{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,9,26]],"date-time":"2025-09-26T00:14:42Z","timestamp":1758845682195,"version":"3.44.0"},"reference-count":48,"publisher":"The Company of Biologists","issue":"18","license":[{"start":{"date-parts":[[2025,9,15]],"date-time":"2025-09-15T00:00:00Z","timestamp":1757894400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/www.biologists.com\/user-licence-1-2\/"}],"funder":[{"DOI":"10.13039\/501100001809","name":"National Natural Science Foundation of China","doi-asserted-by":"crossref","award":["32271157","32293204"],"award-info":[{"award-number":["32271157","32293204"]}],"id":[{"id":"10.13039\/501100001809","id-type":"DOI","asserted-by":"crossref"}]},{"DOI":"10.13039\/501100002855","name":"Ministry of Science and Technology of the People's Republic of China","doi-asserted-by":"crossref","award":["2019YFA0802104"],"award-info":[{"award-number":["2019YFA0802104"]}],"id":[{"id":"10.13039\/501100002855","id-type":"DOI","asserted-by":"crossref"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2025,9,15]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n               <jats:p>Renin is mainly stored in the renin granules (RGs) of juxtaglomerular cells (JGCs). RGs are a type of lysosome-related organelle (LRO), and knowledge on the underlying mechanism of RG biogenesis is limited. Hermansky\u2013Pudlak syndrome (HPS) is characterized by multiple LRO defects. Whether there are RG defects in HPS is unknown. Using different mouse models of HPS, we found that the active renin content was reduced in the kidneys of buff (bf) mice, which carry a homozygous point mutation (D251E) of Vps33a, a subunit of the HOPS complex. We observed that bf mice exhibited smaller RGs than did heterozygous mice. Knockdown of Vps33a, Snap23, Stx11 and Vamp8 in As4.1 cells impaired the biogenesis of RGs. Interaction between mutant Vps33aD251E and Stx11 was enhanced. We concluded that Vps33a is likely to be involved in the fusion process during RG biogenesis, which is regulated by the SNARE complex (Snap23\u2013Stx11\u2013Vamp8). The enhanced interaction of Vps33aD251E\u2013Stx11 might impair the function of the SNARE complex, which is required for RG biogenesis. This highlights that defects in RG biogenesis can lead to hyporeninemia.<\/jats:p>","DOI":"10.1242\/jcs.263767","type":"journal-article","created":{"date-parts":[[2025,8,26]],"date-time":"2025-08-26T08:39:47Z","timestamp":1756197587000},"source":"Crossref","is-referenced-by-count":0,"title":["Impaired biogenesis of renin granules in juxtaglomerular cells of Vps33a (D251E) mutant mice"],"prefix":"10.1242","volume":"138","author":[{"given":"Huipeng","family":"Wang","sequence":"first","affiliation":[{"name":"Beijing Pediatric Research Institute; MOE Key Laboratory of Major Diseases in Children; Genetics and Birth Defects Control Center, National Center for Children's Health; Beijing Children's Hospital, Capital Medical University 1 Laboratory for Genetics of Birth Defects , , Beijing 100045 ,","place":["China"]},{"name":"Institute of Children's Health, Henan Academy of Medical Sciences 2 , Zhengzhou 451162 ,","place":["China"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2275-1927","authenticated-orcid":false,"given":"Zhenhua","family":"Hao","sequence":"additional","affiliation":[{"name":"Beijing Pediatric Research Institute; MOE Key Laboratory of Major Diseases in Children; Genetics and Birth Defects Control Center, National Center for Children's Health; Beijing Children's Hospital, Capital Medical University 1 Laboratory for Genetics of Birth Defects , , Beijing 100045 ,","place":["China"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0248-5510","authenticated-orcid":false,"given":"Wei","family":"Li","sequence":"additional","affiliation":[{"name":"Beijing Pediatric Research Institute; MOE Key Laboratory of Major Diseases in Children; Genetics and Birth Defects Control Center, National Center for Children's Health; Beijing Children's Hospital, Capital Medical University 1 Laboratory for Genetics of Birth Defects , , Beijing 100045 ,","place":["China"]},{"name":"Institute of Children's Health, Henan Academy of Medical Sciences 2 , Zhengzhou 451162 ,","place":["China"]}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"237","published-online":{"date-parts":[[2025,9,25]]},"reference":[{"key":"2025092506042670500_JCS263767C1","doi-asserted-by":"publisher","first-page":"F26","DOI":"10.1152\/ajprenal.1998.274.1.F26","article-title":"Distinct localization of renin and GLUT-4 in juxtaglomerular cells of mouse kidney","volume":"274","author":"Anderson","year":"1998","journal-title":"Am. 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