Disease relevance of Prkd2

• Polycystic kidney disease (PKD) is associated with mutations in PKD1 and PKD2 and vascular abnormalities [1].
• Two of the founding members of the PKD family, PKD1 and PKD2, are responsible for the majority of cases of autosomal dominant polycystic kidney disease [2].

High impact information on Prkd2

• Mutations of either PKD1 or PKD2 are associated with autosomal dominant polycystic kidney disease (ADPKD) [3].
• We show that, when polycystin-1 is expressed alone in a PKD2 null cell line, it localizes to the cell surface, as well as to the endoplasmic reticulum [4].
• Similar to those in aortas, de-endothelialized Pkd2(+/-) resistance (fourth-order mesenteric) arteries responded to PE with a stronger contraction but a lesser [Ca(2+)](i) rise than in wt [5].
• Quantitative analyses of contractile proteins demonstrated elevated expressions in smooth muscle alpha-actin and myosin heavy chain in Pkd2(+/-) arteries, changes that likely contribute to the higher F(max) [5].
• Ca(2+)-dependent calmodulin/myosin light-chain kinase-mediated contraction was examined by direct Ca(2+) (pCa8-5) stimulation to beta-escin permeabilized aortic strips; the pCa-force curve in Pkd2(+/-) strips was not shifted, thereby indicating that PE induced dosage-response alteration that resulted from Ca(2+)-independent mechanisms [5].

Biological context of Prkd2

• Protein kinase D2 contributes to either IL-2 promoter regulation or induction of cell death upon TCR stimulation depending on its activity in Jurkat cells [6].

Anatomical context of Prkd2

• Pkd2+/- vascular smooth muscles develop exaggerated vasocontraction in response to phenylephrine stimulation [5].

Other interactions of Prkd2

• Anomalous structure of primary cilia and/or impairment of increases in intracellular Ca2+ concentration in response to fluid flow are thought to result in renal cyst formation in conditional kif3a knockout, Tg737 and pkd1/pkd2 mutant mice [7].

References