{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,23]],"date-time":"2026-10-23T20:05:16Z","timestamp":1792785916152,"version":"4.5.0"},"reference-count":64,"publisher":"Oxford University Press (OUP)","issue":"14","license":[{"start":{"date-parts":[[2020,6,26]],"date-time":"2020-06-26T00:00:00Z","timestamp":1593129600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/journals\/pages\/open_access\/funder_policies\/chorus\/standard_publication_model"}],"funder":[{"name":"Deutsche Muskelstiftung","award":["E-2019-01"],"award-info":[{"award-number":["E-2019-01"]}]},{"name":"SMA Europe"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2020,8,11]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Spinal muscular atrophy (SMA) occurs as a result of cell-ubiquitous depletion of the essential survival motor neuron (SMN) protein. Characteristic disease pathology is driven by a particular vulnerability of the ventral motor neurons of the spinal cord to decreased SMN. Perhaps not surprisingly, many other organ systems are also impacted by SMN depletion. The normal kidney expresses very high levels of SMN protein, equivalent to those found in the nervous system and liver, and levels are dramatically lowered by ~90\u201395% in mouse models of SMA. Taken together, these data suggest that renal pathology may be present in SMA. We have addressed this using an established mouse model of severe SMA. Nephron number, as assessed by gold standard stereological techniques, was significantly reduced. In addition, morphological assessment showed decreased renal vasculature, particularly of the glomerular capillary knot, dysregulation of nephrin and collagen IV, and ultrastructural changes in the trilaminar filtration layers of the nephron. To explore the molecular drivers underpinning this process, we correlated these findings with quantitative PCR measurements and protein analyses of glial cell-line-derived neurotrophic factor, a crucial factor in ureteric bud branching and subsequent nephron development. Glial cell-line-derived neurotrophic factor levels were significantly reduced at early stages of disease in SMA mice. Collectively, these findings reveal significant renal pathology in a mouse model of severe SMA, further reinforcing the need to develop and administer systemic therapies for this neuromuscular disease.<\/jats:p>","DOI":"10.1093\/hmg\/ddaa126","type":"journal-article","created":{"date-parts":[[2020,6,16]],"date-time":"2020-06-16T15:11:26Z","timestamp":1592320286000},"page":"2365-2378","source":"Crossref","is-referenced-by-count":27,"title":["Renal pathology in a mouse model of severe Spinal Muscular Atrophy is associated with downregulation of Glial Cell-Line Derived Neurotrophic Factor (GDNF)"],"prefix":"10.1093","volume":"29","author":[{"given":"Hazel","family":"Allardyce","sequence":"first","affiliation":[{"name":"Institute of Medical Sciences, School of Medicine, Medical Sciences and Nutrition, University of Aberdeen, Aberdeen AB25 2ZD, UK"},{"name":"Euan Macdonald Centre for Motor Neurone Disease Research, University of Edinburgh, Edinburgh EH16 4SB, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Daniela","family":"Kuhn","sequence":"additional","affiliation":[{"name":"Hannover Medical School, Institute of Neuroanatomy and Cell Biology, Hannover 30625, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elena","family":"Hernandez-Gerez","sequence":"additional","affiliation":[{"name":"Institute of Medical Sciences, School of Medicine, Medical Sciences and Nutrition, University of Aberdeen, Aberdeen AB25 2ZD, UK"},{"name":"Euan Macdonald Centre for Motor Neurone Disease Research, University of Edinburgh, Edinburgh EH16 4SB, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Niko","family":"Hensel","sequence":"additional","affiliation":[{"name":"Hannover Medical School, Institute of Neuroanatomy and Cell Biology, Hannover 30625, Germany"},{"name":"Center for Systems Neuroscience (ZSN) Hannover, University of Veterinary Medicine Hannover, Hannover 30559, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yu-Ting","family":"Huang","sequence":"additional","affiliation":[{"name":"Euan Macdonald Centre for Motor Neurone Disease Research, University of Edinburgh, Edinburgh EH16 4SB, UK"},{"name":"Edinburgh Medical School: Biomedical Sciences, College of Medicine & Veterinary Medicine, University of Edinburgh, Edinburgh EH8 9AG, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kiterie","family":"Faller","sequence":"additional","affiliation":[{"name":"Euan Macdonald Centre for Motor Neurone Disease Research, University of Edinburgh, Edinburgh EH16 4SB, UK"},{"name":"Edinburgh Medical School: Biomedical Sciences, College of Medicine & Veterinary Medicine, University of Edinburgh, Edinburgh EH8 9AG, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0306-5577","authenticated-orcid":false,"given":"Thomas H","family":"Gillingwater","sequence":"additional","affiliation":[{"name":"Euan Macdonald Centre for Motor Neurone Disease Research, University of Edinburgh, Edinburgh EH16 4SB, UK"},{"name":"Edinburgh Medical School: Biomedical Sciences, College of Medicine & Veterinary Medicine, University of Edinburgh, Edinburgh EH8 9AG, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fabio","family":"Quondamatteo","sequence":"additional","affiliation":[{"name":"Anatomy Facility, School of Life Sciences, University of Glasgow, University Avenue, Glasgow G12 8QQ, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Peter","family":"Claus","sequence":"additional","affiliation":[{"name":"Hannover Medical School, Institute of Neuroanatomy and Cell Biology, Hannover 30625, Germany"},{"name":"Center for Systems Neuroscience (ZSN) Hannover, University of Veterinary Medicine Hannover, Hannover 30559, Germany"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Simon H","family":"Parson","sequence":"additional","affiliation":[{"name":"Institute of Medical Sciences, School of Medicine, Medical Sciences and Nutrition, University of Aberdeen, Aberdeen AB25 2ZD, UK"},{"name":"Euan Macdonald Centre for Motor Neurone Disease Research, University of Edinburgh, Edinburgh EH16 4SB, UK"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"286","published-online":{"date-parts":[[2020,6,26]]},"reference":[{"key":"2020081306463193700_ref1","doi-asserted-by":"crossref","first-page":"155","DOI":"10.1016\/0092-8674(95)90460-3","article-title":"Identification and characterization of a spinal muscular atrophy-determining gene","volume":"80","author":"Lefebvre","year":"1995","journal-title":"Cell"},{"key":"2020081306463193700_ref2","doi-asserted-by":"crossref","first-page":"6307","DOI":"10.1073\/pnas.96.11.6307","article-title":"A single nucleotide in the SMN gene regulates splicing and is responsible for spinal muscular atrophy","volume":"96","author":"Lorson","year":"1999","journal-title":"Proc. Natl. Acad. Sci. U. S. A."},{"key":"2020081306463193700_ref3","doi-asserted-by":"crossref","first-page":"333","DOI":"10.1093\/hmg\/9.3.333","article-title":"The human centromeric survival motor neuron gene (SMN2) rescues embryonic lethality in SMN\u2212\/\u2212 mice and results in a mouse with spinal muscular atrophy","volume":"9","author":"Monani","year":"2000","journal-title":"Hum. Mol. Genet."},{"key":"2020081306463193700_ref4","doi-asserted-by":"crossref","first-page":"40","DOI":"10.1016\/j.molmed.2012.11.002","article-title":"Spinal muscular atrophy: going beyond the motor neuron","volume":"19","author":"Hamilton","year":"2013","journal-title":"Trends Mol. Med."},{"key":"2020081306463193700_ref5","doi-asserted-by":"crossref","first-page":"965","DOI":"10.1111\/joa.12793","article-title":"Developmental and degenerative cardiac defects in the Taiwanese mouse model of severe spinal muscular atrophy","volume":"232","author":"Maxwell","year":"2018","journal-title":"J. Anat."},{"key":"2020081306463193700_ref6","doi-asserted-by":"crossref","first-page":"292","DOI":"10.1016\/j.jpeds.2009.01.071","article-title":"Vascular perfusion abnormalities in infants with spinal muscular atrophy","volume":"155","author":"Araujo","year":"2009","journal-title":"J. Pediatr."},{"key":"2020081306463193700_ref7","doi-asserted-by":"crossref","first-page":"81","DOI":"10.1111\/j.1399-0004.1990.tb03553.x","article-title":"Spinal muscular atrophy type I combined with atrial septal defect in three sibs","volume":"38","author":"M\u00f8ller","year":"1990","journal-title":"Clin. 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Genet."},{"key":"2020081306463193700_ref11","first-page":"1","article-title":"Survival motor neuron (SMN) protein is required for normal mouse liver development","volume":"6","author":"Szunyogova","year":"2016","journal-title":"Sci. Rep."},{"key":"2020081306463193700_ref12","doi-asserted-by":"crossref","first-page":"1731","DOI":"10.1016\/S0002-9440(10)63428-1","article-title":"Deletion of murine Smn exon 7 directed to liver leads to severe defect of liver development associated with iron overload","volume":"165","author":"Vitte","year":"2004","journal-title":"Am. J. Pathol."},{"key":"2020081306463193700_ref13","doi-asserted-by":"crossref","first-page":"1519","DOI":"10.1002\/acn3.50855","article-title":"Abnormal fatty acid metabolism is a core component of spinal muscular atrophy","volume":"6","author":"Deguise","year":"2019","journal-title":"Ann. Clin. Transl. 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