Special Issue "The Kidney: Development, Disease and Regeneration"

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A special issue of Cells (ISSN 2073-4409).

Deadline for manuscript submissions: closed (20 August 2015)

Special Issue Editor

Guest Editor
Prof. Dr. Christoph Englert

Leibniz Institute for Age Research, Fritz Lipmann Institute, 07745 Jena, Germany
Website | E-Mail
Fax: +49 3641 656040
Interests: regulation of gene expression; development; organogenesis; cellular and organismic aging

Special Issue Information

Dear Colleagues,

The kidney is a central and complex organ whose importance cannot be overestimated. It is a pivotal regulator of homeostasis and produces a number of hormones. Its functional unit, the nephron, contains over 10,000 cells and at least 12 different cell types. Each of those has a very specific function and is located in a particular position in relation to other cells along the nephron. In our ageing societies the incidence of kidney diseases increases and constitutes a serious public health problem. Kidney diseases include hypertension, diabetic kidney disease, nephrotic syndromes, IgA nephropathy, idiopathic nephropathy, acute kidney injury, chronic kidney disease, fibrosis, renal cysts and various kidney cancers. While regenerative capacity of the kidney in mammals is limited, the kidney of, e.g., fish can regenerate quite well. Recent advances in this area might help to eventually provide insights into how the regeneration of the mammalian kidney might be enhanced. This Special Issue will provide an Open Access opportunity to publish research work and review articles related to the development, diseases and regeneration of the kidney, and offers comprehensive new insights into this exciting and important research field.

Dr. Christoph Englert
Guest Editor

Submission

Manuscripts should be submitted online at www.mdpi.com by registering and logging in to this website. Once you are registered, click here to go to the submission form. Manuscripts can be submitted until the deadline. Papers will be published continuously (as soon as accepted) and will be listed together on the special issue website. Research articles, review articles as well as communications are invited. For planned papers, a title and short abstract (about 100 words) can be sent to the Editorial Office for announcement on this website.

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Keywords

  • Kidney development
  • pronephros
  • mesonephros
  • metanephros
  • disease
  • nephron
  • nephrotic syndromes
  • polycystic kidney disease
  • regeneration
  • glomerulus
  • tubule
  • podocyte
  • slit diaphragm
  • foot process
  • glomerular filtration rate

Published Papers (8 papers)

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Research

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Open AccessArticle Uremic Toxins Induce ET-1 Release by Human Proximal Tubule Cells, which Regulates Organic Cation Uptake Time-Dependently
Cells 2015, 4(3), 234-252; doi:10.3390/cells4030234
Received: 2 May 2015 / Revised: 12 June 2015 / Accepted: 19 June 2015 / Published: 26 June 2015
PDF Full-text (1543 KB) | HTML Full-text | XML Full-text | Supplementary Files
Abstract
In renal failure, the systemic accumulation of uremic waste products is strongly associated with the development of a chronic inflammatory state. Here, the effect of cationic uremic toxins on the release of inflammatory cytokines and endothelin-1 (ET-1) was investigated in conditionally immortalized proximal
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In renal failure, the systemic accumulation of uremic waste products is strongly associated with the development of a chronic inflammatory state. Here, the effect of cationic uremic toxins on the release of inflammatory cytokines and endothelin-1 (ET-1) was investigated in conditionally immortalized proximal tubule epithelial cells (ciPTEC). Additionally, we examined the effects of ET-1 on the cellular uptake mediated by organic cation transporters (OCTs). Exposure of ciPTEC to cationic uremic toxins initiated production of the inflammatory cytokines IL-6 (117 ± 3%, p < 0.001), IL-8 (122 ± 3%, p < 0.001), and ET-1 (134 ± 5%, p < 0.001). This was accompanied by a down-regulation of OCT mediated 4-(4-(dimethylamino)styryl)-N-methylpyridinium-iodide (ASP+) uptake in ciPTEC at 30 min (23 ± 4%, p < 0.001), which restored within 60 min of incubation. Exposure to ET-1 for 24 h increased the ASP+ uptake significantly (20 ± 5%, p < 0.001). These effects could be blocked by BQ-788, indicating activation of an ET-B-receptor-mediated signaling pathway. Downstream the receptor, iNOS inhibition by (N(G)‐monomethyl‐l‐arginine) l-NMMA acetate or aminoguanidine, as well as protein kinase C activation, ameliorated the short-term effects. These results indicate that uremia results in the release of cytokines and ET-1 from human proximal tubule cells, in vitro. Furthermore, ET-1 exposure was found to regulate proximal tubular OCT transport activity in a differential, time-dependent, fashion. Full article
(This article belongs to the Special Issue The Kidney: Development, Disease and Regeneration)

Review

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Open AccessReview The Role of G-Protein-Coupled Receptor Proteolysis Site Cleavage of Polycystin-1 in Renal Physiology and Polycystic Kidney Disease
Cells 2016, 5(1), 3; doi:10.3390/cells5010003
Received: 3 December 2015 / Revised: 18 January 2016 / Accepted: 19 January 2016 / Published: 21 January 2016
Cited by 1 | PDF Full-text (875 KB) | HTML Full-text | XML Full-text
Abstract
Polycystin-1 (PC1) plays an essential role in renal tubular morphogenesis, and PC1 dysfunction causes human autosomal dominant polycystic kidney disease. A fundamental characteristic of PC1 is post-translational modification via cleavage at the juxtamembrane GPCR proteolysis site (GPS) motif that is part of the
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Polycystin-1 (PC1) plays an essential role in renal tubular morphogenesis, and PC1 dysfunction causes human autosomal dominant polycystic kidney disease. A fundamental characteristic of PC1 is post-translational modification via cleavage at the juxtamembrane GPCR proteolysis site (GPS) motif that is part of the larger GAIN domain. Given the considerable biochemical complexity of PC1 molecules generated in vivo by this process, GPS cleavage has several profound implications on the intracellular trafficking and localization in association with their particular function. The critical nature of GPS cleavage is further emphasized by the increasing numbers of PKD1 mutations that significantly affect this cleavage process. The GAIN domain with the GPS motif therefore represents the key structural element with fundamental importance for PC1 and might be polycystic kidney disease’s (PKD) Achilles’ heel in a large spectrum of PKD1 missense mutations. We highlight the central roles of PC1 cleavage for the regulation of its biogenesis, intracellular trafficking and function, as well as its significance in polycystic kidney disease. Full article
(This article belongs to the Special Issue The Kidney: Development, Disease and Regeneration)
Open AccessReview Role of the Polycystins in Cell Migration, Polarity, and Tissue Morphogenesis
Cells 2015, 4(4), 687-705; doi:10.3390/cells4040687
Received: 7 August 2015 / Revised: 20 October 2015 / Accepted: 26 October 2015 / Published: 30 October 2015
Cited by 2 | PDF Full-text (1471 KB) | HTML Full-text | XML Full-text
Abstract
Cystic kidney diseases (CKD) is a class of disorders characterized by ciliary dysfunction and, therefore, belonging to the ciliopathies. The prototype CKD is autosomal dominant polycystic kidney disease (ADPKD), whose mutated genes encode for two membrane-bound proteins, polycystin-1 (PC-1) and polycystin-2 (PC-2), of
[...] Read more.
Cystic kidney diseases (CKD) is a class of disorders characterized by ciliary dysfunction and, therefore, belonging to the ciliopathies. The prototype CKD is autosomal dominant polycystic kidney disease (ADPKD), whose mutated genes encode for two membrane-bound proteins, polycystin-1 (PC-1) and polycystin-2 (PC-2), of unknown function. Recent studies on CKD-associated genes identified new mechanisms of morphogenesis that are central for establishment and maintenance of proper renal tubular diameter. During embryonic development in the mouse and lower vertebrates a convergent-extension (CE)-like mechanism based on planar cell polarity (PCP) and cellular intercalation is involved in “sculpting” the tubules into a narrow and elongated shape. Once the appropriate diameter is established, further elongation occurs through oriented cell division (OCD). The polycystins (PCs) regulate some of these essential processes. In this review we summarize recent work on the role of PCs in regulating cell migration, the cytoskeleton, and front-rear polarity. These important properties are essential for proper morphogenesis of the renal tubules and the lymphatic vessels. We highlight here several open questions and controversies. Finally, we try to outline some of the next steps required to study these processes and their relevance in physiological and pathological conditions. Full article
(This article belongs to the Special Issue The Kidney: Development, Disease and Regeneration)
Figures

Open AccessReview Epithelial-to-Mesenchymal Transition in Diabetic Nephropathy: Fact or Fiction?
Cells 2015, 4(4), 631-652; doi:10.3390/cells4040631
Received: 28 August 2015 / Revised: 29 September 2015 / Accepted: 30 September 2015 / Published: 9 October 2015
Cited by 8 | PDF Full-text (1384 KB) | HTML Full-text | XML Full-text
Abstract
The pathophysiology of diabetic nephropathy (DN), one of the most serious complications in diabetic patients and the leading cause of end-stage renal disease worldwide, is complex and not fully elucidated. A typical hallmark of DN is the excessive deposition of extracellular matrix (ECM)
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The pathophysiology of diabetic nephropathy (DN), one of the most serious complications in diabetic patients and the leading cause of end-stage renal disease worldwide, is complex and not fully elucidated. A typical hallmark of DN is the excessive deposition of extracellular matrix (ECM) proteins in the glomerulus and in the renal tubulointerstitium, eventually leading to glomerulosclerosis and interstitial fibrosis. Although it is obvious that myofibroblasts play a major role in the synthesis and secretion of ECM, the origin of myofibroblasts in DN remains the subject of controversial debates. A number of studies have focused on epithelial-to-mesenchymal transition (EMT) as one source of matrix-generating fibroblasts in the diseased kidney. EMT is characterized by the acquisition of mesenchymal properties by epithelial cells, preferentially proximal tubular cells and podocytes. In this review we comprehensively review the literature and discuss arguments both for and against a function of EMT in renal fibrosis in DN. While the precise extent of the contribution to nephrotic fibrosis is certainly arduous to quantify, the picture that emerges from this extensive body of literature suggests EMT as a major source of myofibroblasts in DN. Full article
(This article belongs to the Special Issue The Kidney: Development, Disease and Regeneration)
Open AccessReview Albumin and Furosemide Combination for Management of Edema in Nephrotic Syndrome: A Review of Clinical Studies
Cells 2015, 4(4), 622-630; doi:10.3390/cells4040622
Received: 7 August 2015 / Revised: 29 September 2015 / Accepted: 30 September 2015 / Published: 7 October 2015
Cited by 1 | PDF Full-text (673 KB) | HTML Full-text | XML Full-text
Abstract
The treatment of edema in patients with nephrotic syndrome is generally managed by dietary sodium restriction and loop diuretics. However, edema does not improve in some patients despite adequate sodium restriction and maximal dose of diuretics. In such patients, combination of albumin and
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The treatment of edema in patients with nephrotic syndrome is generally managed by dietary sodium restriction and loop diuretics. However, edema does not improve in some patients despite adequate sodium restriction and maximal dose of diuretics. In such patients, combination of albumin and a loop diuretic may improve edema by diuresis and natriuresis. The response to this combination of albumin and a diuretic has not been observed in all studies. The purpose of this review is to discuss the physiology of diuresis and natriuresis of this combination therapy, and provide a brief summary of various studies that have used albumin and a loop diuretic to improve diuretic-resistant edema. Also, the review suggests various reasons for not observing similar results by various investigators. Full article
(This article belongs to the Special Issue The Kidney: Development, Disease and Regeneration)
Open AccessReview Nephron Patterning: Lessons from Xenopus, Zebrafish, and Mouse Studies
Cells 2015, 4(3), 483-499; doi:10.3390/cells4030483
Received: 7 August 2015 / Revised: 1 September 2015 / Accepted: 2 September 2015 / Published: 11 September 2015
Cited by 6 | PDF Full-text (3575 KB) | HTML Full-text | XML Full-text
Abstract
The nephron is the basic structural and functional unit of the vertebrate kidney. To ensure kidney functions, the nephrons possess a highly segmental organization where each segment is specialized for the secretion and reabsorption of particular solutes. During embryogenesis, nephron progenitors undergo a
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The nephron is the basic structural and functional unit of the vertebrate kidney. To ensure kidney functions, the nephrons possess a highly segmental organization where each segment is specialized for the secretion and reabsorption of particular solutes. During embryogenesis, nephron progenitors undergo a mesenchymal-to-epithelial transition (MET) and acquire different segment-specific cell fates along the proximo-distal axis of the nephron. Even if the morphological changes occurring during nephrogenesis are characterized, the regulatory networks driving nephron segmentation are still poorly understood. Interestingly, several studies have shown that the pronephric nephrons in Xenopus and zebrafish are segmented in a similar fashion as the mouse metanephric nephrons. Here we review functional and molecular aspects of nephron segmentation with a particular interest on the signaling molecules and transcription factors recently implicated in kidney development in these three different vertebrate model organisms. A complete understanding of the mechanisms underlying nephrogenesis in different model organisms will provide novel insights on the etiology of several human renal diseases. Full article
(This article belongs to the Special Issue The Kidney: Development, Disease and Regeneration)
Open AccessReview Recent Advances in Elucidating the Genetic Mechanisms of Nephrogenesis Using Zebrafish
Cells 2015, 4(2), 218-233; doi:10.3390/cells4020218
Received: 8 April 2015 / Revised: 19 May 2015 / Accepted: 22 May 2015 / Published: 27 May 2015
Cited by 4 | PDF Full-text (1554 KB) | HTML Full-text | XML Full-text
Abstract
The kidney is comprised of working units known as nephrons, which are epithelial tubules that contain a series of specialized cell types organized into a precise pattern of functionally distinct segment domains. There is a limited understanding of the genetic mechanisms that establish
[...] Read more.
The kidney is comprised of working units known as nephrons, which are epithelial tubules that contain a series of specialized cell types organized into a precise pattern of functionally distinct segment domains. There is a limited understanding of the genetic mechanisms that establish these discrete nephron cell types during renal development. The zebrafish embryonic kidney serves as a simplified yet conserved vertebrate model to delineate how nephron segments are patterned from renal progenitors. Here, we provide a concise review of recent advances in this emerging field, and discuss how continued research using zebrafish genetics can be applied to gain insights about nephrogenesis. Full article
(This article belongs to the Special Issue The Kidney: Development, Disease and Regeneration)
Open AccessReview Signaling during Kidney Development
Cells 2015, 4(2), 112-132; doi:10.3390/cells4020112
Received: 28 February 2015 / Revised: 24 March 2015 / Accepted: 30 March 2015 / Published: 10 April 2015
Cited by 4 | PDF Full-text (1282 KB) | HTML Full-text | XML Full-text
Abstract
The kidney plays an essential role during excretion of metabolic waste products, maintenance of key homeostasis components such as ion concentrations and hormone levels. It influences the blood pressure, composition and volume. The kidney tubule system is composed of two distinct cell populations:
[...] Read more.
The kidney plays an essential role during excretion of metabolic waste products, maintenance of key homeostasis components such as ion concentrations and hormone levels. It influences the blood pressure, composition and volume. The kidney tubule system is composed of two distinct cell populations: the nephrons forming the filtering units and the collecting duct system derived from the ureteric bud. Nephrons are composed of glomeruli that filter the blood to the Bowman’s capsule and tubular structures that reabsorb and concentrate primary urine. The collecting duct is a Wolffian duct-derived epithelial tube that concentrates and collects urine and transfers it via the renal pelvis into the bladder. The mammalian kidney function depends on the coordinated development of specific cell types within a precise architectural framework. Due to the availability of modern analysis techniques, the kidney has become a model organ defining the paradigm to study organogenesis. As kidney diseases are a problem worldwide, the understanding of mammalian kidney cells is of crucial importance to develop diagnostic tools and novel therapies. This review focuses on how the pattern of renal development is generated, how the inductive signals are regulated and what are their effects on proliferation, differentiation and morphogenesis. Full article
(This article belongs to the Special Issue The Kidney: Development, Disease and Regeneration)

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