig. 2Fig. 2.


Pathological features of proteinuric nephropathy resembling Alport syndrome in a young Pyrenean Mountain dog.

Sugahara G, Naito I, Miyagawa Y, Komiyama T, Takemura N, Kobayashi R, Mineshige T, Kamiie J, Shirota K - J. Vet. Med. Sci. (2015)

Transmission electron microscopic features of the biopsy specimen. The lamina densa ofthe GBM is irregularly and globally thickened with multilaminar splitting andfragmentation (arrows). Severe foot process effacement is also observed (arrowheads).CL, capillary lumen; US, urinary space; Po, podocyte. TEM. Bar, 2µm.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4591163&req=5

fig_002: Transmission electron microscopic features of the biopsy specimen. The lamina densa ofthe GBM is irregularly and globally thickened with multilaminar splitting andfragmentation (arrows). Severe foot process effacement is also observed (arrowheads).CL, capillary lumen; US, urinary space; Po, podocyte. TEM. Bar, 2µm.
Mentions: The ultrastructural appearance of the GBM included distinctive morphological features of HN,namely, irregular and global thickening of the GBM with multilaminar splitting andfragmentation of the lamina densa (Fig. 2Fig. 2.

Bottom Line: Ultrastructural examination revealed multilaminar splitting and fragmentation of the glomerular basement membrane (GBM) and diffuse podocyte foot process effacement.Immunofluorescent staining for α(IV) chains revealed presence of α5(IV) and complete absence of α3(IV) and α4(IV) chains in the GBM.Immunohistochemistry also revealed decreased and altered expression of nephrin and podocin in the glomeruli compared with normal canine glomeruli.

View Article: PubMed Central - PubMed

Affiliation: Laboratory of Veterinary Pathology, School of Veterinary Medicine, Azabu University, 1-17-71 Fuchinobe, Chuo-ku, Sagamihara, Kanagawa 252-5201, Japan.

ABSTRACT
The renal biopsy tissue from a 9-month-old, male Pyrenean Mountain dog with renal disorder and severe proteinuria was examined. Ultrastructural examination revealed multilaminar splitting and fragmentation of the glomerular basement membrane (GBM) and diffuse podocyte foot process effacement. Immunofluorescent staining for α(IV) chains revealed presence of α5(IV) and complete absence of α3(IV) and α4(IV) chains in the GBM. Immunohistochemistry also revealed decreased and altered expression of nephrin and podocin in the glomeruli compared with normal canine glomeruli. These results suggested that the glomerular disease of the present case might be consistent with canine hereditary nephropathy resembling human Alport syndrome caused by genetic defect of type IV collagen, and indicated possible contribution of podocyte injury to severe proteinuria in this case.

No MeSH data available.


Related in: MedlinePlus