bims-bicyki Biomed News
on Bicaudal-C1 and interactors in cystic kidney disease
Issue of 2022–01–30
thirteen papers selected by
Céline Gagnieux, École Polytechnique Fédérale de Lausanne



  1. Commun Biol. 2022 Jan 25. 5(1): 93
      Primary cilia are sensory organelles on many postmitotic cells. The ciliary membrane is continuous with the plasma membrane but differs in its phospholipid composition with phosphatidylinositol 4,5-bisposphate (PIP2) being much reduced toward the ciliary tip. In order to determine the functional significance of this difference, we used chemically induced protein dimerization to rapidly synthesize or degrade PIP2 selectively in the ciliary membrane. We observed ciliary fission when PIP2 was synthesized and a growing ciliary length when PIP2 was degraded. Ciliary fission required local actin polymerisation in the cilium, the Rho kinase Rac, aurora kinase A (AurkA) and histone deacetylase 6 (HDAC6). This pathway was previously described for ciliary disassembly before cell cycle re-entry. Activating ciliary receptors in the presence of dominant negative dynamin also increased ciliary PIP2, and the associated vesicle budding required ciliary PIP2. Finally, ciliary shortening resulting from constitutively increased ciliary PIP2 was mediated by the same actin - AurkA - HDAC6 pathway. Taken together, changes in ciliary PIP2 are a unifying point for ciliary membrane stability and turnover. Different stimuli increase ciliary PIP2 to secrete vesicles and reduce ciliary length by a common pathway. The paucity of PIP2 in the distal cilium therefore ensures ciliary stability.
    DOI:  https://doi.org/10.1038/s42003-022-03028-1
  2. Ir J Med Sci. 2022 Jan 23.
       BACKGROUND: Among one of the common hereditary causes of chronic kidney disease is autosomal-dominant polycystic kidney disease (ADPKD), and its incidence rate is reported as one between 500 and 1.000 individuals. The most common complications of ADPKD are hypertension (HT) and end-stage renal disease (ESRD). HT occurring in the early stage of ADPKD leads to deteriorations in renal function.
    AIMS: It was aimed to investigate the ischemia-modified albumin (IMA) levels and the effect of renin-angiotensin-aldosterone system (RAAS) blockers on serum IMA levels in patients with ADPKD.
    METHODS: One hundred and fifteen patients were included as ADPKD (n = 50), HT (n = 35), and healthy control (HC) groups (n = 30). Patients with ADPKD and HT were divided into two subgroups as RAAS blocker-users and non-users.
    RESULTS: Serum IMA levels were detected as 0.42 (0.17-0.80) in ADPKD and 0.28 (0.04-0.51) in HT and 0.36 (0.22-0.56) in HC groups as absorbance units (ABSU), and the highest serum IMA level was seen in Group ADPKD. Serum IMA levels were 0.33 ± 0.14 in RAAS blocker-users and 0.41 ± 0.11 ABSU in non-users with ADPKD. Serum IMA levels were witnessed to be significantly lower in RAAS blocker-users in Groups ADPKD (p = 0.038) and HT (p = 0.004), compared to non-users. Given basal and 6-month values of those with ADPKD, the levels of serum IMA within 6 months were significantly lower (p = 0.002).
    CONCLUSIONS: We consider that serum IM levels should be assessed in oxidative stress (OS)-related conditions, such as ADPKD, and RAAS blockers may be effective in reducing serum IMA levels in ADPKD and HT patients.
    Keywords:  Autosomal dominant polycystic kidney disease; Ischemia modified albumin; Oxidative stress; Renin–angiotensin–aldosterone blockade
    DOI:  https://doi.org/10.1007/s11845-022-02925-y
  3. FEBS J. 2022 Jan 24.
      Centrioles and cilia are conserved, microtubule-based structures critical for cell function and development. Their dysfunction causes cancer and developmental disorders. How microtubules are organized into ordered structures by microtubule-associated proteins (MAPs) and tubulin modifications is best understood during mitosis but is largely unexplored for the centrioles and the ciliary axoneme, which are composed of stable microtubules that maintain their length at steady state. In particular, we know little about the identity of the centriolar and ciliary MAPs and how they work together during the assembly and maintenance of the cilium and centriole. Here, we identified the Enkurin domain containing 1 (ENKD1) as a component of the centriole wall and the axoneme in mammalian cells and showed that it has extensive proximity interactions with these compartments and MAPs. Using in vitro and cellular assays, we found that ENKD1 is a new MAP that regulates microtubule organization and stability. Consistently, we observed increase in tubulin polymerization and microtubule stability, as well as disrupted microtubule organization in ENKD1 overexpression. Cells depleted for ENKD1 were defective in ciliary length and content regulation and failed to respond to Hedgehog pathway activation. Together, our results advance our understanding of the functional and regulatory relationship between MAPs and the primary cilium.
    Keywords:  ENKD1; centrosome; hedgehog signaling; microtubules; primary cilium
    DOI:  https://doi.org/10.1111/febs.16367
  4. Indian J Nephrol. 2021 Nov-Dec;31(6):31(6): 507-510
       Introduction: Patients with autosomal dominant polycystic kidney disease (ADPKD) who have normal renal function (creatinine clearance, >90 ml per minute per 1.73 m2 of body-surface area) might potentially benefit from frequent water intake that would be sufficient to reduce plasma AVP levels and decrease the average urine osmolality, bringing it closer to that of plasma.
    Materials and Methods: In this cross-sectional study, the patients of ADPKD, chronic kidney disease stages 1-5 were included. We formed a questionnaire on the dietary recommendation to the patients. The questions enquired whether the patients received the recommendation from the faculty and the postgraduates of the nephrology department that (a) they should consume at least 3000 mL of water per day, (b) that they should not consume coffee and tea,(c) adherence of patients to the advice of the nephrologists.
    Results: Of 294 patients, 142 (48.2%) did not receive any dietary recommendation. The rest 152 (51.7%) were given the appropriate dietary recommendation. Majority of the patients mentioned that they lacked the access to the water when they intend to consume. Despite the advice from the nephrologists, 95 (32.3%) failed to observe the abstinence from coffee and tea. The reason expressed for not quitting coffee and tea was the force of the habit.
    Conclusion: Treating doctorsfailed to inform 48% of patients the proper diet. Only 20.3% of patients consumed >3.0 litre of water per day. The demand of the agricultural work at a place away from home deprived majority of the participants of the study from the potable water.
    Keywords:  ADPKD; Adherence; coffee; cyclic AMP; plasma arginine vasopressin; tea; water drinking
    DOI:  https://doi.org/10.4103/ijn.IJN_379_19
  5. J Reprod Immunol. 2022 Jan 19. pii: S0165-0378(22)00014-6. [Epub ahead of print]150 103486
      Primary cilia regulate cellular signaling and are involved in both sensing and transducing extracellular stimuli. A recent study of patients with recurrent miscarriage (RM) identified mutations affecting DYNC2H1, which were involved in ciliary biogenesis. However, there has been no study concerning primary cilia in the decidua. We compared the number and the length of primary cilia in the decidua of 15 patients with unexplained RM with those of 7 pregnant controls who underwent an artificial termination of pregnancy. Immunohistochemistry was performed using antibodies against primary cilia, extravillous trophoblasts (EVTs), macrophages, uterine Natural Killer (uNK) cells, decidual stromal cells, and the activation of TGF-β and CREB signaling in the decidua of early pregnancy was studied. The density of decidual stromal cells, but not EVTs, macrophages or uNK cells, was found to be significantly higher in the decidua of patients compared to controls. The percentage of ciliated decidual stromal cells was significantly decreased in patients. There was no difference in the primary ciliary length. Regarding TGF-β signaling, p-Smad2 in these cells was diminished significantly in patients, and most of the TGF-β-activated decidual stromal cells of both patients and controls had primary cilia. No difference in the activation of CREB was found. Abnormal primary cilia on decidual stromal cells may be one of the explanatory factors for unknown RM. The inactivation of TGF-β signaling may lead to abnormal ciliogenesis in the decidua.
    Keywords:  CREB signaling; Decidual cells; Primary cilia; Recurrent miscarriage; TGF-β signaling
    DOI:  https://doi.org/10.1016/j.jri.2022.103486
  6. J Hypertens. 2022 Jan 25.
       OBJECTIVE: : Arterial hypertension is a common complication in patients with autosomal recessive polycystic kidney disease (ARPKD), occurring in 33-75% of children when measured by office blood pressure (OBP). Ambulatory blood pressure monitoring (ABPM) is a superior tool for investigating blood pressure relative to OBP. The aim of our study was to investigate the prevalence and control of hypertension in children with ARPKD based on ABPM.
    METHODS: This retrospective study evaluated 36 children with ARPKD and at least one ABPM performed in two our tertiary paediatric nephrology centres and 29 children with at least two ABPM. Ambulatory hypertension was defined as mean daytime or night-time BP at least 95th percentile or use of antihypertensives and controlled hypertension as normal ambulatory BP in children on antihypertensive drugs.
    RESULTS: The first ABPM study revealed ambulatory hypertension in 94% of children. Untreated or uncontrolled ambulatory hypertension was diagnosed in 67% and controlled hypertension in only 28%. Masked hypertension was found in 5.5% and white-coat hypertension in 14%. The last ABPM study revealed ambulatory hypertension in 86% (all 86% hypertensive children on drugs, i.e. no untreated hypertension), the prevalence of controlled hypertension increased to 59%. Masked hypertension was detected in 8.3% and white-coat hypertension in 10%. Ambulatory blood pressure correlated neither with kidney length nor with glomerular filtration rate. Echocardiography demonstrated left ventricular hypertrophy (LVH) in 27% of children at the time of their first ABPM.
    CONCLUSION: The prevalence of ambulatory hypertension is very high in children with ARPKD, while the control of hypertension improves over time.
    DOI:  https://doi.org/10.1097/HJH.0000000000002973
  7. Zhonghua Yi Xue Yi Chuan Xue Za Zhi. 2022 Feb 10. 39(2): 205-208
       OBJECTIVE: To explore the genetic basis for a fetus featuring infantile polycystic kidney disease (IPKD).
    METHODS: Following elective abortion, fetal tissue and peripheral blood samples of its parents were collected for the extraction of genomic DNA. Whole exome sequencing was carried out to detect potential variants correlated with the phenotype.
    RESULTS: The fetus was found to harbor a heterozygous c.1370C>T (p.P457L) variant of the HNF1B gene, which was unreported previously. The same variant was not detected in either parent.
    CONCLUSION: The heterozygous c.1370C>T (p.P457L) variant of the HNF1B gene probably underlay the IPKD in this fetus. Above finding has enabled genetic counseling and prenatal diagnosis for the family.
    DOI:  https://doi.org/10.3760/cma.j.cn511374-20210109-00024
  8. J Cell Sci. 2022 Jan 15. pii: jcs259395. [Epub ahead of print]135(2):
      Centrosomes are the main microtubule-organizing centres, playing essential roles in the organization of the cytoskeleton during interphase, and in the mitotic spindle, which controls chromosome segregation, during cell division. Centrosomes also act as the basal body of cilia, regulating cilium length and affecting extracellular signal reception as well as the integration of intracellular signalling pathways. Centrosomes are self-replicative and duplicate once every cell cycle to generate two centrosomes. The core support structure of the centrosome consists of two molecularly distinct centrioles. The mother (mature) centriole exhibits accessory appendages and is surrounded by both pericentriolar material and centriolar satellites, structures that the daughter (immature) centriole lacks. In this Review, we discuss what is currently known about centrosome duplication, its dialogue with the cell cycle and the sequential acquisition of specific components during centriole maturation. We also describe our current understanding of the mature centriolar structures that are required to build a cilium. Altogether, the built-in centrosome asymmetries that stem from the two centrosomes inheriting molecularly different centrioles sets the foundation for cell division being an intrinsically asymmetric process.
    Keywords:  Cell division; Centrosome asymmetries; Centrosome maturation; Primary cilium
    DOI:  https://doi.org/10.1242/jcs.259395
  9. J Med Chem. 2022 Jan 27. 65(2): 1265-1282
      NIMA-related kinase 1 (Nek1) has lately garnered attention for its widespread function in ciliogenesis, apoptosis, and the DNA-damage response. Despite its involvement in various diseases and its potential as a cancer drug target, no directed medicinal chemistry efforts toward inhibitors against this dark kinase are published. Here, we report the structure-guided design of a potent small-molecule Nek1 inhibitor, starting from a scaffold identified by kinase cross-screening analysis. Seven lead compounds were identified in silico and evaluated for their inhibitory activity. The top compound, 10f, was further profiled for efficacy, toxicity, and bioavailability in a zebrafish polycystic kidney disease model. Administration of 10f caused the expansion of fluorescence-labeled proximal convoluted tubules, supporting our hypothesis that Nek1-inhibition causes cystic kidneys in zebrafish embryos. Compound 10f displayed insignificant inhibition in 48 of 50 kinases in a selectivity test panel. The findings provide a powerful tool to further elucidate the function and pharmacology of this neglected kinase.
    DOI:  https://doi.org/10.1021/acs.jmedchem.0c02118
  10. Front Physiol. 2021 ;12 824971
      
    Keywords:  Ang II; Wnt/β-catenin; hypoxia; hypoxia-inducible factor (HIF); polycystic kidney disease (PKD); vascular endothelial growth factor (VEGF)
    DOI:  https://doi.org/10.3389/fphys.2021.824971
  11. Case Rep Urol. 2022 ;2022 2517674
      Adjustment of immunosuppressive and COVID-19 treatment in terms of drug interactions is still challenging. Herein, we report a 45-year-old woman with end-stage renal disease due to autosomal dominant polycystic diseases (ADPKD) with COVID-19 and pulmonary involvement following kidney transplantation. The patient was properly treated by discontinuation of immunosuppressive drugs, bronchoscopy, and high volume of blood transfusions. The fact that we quickly used early intubation and a new treatment regimen that suppressed immune systems may help physicians develop optimal treatment strategies for similar severe cases. However, this treatment method requires more detailed evaluations due to the contradictory results in reviewing other studies.
    DOI:  https://doi.org/10.1155/2022/2517674
  12. Front Oncol. 2021 ;11 692265
       Objective: Accumulating evidence has highlighted the roles of long noncoding RNAs (lncRNAs) as competing endogenous RNAs (ceRNAs) of microRNAs (miRNAs) through their binding sites in the progression of glioma. Hereby, we aim to explore the role of LINC00883 as a regulator of miR-136 and its target, NIMA-related kinase 1 (NEK1), thus, its involvement in the drug resistance of glioma cells.
    Methods and Results: Mechanistic investigations by dual-luciferase reporter, RNA pull-down, and RNA-binding protein immunoprecipitation (RIP) assays indicated that LINC00883 bound to miR-136, thereby blocking miR-136-induced downregulation of NEK1. Through gain-of-function experiments in U251 cells that presented a high drug resistance, we found that ectopic expression of LINC00883 resulted in increased MRP (encoding multidrug resistance-associated protein), limited cell apoptosis, and increased proliferation. Expectedly, depleting LINC00883 yielded tumor-suppressive and anti-chemoresistance effects on U251 cells by increasing miR-136 and inhibiting NEK1. Next, drug-resistant glioma cell line SOWZ1, drug-sensitive glioma cell line SOWZ2, and drug-resistant glioma cell line SOWZ2-BCNU (SOWZ2 cultured in BCNU) were applied to validate the roles of LINC00883 in the regulation of multidrug resistance. LINC00883 knockdown suppressed the viability of SWOZ1, SWOZ2, and SWOZ2-BCNU cells.
    Conclusion: In conclusion, LINC00883 knockdown reduces drug resistance in glioma. Hence, our study provides a future strategy to prevent drug resistance-induced therapeutic failure in glioma.
    Keywords:  LINC00883; NIMA-related kinase 1; glioma; long noncoding RNA; microRNA-136; multidrug resistance
    DOI:  https://doi.org/10.3389/fonc.2021.692265
  13. Hum Reprod. 2022 Jan 27. pii: deac010. [Epub ahead of print]
       STUDY QUESTION: Does age affect endometrial gene expression?
    SUMMARY ANSWER: Using unsupervised artificial intelligence methods, we report for the first time that endometrial gene expression changes from 35 years of age in women.
    WHAT IS KNOWN ALREADY: Female fertility declines with age, largely attributed to declining oocyte quality and ovarian reserve. Combined with other evidence, a longstanding paradigm holds that age does not affect the endometrial function and age has not been controlled for properly in endometrial studies.
    STUDY DESIGN, SIZE, DURATION: A retrospective in silico analysis was performed of endometrial transcriptomic data from the Gene Expression Omnibus (GEO) sample repository for 27 women of different ages. Results were validated in an independent gene expression dataset of 20 endometrial samples from women aged 23-43 years.
    PARTICIPANTS/MATERIALS, SETTING, METHODS: A systematic search was performed in GEO from October 2016 to January 2019 to identify transcriptomic studies involving women of different ages. Included samples were from norm-ovulatory, women of reproductive age (23-49 years) with regular menstrual cycles who were free of endometriosis and used as controls in a previous endometrial study. We used raw gene expression data and metadata from these samples to investigate the effect of age on endometrial gene expression. Files were downloaded, pre-processed and explored for potential confounding variables and outliers. Artificial intelligence methods were applied to define age groups, and differential expression and functional analyses were applied to demonstrate and understand the effect of age on gene expression at the molecular level. Functional results were validated in an independent gene expression dataset of 20 endometrial samples from women aged 23-43 years.
    MAIN RESULTS AND THE ROLE OF CHANCE: Analysis of the initially retrieved endometrial datasets revealed the age of participants was not available (33.33%) or traceable (43.33%) in most studies. However, one study was suitable for age analysis (GSE4888, n = 27, 23-49 years). Samples showed different transcriptomic profiles according to age, beginning at 35 years. A total of 5778 differentially expressed genes and 27 significantly altered endometrial functions (false discovery rate (FDR) < 0.05) were associated with endometrial gene expression changes related to age. Interestingly, 81.48% of affected functions were related to up-regulation of ciliary processes, with 91 genes involved in cilia motility and ciliogenesis. Other functions included dysregulation of the vascular endothelial growth factor signalling pathway and inhibition of epithelial proliferation triggered by 37 genes involved in cell cycle arrest, angiogenesis, insulin signalling and telomere protection. These findings were validated in an independent dataset using a non-targeted approach; 20 up-regulated ciliary processes (FDR < 0.02) and 6 down-regulated functions related to cell cycle arrest were identified as affected by age, among other hallmarks of ageing such as DNA repair inhibition or sugar metabolism (FDR < 0.05).
    LARGE SCALE DATA: Data underlying this article are available in GEO, IDs: GSE4888 (main dataset) and GSE102131 (validation dataset).
    LIMITATIONS, REASONS FOR CAUTION: This study is limited in size, as are most studies of endometrial transcriptomics where whole-transcriptome analysis considers nearly 22 000 variables in a relatively small population. Yet, our study includes a main sample set and subsequent validation set that enhances reproducibility of our results and provides reasonable evidence for concluding that age affects endometrial gene expression. A larger study prospectively controlling for patient characteristics is needed to accurately describe changes related to age, with a higher sample size and across a wide age range. Additional studies also are necessary to determine the endometrial ageing contribution to infertility for ultimate translation to a clinical setting.
    WIDER IMPLICATIONS OF THE FINDINGS: Our findings support an influence of age on the endometrium in a genome-wide functional approach, breaking the endometrial ageing paradigm in human reproduction. To our knowledge, this work is the first to identify, using a genome-wide functional non-targeted approach, ciliary processes as the primary dysregulated function associated with maternal age. These results should guide the research community to control for age as a potential confounding variable in endometrial gene expression studies and to consider endometrial ageing in further studies as a potential cause of infertility in the clinical setting. The reported functional dysregulations could contribute to diminished embryo implantation with age and further studies will demonstrate if such dysregulation underlies some cases of implantation failure. Additionally, the discovery of these functional alterations could enable mechanistic studies, particularly around the age-related increase in uterine pathologies.
    STUDY FUNDING/COMPETING INTEREST(S): This research was funded by the Instituto de Salud Carlos III through Miguel Servet programme (CP20/00118) granted to Patricia Diaz-Gimeno (Spanish Government) co-funded by FEDER; and by IVI Foundation (1706-FIVI-041-PD). A.D.-P. (FPU/15/01398) and A.P.-L. (FPU18/01777) are granted by the pre-doctoral programme fellowship from the Ministry of Science, Innovation and Universities (Spanish Government). The authors do not have any competing interests to declare.
    TRIAL REGISTRATION NUMBER: N/A.
    Keywords:  cell cycle arrest; ciliary processes; endometrial age; endometrial ageing; endometrial gene expression; endometrial proliferation; endometrial receptivity; fertility; functional genomics; implantation failure
    DOI:  https://doi.org/10.1093/humrep/deac010