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Vascular expression of polycystin.

M D Griffin¹, V E Torres, J P Grande

¹Nephrology Research Unit, Mayo Clinic, Rochester, Minnesota 55905, USA.

Journal of the American Society of Nephrology : JASN

|April 1, 1997

Summary

Mutations in the PKD1 gene cause autosomal dominant polycystic kidney disease (AD-PKD). Polycystin protein is expressed in vascular smooth muscle cells, suggesting a role in arterial complications of AD-PKD.

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Area of Science:

Vascular Biology
Genetics
Nephrology

Background:

Autosomal dominant polycystic kidney disease (AD-PKD) is linked to PKD1 gene mutations.
AD-PKD patients exhibit a higher prevalence of arterial abnormalities.
The function of polycystin, the protein encoded by PKD1, remains largely unknown.

Purpose of the Study:

To investigate the presence and distribution of polycystin in arterial tissues.
To explore the potential role of polycystin in the arterial complications associated with AD-PKD.

Main Methods:

Immunostaining using an antiserum against the polycystin protein was performed on normal and AD-PKD arterial specimens.
Tissue digestion with proteases and elastase was employed to enhance or modify immunostaining.
Immunostaining for smooth muscle actin and preadsorption with peptide antigen were used as controls.

Main Results:

Polycystin was detected in vascular smooth muscle cells of normal adult elastic arteries, with enhanced staining after protease/elastase digestion.
AD-PKD arterial specimens (aneurysms, dissections, dolichoectatic arteries) showed polycystin in smooth muscle cells and myofibroblasts, alongside disrupted elastic laminae.
Intracranial aneurysms from non-AD-PKD patients also exhibited variable polycystin immunostaining.

Conclusions:

The expression of polycystin in arterial smooth muscle cells is confirmed.
These findings suggest a direct pathogenic role for AD-PKD-related mutations in the development of arterial complications.

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