{"title":"SGLT2 inhibitors in diabetic and non-diabetic kidney transplant recipients: current knowledge and expectations","authors":"E. Polychronopoulou, Fanny Bourdon, Daniel Teta","doi":"10.3389/fneph.2024.1332397","DOIUrl":"https://doi.org/10.3389/fneph.2024.1332397","url":null,"abstract":"The beneficial effect of sodium-glucose cotransporter-2 inhibitors (SGLT2i) have been shown recently in numerous randomized controlled trials (RCT) and systematic reviews. According to KDIGO guidelines, SGLT2i currently represent a first choice for diabetic patients with chronic kidney disease (CKD). In addition, a recent meta-analysis of 13 large led by the ‘SGLT2 inhibitor Meta-Analysis Cardio-Renal Trialists’ Consortium’ (SMART-C) provided solid evidence of SGLT2i beneficial effects in CKD or in patients with heart failure, with and without diabetes. Collectively, the patients treated with SGLT2i had a decreased risk of CKD progression, acute kidney injury (AKI), end-stage kidney disease (ESKD) or death from heart failure. Whether these cardio-renal benefits should be extrapolated to kidney transplant recipients (KTR) needs to be assessed in further studies. In this article, we report recent data accumulated so far in the literature, looking at the efficacy and safety of SGLT2i in diabetic and non-diabetic KTR. We found encouraging data regarding the use of SGLT2i in KTR with diabetes. These agents appeared to be safe, and they reduced body weight and blood pressure in this group of patients. Potential effects on kidney graft function and survival are yet to be investigated.","PeriodicalId":502454,"journal":{"name":"Frontiers in Nephrology","volume":"303 3","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-04-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140703715","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Mario Alamilla-Sanchez, J. D. Diaz Garcia, Valeria Yanez Salguero, Fleuvier Morales Lopez, Victor Manuel Ulloa Galván, Francisco Velasco Garcia-Lascurain, Benjamin Martin Yama Estrella
{"title":"Chemotherapy-induced tubulopathy: a case report series","authors":"Mario Alamilla-Sanchez, J. D. Diaz Garcia, Valeria Yanez Salguero, Fleuvier Morales Lopez, Victor Manuel Ulloa Galván, Francisco Velasco Garcia-Lascurain, Benjamin Martin Yama Estrella","doi":"10.3389/fneph.2024.1384208","DOIUrl":"https://doi.org/10.3389/fneph.2024.1384208","url":null,"abstract":"Acquired tubulopathies are frequently underdiagnosed. They can be characterized by the renal loss of specific electrolytes or organic solutes, suggesting the location of dysfunction. These tubulopathies phenotypically can resemble Bartter or Gitelman syndrome). These syndromes are infrequent, they may present salt loss resembling the effect of thiazides (Gitelman) or loop diuretics (Bartter). They are characterized by potentially severe hypokalemia, associated with metabolic alkalosis, secondary hyperaldosteronism, and often hypomagnesemia. Tubular dysfunction has been described as nephrotoxic effects of platinum-based chemotherapy. We present 4 cases with biochemical signs of tubular dysfunction (Bartter-like/Gitelman-like phenotype) related to chemotherapy.","PeriodicalId":502454,"journal":{"name":"Frontiers in Nephrology","volume":"3 4‐5","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-04-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140716062","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Bioinformatics-driven identification of prognostic biomarkers in kidney renal clear cell carcinoma","authors":"Varinder Madhav Verma, Sanjeev Puri, V. Puri","doi":"10.3389/fneph.2024.1349859","DOIUrl":"https://doi.org/10.3389/fneph.2024.1349859","url":null,"abstract":"Renal cell carcinoma (RCC), particularly the clear cell subtype (ccRCC), poses a significant global health concern due to its increasing prevalence and resistance to conventional therapies. Early detection of ccRCC remains challenging, resulting in poor patient survival rates. In this study, we employed a bioinformatic approach to identify potential prognostic biomarkers for kidney renal clear cell carcinoma (KIRC). By analyzing RNA sequencing data from the TCGA-KIRC project, differentially expressed genes (DEGs) associated with ccRCC were identified. Pathway analysis utilizing the Qiagen Ingenuity Pathway Analysis (IPA) tool elucidated key pathways and genes involved in ccRCC dysregulation. Prognostic value assessment was conducted through survival analysis, including Cox univariate proportional hazards (PH) modeling and Kaplan–Meier plotting. This analysis unveiled several promising biomarkers, such as MMP9, PIK3R6, IFNG, and PGF, exhibiting significant associations with overall survival and relapse-free survival in ccRCC patients. Cox multivariate PH analysis, considering gene expression and age at diagnosis, further confirmed the prognostic potential of MMP9, IFNG, and PGF genes. These findings enhance our understanding of ccRCC and provide valuable insights into potential prognostic biomarkers that can aid healthcare professionals in risk stratification and treatment decision-making. The study also establishes a foundation for future research, validation, and clinical translation of the identified prognostic biomarkers, paving the way for personalized approaches in the management of KIRC.","PeriodicalId":502454,"journal":{"name":"Frontiers in Nephrology","volume":"24 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-04-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140742337","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"NELL1 membranous nephropathy: clinical associations provide mechanistic clues","authors":"N. Andeen, V. Kung, Rupali Avasare","doi":"10.3389/fneph.2024.1323432","DOIUrl":"https://doi.org/10.3389/fneph.2024.1323432","url":null,"abstract":"Neural epidermal growth factor-like 1 (NELL1) membranous nephropathy (MN) is notable for its segmental deposit distribution, IgG1 dominant deposits, and comparatively high rate of spontaneous remission. It has been associated with a variety of exposures and secondary conditions, specifically use of thiol-containing medications – including lipoic acid, bucillamine, and tiopronin – as well as traditional indigenous medications (TIM) particularly those with high mercury content, and non-steroid anti-inflammatory drugs (NSAIDs). Malignancies, graft vs. host disease (GVHD), infection, and autoimmune conditions have also been associated with NELL1 MN. Herein, we provide a detailed summary of the clinicopathologic features of NELL1 and associations with underlying conditions, with a focus on treatment and outcomes. Rare cases of dual NELL1 and phospholipase A2 receptor (PLA2R) positive MN are reviewed. Genome-wide association study of NELL1, role of NELL1 in other physiologic and pathologic processes, and connection between NELL1 MN and malignancy with relevance of NELL1 tumor staining are examined. Finally, relationships and potential disease mechanisms of thiol- and mercury- associated NELL1 MN are discussed.","PeriodicalId":502454,"journal":{"name":"Frontiers in Nephrology","volume":"115 25","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-03-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140380004","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Mythri Shankar, Gouri Satheesh, K. A., S. C. G., Gireesh G Reddy
{"title":"Gender disparity in maintenance hemodialysis units in South India: a cross-sectional observational study","authors":"Mythri Shankar, Gouri Satheesh, K. A., S. C. G., Gireesh G Reddy","doi":"10.3389/fneph.2024.1322003","DOIUrl":"https://doi.org/10.3389/fneph.2024.1322003","url":null,"abstract":"Diseases manifest differently according to gender in many medical specialties. However, sex differences in kidney diseases have not been well explored worldwide, especially in India. These differences could also be attributed to sociocultural factors. Although CKD is more prevalent in women worldwide, most men are initiated on kidney replacement therapy (KRT). This study aimed to examine sex disparities in patients on maintenance hemodialysis.A cross-sectional observational study was conducted in two maintenance hemodialysis units at the Institute of Nephrourology, a tertiary care referral government center in Bengaluru, India. Demographic characteristics and laboratory parameters were also recorded.In total, 374 adult patients (aged >18 years) were included in the study. Most patients (72.7%) were men. Mean age in men was 46.95 ± 12.65 years, and women was 46.63 ± 13.66 years. There was no significant difference in marital status and the availability of caretakers between the groups. Spouses were the predominant caretakers for both sexes (64% men and 51% women, P = 0.14). Sons cared more for patients with mother than fathers (19.6% vs 8.8%, P = 0.074). Diabetic nephropathy was the most common cause of ESKD in both groups (33.1% vs 31.3%, P = 0.92). Men had a significantly longer duration of HTN and received more HD sessions per week than women. Mean hemoglobin (9.9 ± 1.79 vs 9.46 ± 1.47 g%) and mean serum creatinine (7.76 ± 2.65 vs 6.41 ± 2.27 mg/dl) were higher in men compared to women (P <0.002). Intradialytic complications, such as hypotension and cramps, were significantly more common in women than in men (P = 0.004). Most men (47.1%) were planning a kidney transplant (and were waitlisted) compared with fewer women (43%). There was no significant difference in the average number of hospitalizations per month or HD vintage.Women tend to initiate dialysis later, and a lesser number are waitlisted for kidney transplantation, which might be partly related to varying access to or delivery of health care services. Factors such as lack of education, insufficient identification of and strategies to address cultural obstacles to healthcare, and a shortage of financial means to afford medical care are potentially correctable elements that might explain this discrepancy.","PeriodicalId":502454,"journal":{"name":"Frontiers in Nephrology","volume":" 34","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-03-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140383627","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Y. Morita, Eri Sakai, Hideaki Isago, Yoshikazu Ono, Yutaka Yatomi, M. Kurano
{"title":"Alterations in urinary ceramides, sphingoid bases, and their phosphates among patients with kidney disease","authors":"Y. Morita, Eri Sakai, Hideaki Isago, Yoshikazu Ono, Yutaka Yatomi, M. Kurano","doi":"10.3389/fneph.2024.1343181","DOIUrl":"https://doi.org/10.3389/fneph.2024.1343181","url":null,"abstract":"To avoid an invasive renal biopsy, noninvasive laboratory testing for the differential diagnosis of kidney diseases is a desirable goal. As sphingolipids are demonstrated to be involved in the pathogenesis of various kidney diseases, we investigated the possible usefulness of the simultaneous measurement of urinary sphingolipids for differentiating kidney diseases.Residual urine specimens were collected from patients who had been clinically diagnosed with chronic glomerulonephritis (CGN), diabetic mellitus (DM), systemic lupus erythematosus (SLE), and arterial hypertension (AH). The urinary sphingolipids—CERs C16:0, C18:0, C18:1, C20:0, C22:0, and C24:0; sphingosine [Sph]; dihydrosphingosine; sphingosine 1-phosphate [S1P]; and dihydroS1P [dhS1P]—were measured by liquid chromatography–tandem mass spectrometry. Based on the results, machine learning models were constructed to differentiate the various kidney diseases.The urinary S1P was higher in patients with DM than in other participants (P < 0.05), whereas dhS1P was lower in the CGN and AH groups compared with control participants (P < 0.05). Sph and dhSph were higher in patients with CGN, AH, and SLE than in those with control participants (P < 0.05). The urinary CERs were significantly higher in patients with CGN, AH, and SLE than in those with control participants (P < 0.05). As a results of constructing a machine learning model discriminating kidney diseases, the resulting diagnostic accuracy and precision were improved from 94.03% and 66.96% to 96.10% and 78.26% respectively, when the urinary CERs, Sph, dhSph, S1P, dhS1P, and their ratios were added to the models.The urinary CERs, sphingoid bases, and their phosphates show alterations among kidney diseases, suggesting their potential involvement in the development of kidney injury.","PeriodicalId":502454,"journal":{"name":"Frontiers in Nephrology","volume":"35 1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-03-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140077529","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Marina Leiva, Gustavo Navarro, J. D. Carpio, Leopoldo Ardiles
{"title":"Case report: Thrombotic thrombocytopenic purpura in a pregnant woman with lupus membranous nephropathy: a diagnostic challenge","authors":"Marina Leiva, Gustavo Navarro, J. D. Carpio, Leopoldo Ardiles","doi":"10.3389/fneph.2024.1343594","DOIUrl":"https://doi.org/10.3389/fneph.2024.1343594","url":null,"abstract":"A 27-year-old female at 20th week of pregnancy was admitted with edema, foamy urine, but normal blood pressure. Her blood count was normal, she had proteinuria of 3 g/day, creatinine 0.4 mg/dl, albumin 2.4 g/dl, and cholesterol 355 mg/dl. Antinuclear antibodies 1/160, but Anti-DNA, anticardiolipin antibodies and lupus anticoagulant were negative, with normal serum C3 and C4. A renal biopsy showed secondary membranous glomerulopathy, most likely lupus class V pure. Steroids, azathioprine, and aspirin were initiated, up to 28 weeks of pregnancy, when she developed severe hypertension, photopsia, headache, anasarca, extensive bruising of the extremities, severe anemia, thrombocytopenia, and creatinine rose to 2.09 mg/dl with preserved diuresis. A female infant, 1045 grams, was delivered by emergency caesarean section. Following the surgery, she experienced diplopia, dysarthria, bradypsychia, and sensory alterations in the lower extremities, necessitating emergency hemodialysis due to pulmonary congestion. Blood smear revealed schistocytes, LDH elevated at 1148 IU/L, while transaminases and liver function remained normal, suggesting thrombotic thrombocytopenic purpura. ADAMTS13 revealed 6% activity with the presence of inhibitor. Mycophenolate and daily plasmapheresis with fresh frozen plasma replacement yielded unsatisfactory response, unaffected by the addition of methylprednisolone pulses and rituximab. Eventually, intravenous cyclophosphamide was introduced, resulting in complete hematological remission and normalization of ADAMTS13, however dialysis-dependence persisted and four years later, right renal cancer prompted bilateral nephrectomy. After a total follow-up of six years, she remained free of neoplastic recurrence and lupus activity, receiving prednisone and hydroxychloroquine. The differential diagnosis of microangiopathic syndrome in a pregnant lupus patient is discussed.","PeriodicalId":502454,"journal":{"name":"Frontiers in Nephrology","volume":"53 3","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-02-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139864209","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Marina Leiva, Gustavo Navarro, J. D. Carpio, Leopoldo Ardiles
{"title":"Case report: Thrombotic thrombocytopenic purpura in a pregnant woman with lupus membranous nephropathy: a diagnostic challenge","authors":"Marina Leiva, Gustavo Navarro, J. D. Carpio, Leopoldo Ardiles","doi":"10.3389/fneph.2024.1343594","DOIUrl":"https://doi.org/10.3389/fneph.2024.1343594","url":null,"abstract":"A 27-year-old female at 20th week of pregnancy was admitted with edema, foamy urine, but normal blood pressure. Her blood count was normal, she had proteinuria of 3 g/day, creatinine 0.4 mg/dl, albumin 2.4 g/dl, and cholesterol 355 mg/dl. Antinuclear antibodies 1/160, but Anti-DNA, anticardiolipin antibodies and lupus anticoagulant were negative, with normal serum C3 and C4. A renal biopsy showed secondary membranous glomerulopathy, most likely lupus class V pure. Steroids, azathioprine, and aspirin were initiated, up to 28 weeks of pregnancy, when she developed severe hypertension, photopsia, headache, anasarca, extensive bruising of the extremities, severe anemia, thrombocytopenia, and creatinine rose to 2.09 mg/dl with preserved diuresis. A female infant, 1045 grams, was delivered by emergency caesarean section. Following the surgery, she experienced diplopia, dysarthria, bradypsychia, and sensory alterations in the lower extremities, necessitating emergency hemodialysis due to pulmonary congestion. Blood smear revealed schistocytes, LDH elevated at 1148 IU/L, while transaminases and liver function remained normal, suggesting thrombotic thrombocytopenic purpura. ADAMTS13 revealed 6% activity with the presence of inhibitor. Mycophenolate and daily plasmapheresis with fresh frozen plasma replacement yielded unsatisfactory response, unaffected by the addition of methylprednisolone pulses and rituximab. Eventually, intravenous cyclophosphamide was introduced, resulting in complete hematological remission and normalization of ADAMTS13, however dialysis-dependence persisted and four years later, right renal cancer prompted bilateral nephrectomy. After a total follow-up of six years, she remained free of neoplastic recurrence and lupus activity, receiving prednisone and hydroxychloroquine. The differential diagnosis of microangiopathic syndrome in a pregnant lupus patient is discussed.","PeriodicalId":502454,"journal":{"name":"Frontiers in Nephrology","volume":"103 S1","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-02-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139804527","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Yusuke Yoshimura, Daisuke Ikuma, H. Mizuno, K. Kono, K. Kinowaki, Hisashi Sugimoto, Hisashi Kamido, Yuichiro Sawada, Masato Mizuta, Shigekazu Kurihara, Y. Oba, M. Yamanouchi, Tatsuya Suwabe, Kenichi Ohashi, Y. Ubara, N. Sawa
{"title":"Efficacy of SGLT2 inhibitors in IgA nephropathy associated with alcoholic liver cirrhosis accompanied by nephrotic syndrome: a case report","authors":"Yusuke Yoshimura, Daisuke Ikuma, H. Mizuno, K. Kono, K. Kinowaki, Hisashi Sugimoto, Hisashi Kamido, Yuichiro Sawada, Masato Mizuta, Shigekazu Kurihara, Y. Oba, M. Yamanouchi, Tatsuya Suwabe, Kenichi Ohashi, Y. Ubara, N. Sawa","doi":"10.3389/fneph.2023.1331757","DOIUrl":"https://doi.org/10.3389/fneph.2023.1331757","url":null,"abstract":"We present a 51-year-old male patient with a history of Child-Pugh Grade B alcoholic liver cirrhosis (ALC) who developed renal impairment (serum creatinine of 2.00 mg/dL) and nephrotic syndrome (a urinary protein level of 4.35 g/gCr). The patient was diagnosed with immunoglobulin A nephropathy (IgAN) associated with ALC based on findings from comprehensive evaluations, including markedly elevated serum IgA levels (883.7 mg/dL), a kidney biopsy revealing significant IgA deposition in the para-mesangial area, and a liver diagnosis showing long-standing advanced ALC. Our treatment approach involved initiating dapagliflozin therapy, a sodium-glucose cotransporter-2 (SGLT2) inhibitor, alongside strict alcohol abstinence. Remarkably, the patient demonstrated a dramatic reduction in proteinuria within one week of dapagliflozin administration. No hypoglycemic events were observed. This case adds valuable clinical insights into the potential therapeutic role of SGLT2 inhibitors in IgAN associated with ALC. Specifically, in cases where conventional steroid therapies may be contraindicated due to coexisting comorbidities such as diabetes or obesity, dapagliflozin emerges as a potentially efficacious alternative. Further investigations are warranted to validate these preliminary observations.","PeriodicalId":502454,"journal":{"name":"Frontiers in Nephrology","volume":"22 11","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-01-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139609051","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Rita Kalot, Zachary Sentell, Thomas M. Kitzler, Elena Torban
{"title":"Primary cilia and actin regulatory pathways in renal ciliopathies","authors":"Rita Kalot, Zachary Sentell, Thomas M. Kitzler, Elena Torban","doi":"10.3389/fneph.2023.1331847","DOIUrl":"https://doi.org/10.3389/fneph.2023.1331847","url":null,"abstract":"Ciliopathies are a group of rare genetic disorders caused by defects to the structure or function of the primary cilium. They often affect multiple organs, leading to brain malformations, congenital heart defects, and anomalies of the retina or skeletal system. Kidney abnormalities are among the most frequent ciliopathic phenotypes manifesting as smaller, dysplastic, and cystic kidneys that are often accompanied by renal fibrosis. Many renal ciliopathies cause chronic kidney disease and often progress to end-stage renal disease, necessitating replacing therapies. There are more than 35 known ciliopathies; each is a rare hereditary condition, yet collectively they account for a significant proportion of chronic kidney disease worldwide. The primary cilium is a tiny microtubule-based organelle at the apex of almost all vertebrate cells. It serves as a “cellular antenna” surveying environment outside the cell and transducing this information inside the cell to trigger multiple signaling responses crucial for tissue morphogenesis and homeostasis. Hundreds of proteins and unique cellular mechanisms are involved in cilia formation. Recent evidence suggests that actin remodeling and regulation at the base of the primary cilium strongly impacts ciliogenesis. In this review, we provide an overview of the structure and function of the primary cilium, focusing on the role of actin cytoskeleton and its regulators in ciliogenesis. We then describe the key clinical, genetic, and molecular aspects of renal ciliopathies. We highlight what is known about actin regulation in the pathogenesis of these diseases with the aim to consider these recent molecular findings as potential therapeutic targets for renal ciliopathies.","PeriodicalId":502454,"journal":{"name":"Frontiers in Nephrology","volume":"51 24","pages":""},"PeriodicalIF":0.0,"publicationDate":"2024-01-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139527788","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}