scholarly journals Function and dynamics of PKD2 in Chlamydomonas reinhardtii flagella

2007 ◽  
Vol 179 (3) ◽  
pp. 501-514 ◽  
Author(s):  
Kaiyao Huang ◽  
Dennis R. Diener ◽  
Aaron Mitchell ◽  
Gregory J. Pazour ◽  
George B. Witman ◽  
...  
Keyword(s):  
Rna Interference ◽  
Kidney Disease ◽  
Chlamydomonas Reinhardtii ◽  
Polycystic Kidney Disease ◽  
Fluorescence Recovery After Photobleaching ◽  
Intraflagellar Transport ◽  
Polycystic Kidney ◽  
Gene Encoding ◽  
Whole Cells ◽  
Flagellar Membrane

To analyze the function of ciliary polycystic kidney disease 2 (PKD2) and its relationship to intraflagellar transport (IFT), we cloned the gene encoding Chlamydomonas reinhardtii PKD2 (CrPKD2), a protein with the characteristics of PKD2 family members. Three forms of this protein (210, 120, and 90 kD) were detected in whole cells; the two smaller forms are cleavage products of the 210-kD protein and were the predominant forms in flagella. In cells expressing CrPKD2–GFP, about 10% of flagellar CrPKD2–GFP was observed moving in the flagellar membrane. When IFT was blocked, fluorescence recovery after photobleaching of flagellar CrPKD2–GFP was attenuated and CrPKD2 accumulated in the flagella. Flagellar CrPKD2 increased fourfold during gametogenesis, and several CrPKD2 RNA interference strains showed defects in flagella-dependent mating. These results suggest that the CrPKD2 cation channel is involved in coupling flagellar adhesion at the beginning of mating to the increase in flagellar calcium required for subsequent steps in mating.

scholarly journals ATP-2 Interacts with the PLAT Domain of LOV-1 and Is Involved inCaenorhabditis elegansPolycystin Signaling

2005 ◽  
Vol 16 (2) ◽  
pp. 458-469 ◽  
Author(s):  
Jinghua Hu ◽  
Maureen M. Barr
Keyword(s):  
Kidney Disease ◽  
Polycystic Kidney Disease ◽  
Atp Synthase ◽  
Male Mating ◽  
Polycystic Kidney ◽  
Gene Encoding ◽  
C Elegans ◽  
Male Mating Behavior ◽  
Evolutionarily Conserved ◽  
Autosomal Dominant Polycystic Kidney

Caenorhabditis elegans is a powerful model to study the molecular basis of autosomal dominant polycystic kidney disease (ADPKD). ADPKD is caused by mutations in the polycystic kidney disease (PKD)1 or PKD2 gene, encoding polycystin (PC)-1 or PC-2, respectively. The C. elegans polycystins LOV-1 and PKD-2 are required for male mating behaviors and are localized to sensory cilia. The function of the evolutionarily conserved polycystin/lipoxygenase/α-toxin (PLAT) domain found in all PC-1 family members remains an enigma. Here, we report that ATP-2, the β subunit of the ATP synthase, physically associates with the LOV-1 PLAT domain and that this interaction is evolutionarily conserved. In addition to the expected mitochondria localization, ATP-2 and other ATP synthase components colocalize with LOV-1 and PKD-2 in cilia. Disrupting the function of the ATP synthase or overexpression of atp-2 results in a male mating behavior defect. We further show that atp-2, lov-1, and pkd-2 act in the same molecular pathway. We propose that the ciliary localized ATP synthase may play a previously unsuspected role in polycystin signaling.

A Novel Gene Encoding a TIG Multiple Domain Protein Is a Positional Candidate for Autosomal Recessive Polycystic Kidney Disease

Genomics ◽  
2002 ◽  
Vol 80 (1) ◽  
pp. 96-104 ◽  
Author(s):  
Huaqi Xiong ◽  
Yongxiong Chen ◽  
Yajun Yi ◽  
Karen Tsuchiya ◽  
Gilbert Moeckel ◽  
...  
Keyword(s):  
Kidney Disease ◽  
Polycystic Kidney Disease ◽  
Autosomal Recessive ◽  
Polycystic Kidney ◽  
Gene Encoding ◽  
Positional Candidate ◽  
Novel Gene ◽  
Domain Protein ◽  
Multiple Domain

scholarly journals Tubby family proteins are adapters for ciliary trafficking of integral membrane proteins

2017 ◽  
Vol 216 (3) ◽  
pp. 743-760 ◽  
Author(s):  
Hemant B. Badgandi ◽  
Sun-hee Hwang ◽  
Issei S. Shimada ◽  
Evan Loriot ◽  
Saikat Mukhopadhyay
Keyword(s):  
Kidney Disease ◽  
Membrane Proteins ◽  
Polycystic Kidney Disease ◽  
Protein Trafficking ◽  
Intraflagellar Transport ◽  
Integral Membrane Proteins ◽  
Dependent Manner ◽  
Polycystic Kidney ◽  
Neuronal Cilia ◽  
Step Model

The primary cilium is a paradigmatic organelle for studying compartmentalized signaling; however, unlike soluble protein trafficking, processes targeting integral membrane proteins to cilia are poorly understood. In this study, we determine that the tubby family protein TULP3 functions as a general adapter for ciliary trafficking of structurally diverse integral membrane cargo, including multiple reported and novel rhodopsin family G protein–coupled receptors (GPCRs) and the polycystic kidney disease–causing polycystin 1/2 complex. The founding tubby family member TUB also localizes to cilia similar to TULP3 and determines trafficking of a subset of these GPCRs to neuronal cilia. Using minimal ciliary localization sequences from GPCRs and fibrocystin (also implicated in polycystic kidney disease), we demonstrate these motifs to be sufficient and TULP3 dependent for ciliary trafficking. We propose a three-step model for TULP3/TUB-mediated ciliary trafficking, including the capture of diverse membrane cargo by the tubby domain in a phosphoinositide 4,5-bisphosphate (PI(4,5)P2)-dependent manner, ciliary delivery by intraflagellar transport complex A binding to the TULP3/TUB N terminus, and subsequent release into PI(4,5)P2-deficient ciliary membrane.

scholarly journals Polycystic kidney disease prevented by transgenic RNA interference

2005 ◽  
Vol 12 (7) ◽  
pp. 831-833 ◽  
Author(s):  
P Bouillet ◽  
M Robati ◽  
M Bath ◽  
A Strasser
Keyword(s):  
Rna Interference ◽  
Kidney Disease ◽  
Polycystic Kidney Disease ◽  
Polycystic Kidney

scholarly journals Chlamydomonas IFT88 and Its Mouse Homologue, Polycystic Kidney Disease Gene Tg737, Are Required for Assembly of Cilia and Flagella

2000 ◽  
Vol 151 (3) ◽  
pp. 709-718 ◽  
Author(s):  
Gregory J. Pazour ◽  
Bethany L. Dickert ◽  
Yvonne Vucica ◽  
E. Scott Seeley ◽  
Joel L. Rosenbaum ◽  
...  
Keyword(s):  
Kidney Disease ◽  
Polycystic Kidney Disease ◽  
Primary Cilia ◽  
Disease Gene ◽  
Intraflagellar Transport ◽  
Polycystic Kidney ◽  
Subunit Protein ◽  
Human Genes ◽  
Protein Particles ◽  
Cilia And Flagella

Intraflagellar transport (IFT) is a rapid movement of multi-subunit protein particles along flagellar microtubules and is required for assembly and maintenance of eukaryotic flagella. We cloned and sequenced a Chlamydomonas cDNA encoding the IFT88 subunit of the IFT particle and identified a Chlamydomonas insertional mutant that is missing this gene. The phenotype of this mutant is normal except for the complete absence of flagella. IFT88 is homologous to mouse and human genes called Tg737. Mice with defects in Tg737 die shortly after birth from polycystic kidney disease. We show that the primary cilia in the kidney of Tg737 mutant mice are shorter than normal. This indicates that IFT is important for primary cilia assembly in mammals. It is likely that primary cilia have an important function in the kidney and that defects in their assembly can lead to polycystic kidney disease.

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