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  1. Article ; Online: The term CAKUT has outlived its usefulness: the case for the prosecution.

    Woolf, Adrian S

    Pediatric nephrology (Berlin, Germany)

    2022  Volume 37, Issue 11, Page(s) 2785–2791

    Abstract: CAKUT stands for Congenital Anomalies of the Kidney and Urinary Tract, and the acronym first appeared in a review article published in 1998. Since then, CAKUT has become a familiar term encountered in the medical literature, especially in nephrology ... ...

    Abstract CAKUT stands for Congenital Anomalies of the Kidney and Urinary Tract, and the acronym first appeared in a review article published in 1998. Since then, CAKUT has become a familiar term encountered in the medical literature, especially in nephrology journals. I reason that the term CAKUT was conceived as not a simple description of various diseases, but more as shorthand for a bold conceptual package that linked the occurrence of diverse types of anatomical malformations with insights from genetic and developmental biology research. Moreover, the angiotensin II receptor type 2 was seen as a paradigmatic molecule in the pathobiology of CAKUT. I contend that the acronym, while appearing as an intellectually good idea at the time it was conceived, has outlived its usefulness. To reach these conclusions, I focus on the complex of research observations that led to the theory behind CAKUT, and then question whether these scientific foundations still stand firm. In addition, it is noted that not all clinicians have adopted the acronym, and I speculate why this is the case. I proceed to demonstrate that there is an incompatibility between the semantic meaning of CAKUT and the diseases for which the term was originally conceived. Instead, I suggest the acronym UTM, standing for Urinary Tract Malformation, is a simpler and less ambiguous one to use. Finally, I contend that the continued use of the acronym is a regressive step for the disciplines of nephrology and urology, taking us back two centuries when all kidney diseases were simply called Bright's disease.
    MeSH term(s) Humans ; Kidney/abnormalities ; Receptors, Angiotensin ; Urinary Tract/abnormalities ; Urogenital Abnormalities/genetics ; Vesico-Ureteral Reflux/genetics
    Chemical Substances Receptors, Angiotensin
    Language English
    Publishing date 2022-05-16
    Publishing country Germany
    Document type Journal Article ; Review ; Research Support, Non-U.S. Gov't
    ZDB-ID 631932-4
    ISSN 1432-198X ; 0931-041X
    ISSN (online) 1432-198X
    ISSN 0931-041X
    DOI 10.1007/s00467-022-05576-4
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    Zs.A 2196: Show issues Location:
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  2. Article ; Online: Making human collecting ducts and modeling disease in the laboratory.

    Woolf, Adrian S

    Kidney international

    2021  Volume 100, Issue 2, Page(s) 263–265

    MeSH term(s) Humans ; Laboratories ; Nephrons ; Organoids
    Language English
    Publishing date 2021-02-17
    Publishing country United States
    Document type Journal Article ; Research Support, Non-U.S. Gov't ; Comment
    ZDB-ID 120573-0
    ISSN 1523-1755 ; 0085-2538
    ISSN (online) 1523-1755
    ISSN 0085-2538
    DOI 10.1016/j.kint.2021.02.012
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    Zs.A 797: Show issues Location:
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  3. Article ; Online: Building human renal tracts.

    Woolf, Adrian S

    Journal of pediatric surgery

    2021  Volume 57, Issue 2, Page(s) 172–177

    Abstract: Severe kidney failure affects several million people worldwide. Among these are children born with abnormal renal tracts, and some carry mutations of genes active in renal tract development. Kidney transplants are in short supply, and long term dialysis ... ...

    Abstract Severe kidney failure affects several million people worldwide. Among these are children born with abnormal renal tracts, and some carry mutations of genes active in renal tract development. Kidney transplants are in short supply, and long term dialysis does not obviate uraemia and its associated harmful effects. It has been envisaged that a combination of stem cell technology, developmental biology, and genetics will revolutionise our understanding of kidney disease and provide novel therapies for kidney failure. Here, we review progress towards making functional kidney tissues from human pluripotent stem cells. Organoids rich in immature glomeruli and tubules can be created in culture from pluripotent stem cells. Moreover, differentiation can be increased by implanting these cells into immunodeficient mice. Challenges remain to be overcome, however, before these tissues can be used for regenerative medicine therapies. Current limitations include the small size of an organoid, the lack of large blood vessels feeding it, and the lack of a urinary tract to plumb the kidney organoid. Pluripotent stem cell technology is also being used to create 'diseases in a dish' to understand the pathobiology underlying human renal tract malformations.
    MeSH term(s) Animals ; Cell Differentiation ; Humans ; Kidney ; Kidney Diseases/therapy ; Mice ; Organoids ; Pluripotent Stem Cells
    Language English
    Publishing date 2021-10-29
    Publishing country United States
    Document type Journal Article ; Review
    ZDB-ID 80165-3
    ISSN 1531-5037 ; 0022-3468
    ISSN (online) 1531-5037
    ISSN 0022-3468
    DOI 10.1016/j.jpedsurg.2021.10.022
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  4. Article ; Online: Growing a new human kidney.

    Woolf, Adrian S

    Kidney international

    2019  Volume 96, Issue 4, Page(s) 871–882

    Abstract: There are 3 reasons to generate a new human kidney. The first is to learn more about the biology of the developing and mature organ. The second is to generate tissues with which to model congenital and acquired kidney diseases. In particular, growing ... ...

    Abstract There are 3 reasons to generate a new human kidney. The first is to learn more about the biology of the developing and mature organ. The second is to generate tissues with which to model congenital and acquired kidney diseases. In particular, growing human kidneys in this manner ultimately should help us understand the mechanisms of common chronic kidney diseases such as diabetic nephropathy and others featuring fibrosis, as well as nephrotoxicity. The third reason is to provide functional kidney tissues that can be used directly in regenerative medicine therapies. The second and third reasons to grow new human kidneys are especially compelling given the millions of persons worldwide whose lives depend on a functioning kidney transplant or long-term dialysis, as well as those with end-stage renal disease who die prematurely because they are unable to access these treatments. As shown in this review, the aim to create healthy human kidney tissues has been partially realized. Moreover, the technology shows promise in terms of modeling genetic disease. In contrast, barely the first steps have been taken toward modeling nongenetic chronic kidney diseases or using newly grown human kidney tissue for regenerative medicine therapies.
    MeSH term(s) Animals ; Humans ; Kidney/physiology ; Kidney Failure, Chronic/physiopathology ; Kidney Failure, Chronic/therapy ; Kidney Transplantation/methods ; Organ Culture Techniques/methods ; Regenerative Medicine/methods ; Tissue Engineering/methods
    Language English
    Publishing date 2019-05-25
    Publishing country United States
    Document type Journal Article ; Research Support, Non-U.S. Gov't ; Review
    ZDB-ID 120573-0
    ISSN 1523-1755 ; 0085-2538
    ISSN (online) 1523-1755
    ISSN 0085-2538
    DOI 10.1016/j.kint.2019.04.040
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  5. Article ; Online: TGF-β1 Inhibits Growth and Branching Morphogenesis In Embryonic Mouse Submandibular and Sublingual Glands in Vitro: (Salivary glands/extracellular matrix/epithelium/mesenchyme/organ culture).

    Hardman, Patricia / Landels, Eleanor / Woolf, Adrian S / Spooner, Brian S

    Development, growth & differentiation

    2023  Volume 36, Issue 6, Page(s) 567–577

    Abstract: Members of the TGF-β superfamily of polypeptides are key regulators in developmental processes. Several studies have shown that expression of TGF-β mRNA and protein are developmentally regulated and that both are prominently expressed in tissues ... ...

    Abstract Members of the TGF-β superfamily of polypeptides are key regulators in developmental processes. Several studies have shown that expression of TGF-β mRNA and protein are developmentally regulated and that both are prominently expressed in tissues undergoing epithelial-mesenchymal interactions such as branching morphogenesis. It has been shown that TGF-β1 protein is present in E 14 mouse submandibular glands at a time when branching is already establihsed. Here we demonstrate by RT-PCR and immunofluorescence that both TGF-β1 mRNA and protein are present in E 13 submandibular and sublingual glands at a time when branching is being initiated. Addition of TGF-β1 to E 13 rudiments resulted in reductions in organ size and inhibition of branching. Sensitivity to TGF-β1 depended on the developmental stage of the rudiments (early or late E 13) and the dose of growth factor used. TGF-β1 Also caused epithelial abnormalities, notably treated organs had elongated ducts. The effects were most pronounced in the sublingual gland. Taken together these results suggest a regulatory role for endogenous TGF-β1 in the growth and morphogenesis of mouse salivary glands.
    Language English
    Publishing date 2023-06-06
    Publishing country Japan
    Document type Journal Article
    ZDB-ID 280433-5
    ISSN 1440-169X ; 0012-1592
    ISSN (online) 1440-169X
    ISSN 0012-1592
    DOI 10.1111/j.1440-169X.1994.00567.x
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    Zs.A 194: Show issues Location:
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  6. Article: Heparanase 2 and Urofacial Syndrome, a Genetic Neuropathy.

    Roberts, Neil A / Woolf, Adrian S

    Advances in experimental medicine and biology

    2020  Volume 1221, Page(s) 807–819

    Abstract: Urofacial syndrome (UFS) is a rare but potentially devastating autosomal recessive disease. It comprises both incomplete urinary bladder emptying and a facial grimace upon smiling. A subset of individuals with the disease has biallelic mutations of HPSE2, ...

    Abstract Urofacial syndrome (UFS) is a rare but potentially devastating autosomal recessive disease. It comprises both incomplete urinary bladder emptying and a facial grimace upon smiling. A subset of individuals with the disease has biallelic mutations of HPSE2, coding for heparanase-2. Heparanase-2 and the classical heparanase are both detected in nerves in the maturing bladder, and mice mutant for Hpse2 have UFS-like bladder voiding defects and abnormally patterned bladder nerves. Other evidence suggests that the heparanase axis plays several roles in the peripheral and central nervous systems, quite apart from UFS-related biology. Some individuals with UFS lack HPSE2 mutations and instead carry biallelic variants of LRIG2, encoding leucine-rich-repeats and immunoglobulin-like-domains 2. Like heparanase-2, LRIG2 is detected in bladder nerves, and mutant Lrig2 mice have urination defects and abnormal patterns of bladder nerves. Further work is now needed to define the precise roles of heparanase-2 and LRIG2 in normal and abnormal neural differentiation.
    MeSH term(s) Animals ; Facies ; Glucuronidase/metabolism ; Humans ; Urologic Diseases/enzymology ; Urologic Diseases/genetics
    Chemical Substances heparanase (EC 3.2.1.-) ; Glucuronidase (EC 3.2.1.31)
    Language English
    Publishing date 2020-04-08
    Publishing country United States
    Document type Journal Article ; Review
    ISSN 2214-8019 ; 0065-2598
    ISSN (online) 2214-8019
    ISSN 0065-2598
    DOI 10.1007/978-3-030-34521-1_35
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  7. Article: From human pluripotent stem cells to functional kidney organoids and models of renal disease.

    Kimber, Susan J / Woolf, Adrian S

    Stem cell investigation

    2018  Volume 5, Page(s) 20

    Language English
    Publishing date 2018-07-21
    Publishing country China
    Document type Editorial ; Comment
    ZDB-ID 2884645-X
    ISSN 2313-0792 ; 2306-9759
    ISSN (online) 2313-0792
    ISSN 2306-9759
    DOI 10.21037/sci.2018.07.02
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  8. Article ; Online: Serum-Free Organ Culture of the Embryonic Mouse Ureter.

    Lopes, Filipa M / Woolf, Adrian S

    Methods in molecular biology (Clifton, N.J.)

    2019  Volume 1926, Page(s) 31–38

    Abstract: The ability to explant and then maintain embryonic tissues in organ culture makes it feasible to study the growth and differentiation of whole organs, or parts or combinations of them, in three dimensions. Moreover, the possible effects of biochemical ... ...

    Abstract The ability to explant and then maintain embryonic tissues in organ culture makes it feasible to study the growth and differentiation of whole organs, or parts or combinations of them, in three dimensions. Moreover, the possible effects of biochemical manipulations or mutations can be explored by visualizing a growing organ. The mammalian renal tract comprises the kidney, ureter, and urinary bladder, and the focus of this chapter is organ culture of the embryonic mouse ureter in serum-free defined medium. Over the culture period, rudiments grow in length, smooth muscle differentiates, and the ureters then undergo peristalsis in a proximal to distal direction.
    MeSH term(s) Animals ; Gene Expression Regulation, Developmental ; Kidney/embryology ; Mice ; Mutation/genetics ; Organ Culture Techniques/methods ; Peristalsis/physiology ; Ureter/embryology ; Urinary Bladder/embryology
    Language English
    Publishing date 2019-02-11
    Publishing country United States
    Document type Journal Article ; Research Support, Non-U.S. Gov't
    ISSN 1940-6029
    ISSN (online) 1940-6029
    DOI 10.1007/978-1-4939-9021-4_3
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  9. Article ; Online: Towards Modelling Genetic Kidney Diseases with Human Pluripotent Stem Cells.

    Rooney, Kirsty M / Woolf, Adrian S / Kimber, Susan J

    Nephron

    2021  Volume 145, Issue 3, Page(s) 285–296

    Abstract: Background: Kidney disease causes major suffering and premature mortality worldwide. With no cure for kidney failure currently available, and with limited options for treatment, there is an urgent need to develop effective pharmaceutical interventions ... ...

    Abstract Background: Kidney disease causes major suffering and premature mortality worldwide. With no cure for kidney failure currently available, and with limited options for treatment, there is an urgent need to develop effective pharmaceutical interventions to slow or prevent kidney disease progression.
    Summary: In this review, we consider the feasibility of using human pluripotent stem cell-derived kidney tissues, or organoids, to model genetic kidney disease. Notable successes have been made in modelling genetic tubular diseases (e.g., cystinosis), polycystic kidney disease, and medullary cystic kidney disease. Organoid models have also been used to test novel therapies that ameliorate aberrant cell biology. Some progress has been made in modelling congenital glomerular disease, even though glomeruli within organoids are developmentally immature. Less progress has been made in modelling structural kidney malformations, perhaps because sufficiently mature metanephric mesenchyme-derived nephrons, ureteric bud-derived branching collecting ducts, and a prominent stromal cell population are not generated together within a single protocol. Key Messages: We predict that the field will advance significantly if organoids can be generated with a full complement of cell lineages and with kidney components displaying key physiological functions, such as glomerular filtration. The future economic upscaling of reproducible organoid generation will facilitate more widespread research applications, including the potential therapeutic application of these stem cell-based technologies.
    MeSH term(s) Genetic Predisposition to Disease ; Humans ; Kidney Diseases/congenital ; Kidney Diseases/genetics ; Kidney Diseases/pathology ; Pluripotent Stem Cells/metabolism
    Language English
    Publishing date 2021-03-26
    Publishing country Switzerland
    Document type Journal Article ; Review
    ZDB-ID 207121-6
    ISSN 2235-3186 ; 1423-0186 ; 1660-8151 ; 0028-2766
    ISSN (online) 2235-3186 ; 1423-0186
    ISSN 1660-8151 ; 0028-2766
    DOI 10.1159/000514018
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    Zs.A 169: Show issues Location:
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  10. Article ; Online: Envisioning treating genetically-defined urinary tract malformations with viral vector-mediated gene therapy.

    Lopes, Filipa M / Woolf, Adrian S / Roberts, Neil A

    Journal of pediatric urology

    2021  Volume 17, Issue 5, Page(s) 610–620

    Abstract: Human urinary tract malformations can cause dysfunctional voiding, urosepsis and kidney failure. Other affected individuals, with severe phenotypes on fetal ultrasound screening, undergo elective termination. Currently, there exist no specific treatments ...

    Abstract Human urinary tract malformations can cause dysfunctional voiding, urosepsis and kidney failure. Other affected individuals, with severe phenotypes on fetal ultrasound screening, undergo elective termination. Currently, there exist no specific treatments that target the primary biological disease mechanisms that generate these urinary tract malformations. Historically, the pathogenesis of human urinary tract malformations has been obscure. It is now established that some such individuals have defined monogenic causes for their disease. In health, the implicated genes are expressed in either differentiating urinary tract smooth muscle cells, urothelial cells or peripheral nerve cells supplying the bladder. The phenotypes arising from mutations of these genes include megabladder, congenital functional bladder outflow obstruction, and vesicoureteric reflux. We contend that these genetic and molecular insights can now inform the design of novel therapies involving viral vector-mediated gene transfer. Indeed, this technology is being used to treat individuals with early onset monogenic disease outside the urinary tract, such as spinal muscular atrophy. Moreover, it has been contended that human fetal gene therapy, which may be necessary to ameliorate developmental defects, could become a reality in the coming decades. We suggest that viral vector-mediated gene therapies should first be tested in existing mouse models with similar monogenic and anatomical aberrations as found in people with urinary tract malformations. Indeed, gene transfer protocols have been successfully pioneered in newborn and fetal mice to treat non-urinary tract diseases. If similar strategies were successful in animals with urinary tract malformations, this would pave the way for personalized and potentially curative treatments for people with urinary tract malformations.
    MeSH term(s) Animals ; Genetic Therapy ; Mice ; Urinary Tract/diagnostic imaging ; Urogenital Abnormalities ; Vesico-Ureteral Reflux
    Language English
    Publishing date 2021-07-07
    Publishing country England
    Document type Journal Article ; Review
    ZDB-ID 2237683-5
    ISSN 1873-4898 ; 1477-5131
    ISSN (online) 1873-4898
    ISSN 1477-5131
    DOI 10.1016/j.jpurol.2021.07.002
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