Alkaline Ceramidase 3 Deficiency Results in Purkinje Cell Degeneration and Cerebellar Ataxia Due to Dyshomeostasis of Sphingolipids in the Brain
Dyshomeostasis of both ceramides and sphingosine-1-phosphate (S1P) in the brain has been implicated in aging-associated neurodegenerative disorders in humans. However, mechanisms that maintain the homeostasis of these bioactive sphingolipids in the brain remain unclear. Mouse alkaline ceramidase 3 (...
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| creator | Wang, Kai Xu, Ruijuan Schrandt, Jennifer Shah, Prithvi Gong, Yong Z Preston, Chet Wang, Louis Yi, Jae Kyo Lin, Chih-Li Sun, Wei Spyropoulos, Demetri D Rhee, Soyoung Li, Mingsong Zhou, Jie Ge, Shaoyu Zhang, Guofeng Snider, Ashley J Hannun, Yusuf A Obeid, Lina M Mao, Cungui |
| description | Dyshomeostasis of both ceramides and sphingosine-1-phosphate (S1P) in the brain has been implicated in aging-associated neurodegenerative disorders in humans. However, mechanisms that maintain the homeostasis of these bioactive sphingolipids in the brain remain unclear. Mouse alkaline ceramidase 3 (Acer3), which preferentially catalyzes the hydrolysis of C18:1-ceramide, a major unsaturated long-chain ceramide species in the brain, is upregulated with age in the mouse brain. Acer3 knockout causes an age-dependent accumulation of various ceramides and C18:1-monohexosylceramide and abolishes the age-related increase in the levels of sphingosine and S1P in the brain; thereby resulting in Purkinje cell degeneration in the cerebellum and deficits in motor coordination and balance. Our results indicate that Acer3 plays critically protective roles in controlling the homeostasis of various sphingolipids, including ceramides, sphingosine, S1P, and certain complex sphingolipids in the brain and protects Purkinje cells from premature degeneration. |
| doi_str_mv | 10.1371/journal.pgen.1005591 |
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However, mechanisms that maintain the homeostasis of these bioactive sphingolipids in the brain remain unclear. Mouse alkaline ceramidase 3 (Acer3), which preferentially catalyzes the hydrolysis of C18:1-ceramide, a major unsaturated long-chain ceramide species in the brain, is upregulated with age in the mouse brain. Acer3 knockout causes an age-dependent accumulation of various ceramides and C18:1-monohexosylceramide and abolishes the age-related increase in the levels of sphingosine and S1P in the brain; thereby resulting in Purkinje cell degeneration in the cerebellum and deficits in motor coordination and balance. Our results indicate that Acer3 plays critically protective roles in controlling the homeostasis of various sphingolipids, including ceramides, sphingosine, S1P, and certain complex sphingolipids in the brain and protects Purkinje cells from premature degeneration.</description><identifier>ISSN: 1553-7404</identifier><identifier>ISSN: 1553-7390</identifier><identifier>EISSN: 1553-7404</identifier><identifier>DOI: 10.1371/journal.pgen.1005591</identifier><identifier>PMID: 26474409</identifier><language>eng</language><publisher>United States: Public Library of Science</publisher><subject>Age ; Aging ; Aging - genetics ; Aging - metabolism ; Aging - pathology ; Alkaline Ceramidase - genetics ; Animals ; Ataxia ; Behavior ; Brain - metabolism ; Brain - pathology ; Ceramides ; Ceramides - genetics ; Ceramides - metabolism ; Cerebellar Ataxia - genetics ; Cerebellar Ataxia - metabolism ; Cerebellar Ataxia - pathology ; Homeostasis ; Homeostasis - genetics ; Humans ; Liver ; Lungs ; Lysophospholipids - genetics ; Lysophospholipids - metabolism ; Mammals ; Metabolites ; Mice ; Mice, Knockout ; Motor ability ; Phosphates ; Physiological aspects ; Purkinje Cells - metabolism ; Purkinje Cells - pathology ; Sphingolipids - genetics ; Sphingolipids - metabolism ; Sphingosine ; Sphingosine - analogs & derivatives ; Sphingosine - genetics ; Sphingosine - metabolism</subject><ispartof>PLoS genetics, 2015-10, Vol.11 (10), p.e1005591-e1005591</ispartof><rights>COPYRIGHT 2015 Public Library of Science</rights><rights>2015 Public Library of Science. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited: Wang K, Xu R, Schrandt J, Shah P, Gong YZ, Preston C, et al. (2015) Alkaline Ceramidase 3 Deficiency Results in Purkinje Cell Degeneration and Cerebellar Ataxia Due to Dyshomeostasis of Sphingolipids in the Brain. PLoS Genet 11(10): e1005591. doi:10.1371/journal.pgen.1005591</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c698t-5789af5413504a648ee6f118be85a2539f3d9e0634bfb65c700c8b59c26ebe0c3</citedby><cites>FETCH-LOGICAL-c698t-5789af5413504a648ee6f118be85a2539f3d9e0634bfb65c700c8b59c26ebe0c3</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://www.ncbi.nlm.nih.gov/pmc/articles/PMC4608763/pdf/$$EPDF$$P50$$Gpubmedcentral$$Hfree_for_read</linktopdf><linktohtml>$$Uhttps://www.ncbi.nlm.nih.gov/pmc/articles/PMC4608763/$$EHTML$$P50$$Gpubmedcentral$$Hfree_for_read</linktohtml><link.rule.ids>230,314,723,776,780,860,881,2096,2915,23845,27901,27902,53766,53768,79342,79343</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/26474409$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Wang, Kai</creatorcontrib><creatorcontrib>Xu, Ruijuan</creatorcontrib><creatorcontrib>Schrandt, Jennifer</creatorcontrib><creatorcontrib>Shah, Prithvi</creatorcontrib><creatorcontrib>Gong, Yong Z</creatorcontrib><creatorcontrib>Preston, Chet</creatorcontrib><creatorcontrib>Wang, Louis</creatorcontrib><creatorcontrib>Yi, Jae Kyo</creatorcontrib><creatorcontrib>Lin, Chih-Li</creatorcontrib><creatorcontrib>Sun, Wei</creatorcontrib><creatorcontrib>Spyropoulos, Demetri D</creatorcontrib><creatorcontrib>Rhee, Soyoung</creatorcontrib><creatorcontrib>Li, Mingsong</creatorcontrib><creatorcontrib>Zhou, Jie</creatorcontrib><creatorcontrib>Ge, Shaoyu</creatorcontrib><creatorcontrib>Zhang, Guofeng</creatorcontrib><creatorcontrib>Snider, Ashley J</creatorcontrib><creatorcontrib>Hannun, Yusuf A</creatorcontrib><creatorcontrib>Obeid, Lina M</creatorcontrib><creatorcontrib>Mao, Cungui</creatorcontrib><title>Alkaline Ceramidase 3 Deficiency Results in Purkinje Cell Degeneration and Cerebellar Ataxia Due to Dyshomeostasis of Sphingolipids in the Brain</title><title>PLoS genetics</title><addtitle>PLoS Genet</addtitle><description>Dyshomeostasis of both ceramides and sphingosine-1-phosphate (S1P) in the brain has been implicated in aging-associated neurodegenerative disorders in humans. However, mechanisms that maintain the homeostasis of these bioactive sphingolipids in the brain remain unclear. Mouse alkaline ceramidase 3 (Acer3), which preferentially catalyzes the hydrolysis of C18:1-ceramide, a major unsaturated long-chain ceramide species in the brain, is upregulated with age in the mouse brain. Acer3 knockout causes an age-dependent accumulation of various ceramides and C18:1-monohexosylceramide and abolishes the age-related increase in the levels of sphingosine and S1P in the brain; thereby resulting in Purkinje cell degeneration in the cerebellum and deficits in motor coordination and balance. Our results indicate that Acer3 plays critically protective roles in controlling the homeostasis of various sphingolipids, including ceramides, sphingosine, S1P, and certain complex sphingolipids in the brain and protects Purkinje cells from premature degeneration.</description><subject>Age</subject><subject>Aging</subject><subject>Aging - genetics</subject><subject>Aging - metabolism</subject><subject>Aging - pathology</subject><subject>Alkaline Ceramidase - genetics</subject><subject>Animals</subject><subject>Ataxia</subject><subject>Behavior</subject><subject>Brain - metabolism</subject><subject>Brain - pathology</subject><subject>Ceramides</subject><subject>Ceramides - genetics</subject><subject>Ceramides - metabolism</subject><subject>Cerebellar Ataxia - genetics</subject><subject>Cerebellar Ataxia - metabolism</subject><subject>Cerebellar Ataxia - pathology</subject><subject>Homeostasis</subject><subject>Homeostasis - genetics</subject><subject>Humans</subject><subject>Liver</subject><subject>Lungs</subject><subject>Lysophospholipids - genetics</subject><subject>Lysophospholipids - metabolism</subject><subject>Mammals</subject><subject>Metabolites</subject><subject>Mice</subject><subject>Mice, Knockout</subject><subject>Motor ability</subject><subject>Phosphates</subject><subject>Physiological aspects</subject><subject>Purkinje Cells - metabolism</subject><subject>Purkinje Cells - pathology</subject><subject>Sphingolipids - genetics</subject><subject>Sphingolipids - metabolism</subject><subject>Sphingosine</subject><subject>Sphingosine - analogs & derivatives</subject><subject>Sphingosine - genetics</subject><subject>Sphingosine - metabolism</subject><issn>1553-7404</issn><issn>1553-7390</issn><issn>1553-7404</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2015</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><sourceid>DOA</sourceid><recordid>eNqVk11rFDEUhgdRbK3-A9GAIHqxazL5mJmbwtr6UShWWvU2ZDJndrPNJmuSkfZf-JPNdLelC14ogSQkz_uew0lOUTwneEpoRd4t_RCcstP1HNyUYMx5Qx4U-4RzOqkYZg_v7feKJzEuMaa8bqrHxV4pWMUYbvaL3zN7qaxxgI4gqJXpVARE0TH0Rhtw-hqdQxxsisg49HUIl8YtR9bazOTIWZSMd0i5bnSANt-ogGZJXRmFjgdAyaPj67jwK_AxqWgi8j26WC-Mm3tr1qa7sU4LQO-DMu5p8ahXNsKz7XpQfP_44dvR58np2aeTo9npRIumThNe1Y3qOSOUY6YEqwFET0jdQs1VyWnT064BLChr-1ZwXWGs65Y3uhQ5R6zpQfFy47u2PsptMaMkFaWClXnKxMmG6LxaynUwKxWupVdG3hz4MJcqJKMtSNFwWvMOtFY9071QPVZAS-C0JW2DafY63EYb2hV0GlwKyu6Y7t44s5Bz_0sygetKjAZvtgbB_xwgJrkyUY_FduCHMe-SlbypBcnoqw06Vzk143qfHfWIyxmjjBFGytFw-hcqjw5WRnuXP0A-3xG83RFkJsFVmqshRnlycf4f7Jd_Z89-7LKv77ELUDYtorfD-AXjLsg2oA4-xgD9XakJlmP73L64HNtHbtsny17cf6Y70W2_0D8H7BXo</recordid><startdate>20151001</startdate><enddate>20151001</enddate><creator>Wang, Kai</creator><creator>Xu, Ruijuan</creator><creator>Schrandt, Jennifer</creator><creator>Shah, Prithvi</creator><creator>Gong, Yong Z</creator><creator>Preston, Chet</creator><creator>Wang, Louis</creator><creator>Yi, Jae Kyo</creator><creator>Lin, Chih-Li</creator><creator>Sun, Wei</creator><creator>Spyropoulos, Demetri D</creator><creator>Rhee, Soyoung</creator><creator>Li, Mingsong</creator><creator>Zhou, Jie</creator><creator>Ge, Shaoyu</creator><creator>Zhang, Guofeng</creator><creator>Snider, Ashley J</creator><creator>Hannun, Yusuf A</creator><creator>Obeid, Lina M</creator><creator>Mao, Cungui</creator><general>Public Library of Science</general><general>Public Library of Science (PLoS)</general><scope>CGR</scope><scope>CUY</scope><scope>CVF</scope><scope>ECM</scope><scope>EIF</scope><scope>NPM</scope><scope>AAYXX</scope><scope>CITATION</scope><scope>IOV</scope><scope>ISN</scope><scope>ISR</scope><scope>7X8</scope><scope>5PM</scope><scope>DOA</scope></search><sort><creationdate>20151001</creationdate><title>Alkaline Ceramidase 3 Deficiency Results in Purkinje Cell Degeneration and Cerebellar Ataxia Due to Dyshomeostasis of Sphingolipids in the Brain</title><author>Wang, Kai ; Xu, Ruijuan ; Schrandt, Jennifer ; Shah, Prithvi ; Gong, Yong Z ; Preston, Chet ; Wang, Louis ; Yi, Jae Kyo ; Lin, Chih-Li ; Sun, Wei ; Spyropoulos, Demetri D ; Rhee, Soyoung ; Li, Mingsong ; Zhou, Jie ; Ge, Shaoyu ; Zhang, Guofeng ; Snider, Ashley J ; Hannun, Yusuf A ; Obeid, Lina M ; Mao, Cungui</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c698t-5789af5413504a648ee6f118be85a2539f3d9e0634bfb65c700c8b59c26ebe0c3</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2015</creationdate><topic>Age</topic><topic>Aging</topic><topic>Aging - genetics</topic><topic>Aging - metabolism</topic><topic>Aging - pathology</topic><topic>Alkaline Ceramidase - genetics</topic><topic>Animals</topic><topic>Ataxia</topic><topic>Behavior</topic><topic>Brain - metabolism</topic><topic>Brain - pathology</topic><topic>Ceramides</topic><topic>Ceramides - genetics</topic><topic>Ceramides - metabolism</topic><topic>Cerebellar Ataxia - genetics</topic><topic>Cerebellar Ataxia - metabolism</topic><topic>Cerebellar Ataxia - pathology</topic><topic>Homeostasis</topic><topic>Homeostasis - genetics</topic><topic>Humans</topic><topic>Liver</topic><topic>Lungs</topic><topic>Lysophospholipids - genetics</topic><topic>Lysophospholipids - metabolism</topic><topic>Mammals</topic><topic>Metabolites</topic><topic>Mice</topic><topic>Mice, Knockout</topic><topic>Motor ability</topic><topic>Phosphates</topic><topic>Physiological aspects</topic><topic>Purkinje Cells - metabolism</topic><topic>Purkinje Cells - pathology</topic><topic>Sphingolipids - genetics</topic><topic>Sphingolipids - metabolism</topic><topic>Sphingosine</topic><topic>Sphingosine - analogs & derivatives</topic><topic>Sphingosine - genetics</topic><topic>Sphingosine - metabolism</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Wang, Kai</creatorcontrib><creatorcontrib>Xu, Ruijuan</creatorcontrib><creatorcontrib>Schrandt, Jennifer</creatorcontrib><creatorcontrib>Shah, Prithvi</creatorcontrib><creatorcontrib>Gong, Yong Z</creatorcontrib><creatorcontrib>Preston, Chet</creatorcontrib><creatorcontrib>Wang, Louis</creatorcontrib><creatorcontrib>Yi, Jae Kyo</creatorcontrib><creatorcontrib>Lin, Chih-Li</creatorcontrib><creatorcontrib>Sun, Wei</creatorcontrib><creatorcontrib>Spyropoulos, Demetri D</creatorcontrib><creatorcontrib>Rhee, Soyoung</creatorcontrib><creatorcontrib>Li, Mingsong</creatorcontrib><creatorcontrib>Zhou, Jie</creatorcontrib><creatorcontrib>Ge, Shaoyu</creatorcontrib><creatorcontrib>Zhang, Guofeng</creatorcontrib><creatorcontrib>Snider, Ashley J</creatorcontrib><creatorcontrib>Hannun, Yusuf A</creatorcontrib><creatorcontrib>Obeid, Lina M</creatorcontrib><creatorcontrib>Mao, Cungui</creatorcontrib><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>Gale In Context: Opposing Viewpoints</collection><collection>Gale In Context: Canada</collection><collection>Gale In Context: Science</collection><collection>MEDLINE - Academic</collection><collection>PubMed Central (Full Participant titles)</collection><collection>DOAJ Directory of Open Access Journals</collection><jtitle>PLoS genetics</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Wang, Kai</au><au>Xu, Ruijuan</au><au>Schrandt, Jennifer</au><au>Shah, Prithvi</au><au>Gong, Yong Z</au><au>Preston, Chet</au><au>Wang, Louis</au><au>Yi, Jae Kyo</au><au>Lin, Chih-Li</au><au>Sun, Wei</au><au>Spyropoulos, Demetri D</au><au>Rhee, Soyoung</au><au>Li, Mingsong</au><au>Zhou, Jie</au><au>Ge, Shaoyu</au><au>Zhang, Guofeng</au><au>Snider, Ashley J</au><au>Hannun, Yusuf A</au><au>Obeid, Lina M</au><au>Mao, Cungui</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Alkaline Ceramidase 3 Deficiency Results in Purkinje Cell Degeneration and Cerebellar Ataxia Due to Dyshomeostasis of Sphingolipids in the Brain</atitle><jtitle>PLoS genetics</jtitle><addtitle>PLoS Genet</addtitle><date>2015-10-01</date><risdate>2015</risdate><volume>11</volume><issue>10</issue><spage>e1005591</spage><epage>e1005591</epage><pages>e1005591-e1005591</pages><issn>1553-7404</issn><issn>1553-7390</issn><eissn>1553-7404</eissn><abstract>Dyshomeostasis of both ceramides and sphingosine-1-phosphate (S1P) in the brain has been implicated in aging-associated neurodegenerative disorders in humans. However, mechanisms that maintain the homeostasis of these bioactive sphingolipids in the brain remain unclear. Mouse alkaline ceramidase 3 (Acer3), which preferentially catalyzes the hydrolysis of C18:1-ceramide, a major unsaturated long-chain ceramide species in the brain, is upregulated with age in the mouse brain. Acer3 knockout causes an age-dependent accumulation of various ceramides and C18:1-monohexosylceramide and abolishes the age-related increase in the levels of sphingosine and S1P in the brain; thereby resulting in Purkinje cell degeneration in the cerebellum and deficits in motor coordination and balance. Our results indicate that Acer3 plays critically protective roles in controlling the homeostasis of various sphingolipids, including ceramides, sphingosine, S1P, and certain complex sphingolipids in the brain and protects Purkinje cells from premature degeneration.</abstract><cop>United States</cop><pub>Public Library of Science</pub><pmid>26474409</pmid><doi>10.1371/journal.pgen.1005591</doi><oa>free_for_read</oa></addata></record> |
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| subjects | Age Aging Aging - genetics Aging - metabolism Aging - pathology Alkaline Ceramidase - genetics Animals Ataxia Behavior Brain - metabolism Brain - pathology Ceramides Ceramides - genetics Ceramides - metabolism Cerebellar Ataxia - genetics Cerebellar Ataxia - metabolism Cerebellar Ataxia - pathology Homeostasis Homeostasis - genetics Humans Liver Lungs Lysophospholipids - genetics Lysophospholipids - metabolism Mammals Metabolites Mice Mice, Knockout Motor ability Phosphates Physiological aspects Purkinje Cells - metabolism Purkinje Cells - pathology Sphingolipids - genetics Sphingolipids - metabolism Sphingosine Sphingosine - analogs & derivatives Sphingosine - genetics Sphingosine - metabolism |
| title | Alkaline Ceramidase 3 Deficiency Results in Purkinje Cell Degeneration and Cerebellar Ataxia Due to Dyshomeostasis of Sphingolipids in the Brain |
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