Vascular expression of polycystin

Research output: Contribution to journalArticle

129 Citations (Scopus)

Abstract

Autosomal dominant polycystic kidney disease (ADPKD) is predominantly caused by mutations of the gene PKD1, which encodes a large protein, polycystin, of unknown function. A variety of arterial abnormalities occur with increased prevalence in ADPKD patients. Using an antiserum against the nonduplicated region of the polycystin protein, immunostaining of vascular smooth muscle cells was detected in normal adult elastic arteries. Partial digestion of tissue slices with nonspecific proteases greatly enhanced this staining. Similar enhancement was seen with specific elastase digestion. Immunostaining for smooth muscle actin was not affected by elastase. Antiserum preadsorbed with peptide antigen gave no staining. In specimens of intracranial aneurysms, aortic dissections, and dolichoectatic arteries from thirteen patients with ADPKD, immunostaining of variable intensity for polycystin was demonstrated in arterial smooth muscle cells and myofibroblasts, along with disruption of elastic laminae. Further elastase digestion did not significantly alter staining patterns. Intracranial aneurysms from patients without ADPKD also showed a variable degree of immunostaining with polycystin antisera in the same distribution. The expression of polycystin in arterial smooth muscle suggests a direct pathogenic role for ADPKD-related mutations in the arterial complications of this disease.

Original languageEnglish (US)
Pages (from-to)616-626
Number of pages11
JournalJournal of the American Society of Nephrology
Volume8
Issue number4
StatePublished - Apr 1997

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TRPP Cation Channels
Autosomal Dominant Polycystic Kidney
Blood Vessels
Pancreatic Elastase
Immune Sera
Digestion
Intracranial Aneurysm
Staining and Labeling
Smooth Muscle Myocytes
Smooth Muscle
Arteries
Mutation
Myofibroblasts
Vascular Smooth Muscle
Dissection
Actins
Proteins
Peptide Hydrolases
Antigens
Peptides

ASJC Scopus subject areas

  • Nephrology

Cite this

Vascular expression of polycystin. / Griffin, Matthew D.; Torres, Vicente; Grande, Joseph Peter; Kumar, Rajiv.

In: Journal of the American Society of Nephrology, Vol. 8, No. 4, 04.1997, p. 616-626.

Research output: Contribution to journalArticle

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