Assessment of dysmyelination with RAFFn MRI: application to murine MPS I
Type I mucopolysaccharidosis (MPS I) is an autosomal recessive lysosomal storage disorder with neurological features. Humans and laboratory animals with MPS I exhibit various white matter abnormalities involving the corpus callosum and other regions. In this study, we first validated a novel MRI tec...
Gespeichert in:
| Veröffentlicht in: | PloS one 2015-02, Vol.10 (2), p.e0116788-e0116788 |
|---|---|
| Hauptverfasser: | , , , , , , , , , , |
| Format: | Artikel |
| Sprache: | eng |
| Schlagworte: | |
| Online-Zugang: | Volltext |
| Tags: |
Tag hinzufügen
Keine Tags, Fügen Sie den ersten Tag hinzu!
|
| container_end_page | e0116788 |
|---|---|
| container_issue | 2 |
| container_start_page | e0116788 |
| container_title | PloS one |
| container_volume | 10 |
| creator | Satzer, David DiBartolomeo, Christina Ritchie, Michael M Storino, Christine Liimatainen, Timo Hakkarainen, Hanne Idiyatullin, Djaudat Mangia, Silvia Michaeli, Shalom Parr, Ann M Low, Walter C |
| description | Type I mucopolysaccharidosis (MPS I) is an autosomal recessive lysosomal storage disorder with neurological features. Humans and laboratory animals with MPS I exhibit various white matter abnormalities involving the corpus callosum and other regions. In this study, we first validated a novel MRI technique, entitled Relaxation Along a Fictitious Field in the rotating frame of rank n (RAFFn), as a measure of myelination and dysmyelination in mice. We then examined differences between MPS I mice and heterozygotes using RAFF5 and histology. RAFF5 (i.e., RAFFn with n = 5) relaxation time constants were highly correlated with histological myelin density (R2 = 0.68, P |
| doi_str_mv | 10.1371/journal.pone.0116788 |
| format | Article |
| fullrecord | <record><control><sourceid>gale_plos_</sourceid><recordid>TN_cdi_plos_journals_1656698792</recordid><sourceformat>XML</sourceformat><sourcesystem>PC</sourcesystem><galeid>A422026196</galeid><doaj_id>oai_doaj_org_article_a539876289f14389a72d1ce954280534</doaj_id><sourcerecordid>A422026196</sourcerecordid><originalsourceid>FETCH-LOGICAL-c692t-b97af39dffceed75d449e21a53e6b5a4acadbae1ddadb761818b280c22f85b423</originalsourceid><addsrcrecordid>eNqNkl9v0zAUxSMEYmPwDRBEQkLw0BL_iWPzgFRNlFXaNNQBr5bj3LSunLiLE6DfHodkU4P2wJMt-3fuvT4-UfQSJXNEMvRh57qmVna-dzXME4RYxvmj6BQJgmcMJ-Tx0f4keub9LklSwhl7Gp3glPEECXYaXSy8B-8rqNvYlXFx8NUBrKlVa1wd_zLtNl4vlss6vlqvPsZqv7dGD3eti6uuMTXEV19v4tXz6EmprIcX43oWfV9-_nZ-Mbu8_rI6X1zONBO4neUiUyURRVlqgCJLC0oFYKRSAixPFVVaFbkCVBRhzRjiiOeYJxrjkqc5xeQsej3U3Vvn5WiCl4iljAmeiZ5YDUTh1E7uG1Op5iCdMvLvgWs2UjWt0RZkaBs0DHNRIkq4UBkukAaR0tAzJTTU-jR26_IKCh1sapSdFJ3e1GYrN-6npITQFPXDvBsLNO62A9_KyngN1qoaXDfMnVCS8R598w_68OtGaqPCA0xdutBX90XlgmKcYBb-NVDzB6jeXKiMDokpTTifCN5PBIFp4Xe7UZ33cnWz_n_2-seUfXvEbkHZduud7foI-SlIB1A3zvsGynuTUSL7wN-5IfvAyzHwQfbq-IPuRXcJJ38A95D5Pg</addsrcrecordid><sourcetype>Open Website</sourcetype><iscdi>true</iscdi><recordtype>article</recordtype><pqid>1656698792</pqid></control><display><type>article</type><title>Assessment of dysmyelination with RAFFn MRI: application to murine MPS I</title><source>MEDLINE</source><source>DOAJ Directory of Open Access Journals</source><source>Public Library of Science (PLoS) Journals Open Access</source><source>EZB-FREE-00999 freely available EZB journals</source><source>PubMed Central</source><source>Free Full-Text Journals in Chemistry</source><creator>Satzer, David ; DiBartolomeo, Christina ; Ritchie, Michael M ; Storino, Christine ; Liimatainen, Timo ; Hakkarainen, Hanne ; Idiyatullin, Djaudat ; Mangia, Silvia ; Michaeli, Shalom ; Parr, Ann M ; Low, Walter C</creator><contributor>Cercignani, Mara</contributor><creatorcontrib>Satzer, David ; DiBartolomeo, Christina ; Ritchie, Michael M ; Storino, Christine ; Liimatainen, Timo ; Hakkarainen, Hanne ; Idiyatullin, Djaudat ; Mangia, Silvia ; Michaeli, Shalom ; Parr, Ann M ; Low, Walter C ; Cercignani, Mara</creatorcontrib><description>Type I mucopolysaccharidosis (MPS I) is an autosomal recessive lysosomal storage disorder with neurological features. Humans and laboratory animals with MPS I exhibit various white matter abnormalities involving the corpus callosum and other regions. In this study, we first validated a novel MRI technique, entitled Relaxation Along a Fictitious Field in the rotating frame of rank n (RAFFn), as a measure of myelination and dysmyelination in mice. We then examined differences between MPS I mice and heterozygotes using RAFF5 and histology. RAFF5 (i.e., RAFFn with n = 5) relaxation time constants were highly correlated with histological myelin density (R2 = 0.68, P<0.001), and RAFF5 clearly distinguished between the hypomyelinated and dysmyelinated shiverer mouse and the wild-type mouse. Bloch-McConnell theoretical analysis revealed slower exchange correlation times and smaller exchange-induced relaxation rate constants for RAFF4 and RAFF5 compared to RAFF1-3, T1ρ, and T2ρ. These data suggest that RAFF5 may assess methylene protons in myelin lipids and proteins, though other mechanisms (e.g. detection of myelin-bound water) may also explain the sensitivity of RAFF5 to myelin. In MPS I mice, mean RAFF5 relaxation time constants were significantly larger for the striatum (P = 0.004) and internal capsule (P = 0.039), and marginally larger for the fornix (P = 0.15). Histological assessment revealed no differences between MPS I mice and heterozygotes in myelin density or corpus callosum thickness. Taken together, these findings support subtle dysmyelination in the brains of mice with MPS I. Dysmyelination may result from myelin lipid abnormalities caused by the absence of α-L-iduronidase. Our findings may help to explain locomotor and cognitive deficits seen in mice with MPS I.</description><identifier>ISSN: 1932-6203</identifier><identifier>EISSN: 1932-6203</identifier><identifier>DOI: 10.1371/journal.pone.0116788</identifier><identifier>PMID: 25680196</identifier><language>eng</language><publisher>United States: Public Library of Science</publisher><subject>Abnormalities ; Animals ; Bound water ; Brain ; Brain research ; Cognitive ability ; Corpus callosum ; Corpus Callosum - pathology ; Correlation analysis ; Data processing ; Female ; Fornix ; Hereditary diseases ; Heterozygote ; Heterozygotes ; Histology ; Iduronidase - genetics ; L-Iduronidase ; Laboratory animals ; Laboratory tests ; Lipids ; Magnetic resonance imaging ; Magnetic Resonance Imaging - methods ; Male ; Mice ; Mucopolysaccharidosis ; Mucopolysaccharidosis I - diagnosis ; Mucopolysaccharidosis I - genetics ; Mucopolysaccharidosis I - pathology ; Mucopolysaccharidosis I - physiopathology ; Myelin ; Myelin Sheath - physiology ; Myelination ; Neostriatum ; Nervous system ; Neurosurgery ; Organ Size ; Proteins ; Protons ; Rate constants ; Relaxation time ; Rotation ; Substantia alba ; Theoretical analysis ; Time Factors</subject><ispartof>PloS one, 2015-02, Vol.10 (2), p.e0116788-e0116788</ispartof><rights>COPYRIGHT 2015 Public Library of Science</rights><rights>2015 Satzer et al. This is an open access article distributed under the terms of the Creative Commons Attribution License: http://creativecommons.org/licenses/by/4.0/ (the “License”), which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited Notwithstanding the ProQuest Terms and Conditions, you may use this content in accordance with the terms of the License.</rights><rights>2015 Satzer et al 2015 Satzer et al</rights><lds50>peer_reviewed</lds50><oa>free_for_read</oa><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c692t-b97af39dffceed75d449e21a53e6b5a4acadbae1ddadb761818b280c22f85b423</citedby><cites>FETCH-LOGICAL-c692t-b97af39dffceed75d449e21a53e6b5a4acadbae1ddadb761818b280c22f85b423</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/PMC4334512/pdf/$$EPDF$$P50$$Gpubmedcentral$$Hfree_for_read</linktopdf><linktohtml>$$Uhttps://www.ncbi.nlm.nih.gov/pmc/articles/PMC4334512/$$EHTML$$P50$$Gpubmedcentral$$Hfree_for_read</linktohtml><link.rule.ids>230,314,727,780,784,864,885,2100,2926,23865,27923,27924,53790,53792,79371,79372</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/25680196$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><contributor>Cercignani, Mara</contributor><creatorcontrib>Satzer, David</creatorcontrib><creatorcontrib>DiBartolomeo, Christina</creatorcontrib><creatorcontrib>Ritchie, Michael M</creatorcontrib><creatorcontrib>Storino, Christine</creatorcontrib><creatorcontrib>Liimatainen, Timo</creatorcontrib><creatorcontrib>Hakkarainen, Hanne</creatorcontrib><creatorcontrib>Idiyatullin, Djaudat</creatorcontrib><creatorcontrib>Mangia, Silvia</creatorcontrib><creatorcontrib>Michaeli, Shalom</creatorcontrib><creatorcontrib>Parr, Ann M</creatorcontrib><creatorcontrib>Low, Walter C</creatorcontrib><title>Assessment of dysmyelination with RAFFn MRI: application to murine MPS I</title><title>PloS one</title><addtitle>PLoS One</addtitle><description>Type I mucopolysaccharidosis (MPS I) is an autosomal recessive lysosomal storage disorder with neurological features. Humans and laboratory animals with MPS I exhibit various white matter abnormalities involving the corpus callosum and other regions. In this study, we first validated a novel MRI technique, entitled Relaxation Along a Fictitious Field in the rotating frame of rank n (RAFFn), as a measure of myelination and dysmyelination in mice. We then examined differences between MPS I mice and heterozygotes using RAFF5 and histology. RAFF5 (i.e., RAFFn with n = 5) relaxation time constants were highly correlated with histological myelin density (R2 = 0.68, P<0.001), and RAFF5 clearly distinguished between the hypomyelinated and dysmyelinated shiverer mouse and the wild-type mouse. Bloch-McConnell theoretical analysis revealed slower exchange correlation times and smaller exchange-induced relaxation rate constants for RAFF4 and RAFF5 compared to RAFF1-3, T1ρ, and T2ρ. These data suggest that RAFF5 may assess methylene protons in myelin lipids and proteins, though other mechanisms (e.g. detection of myelin-bound water) may also explain the sensitivity of RAFF5 to myelin. In MPS I mice, mean RAFF5 relaxation time constants were significantly larger for the striatum (P = 0.004) and internal capsule (P = 0.039), and marginally larger for the fornix (P = 0.15). Histological assessment revealed no differences between MPS I mice and heterozygotes in myelin density or corpus callosum thickness. Taken together, these findings support subtle dysmyelination in the brains of mice with MPS I. Dysmyelination may result from myelin lipid abnormalities caused by the absence of α-L-iduronidase. Our findings may help to explain locomotor and cognitive deficits seen in mice with MPS I.</description><subject>Abnormalities</subject><subject>Animals</subject><subject>Bound water</subject><subject>Brain</subject><subject>Brain research</subject><subject>Cognitive ability</subject><subject>Corpus callosum</subject><subject>Corpus Callosum - pathology</subject><subject>Correlation analysis</subject><subject>Data processing</subject><subject>Female</subject><subject>Fornix</subject><subject>Hereditary diseases</subject><subject>Heterozygote</subject><subject>Heterozygotes</subject><subject>Histology</subject><subject>Iduronidase - genetics</subject><subject>L-Iduronidase</subject><subject>Laboratory animals</subject><subject>Laboratory tests</subject><subject>Lipids</subject><subject>Magnetic resonance imaging</subject><subject>Magnetic Resonance Imaging - methods</subject><subject>Male</subject><subject>Mice</subject><subject>Mucopolysaccharidosis</subject><subject>Mucopolysaccharidosis I - diagnosis</subject><subject>Mucopolysaccharidosis I - genetics</subject><subject>Mucopolysaccharidosis I - pathology</subject><subject>Mucopolysaccharidosis I - physiopathology</subject><subject>Myelin</subject><subject>Myelin Sheath - physiology</subject><subject>Myelination</subject><subject>Neostriatum</subject><subject>Nervous system</subject><subject>Neurosurgery</subject><subject>Organ Size</subject><subject>Proteins</subject><subject>Protons</subject><subject>Rate constants</subject><subject>Relaxation time</subject><subject>Rotation</subject><subject>Substantia alba</subject><subject>Theoretical analysis</subject><subject>Time Factors</subject><issn>1932-6203</issn><issn>1932-6203</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2015</creationdate><recordtype>article</recordtype><sourceid>EIF</sourceid><sourceid>ABUWG</sourceid><sourceid>AFKRA</sourceid><sourceid>AZQEC</sourceid><sourceid>BENPR</sourceid><sourceid>CCPQU</sourceid><sourceid>DWQXO</sourceid><sourceid>GNUQQ</sourceid><sourceid>DOA</sourceid><recordid>eNqNkl9v0zAUxSMEYmPwDRBEQkLw0BL_iWPzgFRNlFXaNNQBr5bj3LSunLiLE6DfHodkU4P2wJMt-3fuvT4-UfQSJXNEMvRh57qmVna-dzXME4RYxvmj6BQJgmcMJ-Tx0f4keub9LklSwhl7Gp3glPEECXYaXSy8B-8rqNvYlXFx8NUBrKlVa1wd_zLtNl4vlss6vlqvPsZqv7dGD3eti6uuMTXEV19v4tXz6EmprIcX43oWfV9-_nZ-Mbu8_rI6X1zONBO4neUiUyURRVlqgCJLC0oFYKRSAixPFVVaFbkCVBRhzRjiiOeYJxrjkqc5xeQsej3U3Vvn5WiCl4iljAmeiZ5YDUTh1E7uG1Op5iCdMvLvgWs2UjWt0RZkaBs0DHNRIkq4UBkukAaR0tAzJTTU-jR26_IKCh1sapSdFJ3e1GYrN-6npITQFPXDvBsLNO62A9_KyngN1qoaXDfMnVCS8R598w_68OtGaqPCA0xdutBX90XlgmKcYBb-NVDzB6jeXKiMDokpTTifCN5PBIFp4Xe7UZ33cnWz_n_2-seUfXvEbkHZduud7foI-SlIB1A3zvsGynuTUSL7wN-5IfvAyzHwQfbq-IPuRXcJJ38A95D5Pg</recordid><startdate>20150213</startdate><enddate>20150213</enddate><creator>Satzer, David</creator><creator>DiBartolomeo, Christina</creator><creator>Ritchie, Michael M</creator><creator>Storino, Christine</creator><creator>Liimatainen, Timo</creator><creator>Hakkarainen, Hanne</creator><creator>Idiyatullin, Djaudat</creator><creator>Mangia, Silvia</creator><creator>Michaeli, Shalom</creator><creator>Parr, Ann M</creator><creator>Low, Walter C</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>ISR</scope><scope>3V.</scope><scope>7QG</scope><scope>7QL</scope><scope>7QO</scope><scope>7RV</scope><scope>7SN</scope><scope>7SS</scope><scope>7T5</scope><scope>7TG</scope><scope>7TM</scope><scope>7U9</scope><scope>7X2</scope><scope>7X7</scope><scope>7XB</scope><scope>88E</scope><scope>8AO</scope><scope>8C1</scope><scope>8FD</scope><scope>8FE</scope><scope>8FG</scope><scope>8FH</scope><scope>8FI</scope><scope>8FJ</scope><scope>8FK</scope><scope>ABJCF</scope><scope>ABUWG</scope><scope>AEUYN</scope><scope>AFKRA</scope><scope>ARAPS</scope><scope>ATCPS</scope><scope>AZQEC</scope><scope>BBNVY</scope><scope>BENPR</scope><scope>BGLVJ</scope><scope>BHPHI</scope><scope>C1K</scope><scope>CCPQU</scope><scope>D1I</scope><scope>DWQXO</scope><scope>FR3</scope><scope>FYUFA</scope><scope>GHDGH</scope><scope>GNUQQ</scope><scope>H94</scope><scope>HCIFZ</scope><scope>K9.</scope><scope>KB.</scope><scope>KB0</scope><scope>KL.</scope><scope>L6V</scope><scope>LK8</scope><scope>M0K</scope><scope>M0S</scope><scope>M1P</scope><scope>M7N</scope><scope>M7P</scope><scope>M7S</scope><scope>NAPCQ</scope><scope>P5Z</scope><scope>P62</scope><scope>P64</scope><scope>PATMY</scope><scope>PDBOC</scope><scope>PIMPY</scope><scope>PQEST</scope><scope>PQQKQ</scope><scope>PQUKI</scope><scope>PRINS</scope><scope>PTHSS</scope><scope>PYCSY</scope><scope>RC3</scope><scope>7X8</scope><scope>5PM</scope><scope>DOA</scope></search><sort><creationdate>20150213</creationdate><title>Assessment of dysmyelination with RAFFn MRI: application to murine MPS I</title><author>Satzer, David ; DiBartolomeo, Christina ; Ritchie, Michael M ; Storino, Christine ; Liimatainen, Timo ; Hakkarainen, Hanne ; Idiyatullin, Djaudat ; Mangia, Silvia ; Michaeli, Shalom ; Parr, Ann M ; Low, Walter C</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c692t-b97af39dffceed75d449e21a53e6b5a4acadbae1ddadb761818b280c22f85b423</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2015</creationdate><topic>Abnormalities</topic><topic>Animals</topic><topic>Bound water</topic><topic>Brain</topic><topic>Brain research</topic><topic>Cognitive ability</topic><topic>Corpus callosum</topic><topic>Corpus Callosum - pathology</topic><topic>Correlation analysis</topic><topic>Data processing</topic><topic>Female</topic><topic>Fornix</topic><topic>Hereditary diseases</topic><topic>Heterozygote</topic><topic>Heterozygotes</topic><topic>Histology</topic><topic>Iduronidase - genetics</topic><topic>L-Iduronidase</topic><topic>Laboratory animals</topic><topic>Laboratory tests</topic><topic>Lipids</topic><topic>Magnetic resonance imaging</topic><topic>Magnetic Resonance Imaging - methods</topic><topic>Male</topic><topic>Mice</topic><topic>Mucopolysaccharidosis</topic><topic>Mucopolysaccharidosis I - diagnosis</topic><topic>Mucopolysaccharidosis I - genetics</topic><topic>Mucopolysaccharidosis I - pathology</topic><topic>Mucopolysaccharidosis I - physiopathology</topic><topic>Myelin</topic><topic>Myelin Sheath - physiology</topic><topic>Myelination</topic><topic>Neostriatum</topic><topic>Nervous system</topic><topic>Neurosurgery</topic><topic>Organ Size</topic><topic>Proteins</topic><topic>Protons</topic><topic>Rate constants</topic><topic>Relaxation time</topic><topic>Rotation</topic><topic>Substantia alba</topic><topic>Theoretical analysis</topic><topic>Time Factors</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Satzer, David</creatorcontrib><creatorcontrib>DiBartolomeo, Christina</creatorcontrib><creatorcontrib>Ritchie, Michael M</creatorcontrib><creatorcontrib>Storino, Christine</creatorcontrib><creatorcontrib>Liimatainen, Timo</creatorcontrib><creatorcontrib>Hakkarainen, Hanne</creatorcontrib><creatorcontrib>Idiyatullin, Djaudat</creatorcontrib><creatorcontrib>Mangia, Silvia</creatorcontrib><creatorcontrib>Michaeli, Shalom</creatorcontrib><creatorcontrib>Parr, Ann M</creatorcontrib><creatorcontrib>Low, Walter C</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: Science</collection><collection>ProQuest Central (Corporate)</collection><collection>Animal Behavior Abstracts</collection><collection>Bacteriology Abstracts (Microbiology B)</collection><collection>Biotechnology Research Abstracts</collection><collection>Proquest Nursing & Allied Health Source</collection><collection>Ecology Abstracts</collection><collection>Entomology Abstracts (Full archive)</collection><collection>Immunology Abstracts</collection><collection>Meteorological & Geoastrophysical Abstracts</collection><collection>Nucleic Acids Abstracts</collection><collection>Virology and AIDS Abstracts</collection><collection>Agricultural Science Collection</collection><collection>Health & Medical Collection</collection><collection>ProQuest Central (purchase pre-March 2016)</collection><collection>Medical Database (Alumni Edition)</collection><collection>ProQuest Pharma Collection</collection><collection>Public Health Database</collection><collection>Technology Research Database</collection><collection>ProQuest SciTech Collection</collection><collection>ProQuest Technology Collection</collection><collection>ProQuest Natural Science Collection</collection><collection>Hospital Premium Collection</collection><collection>Hospital Premium Collection (Alumni Edition)</collection><collection>ProQuest Central (Alumni) (purchase pre-March 2016)</collection><collection>Materials Science & Engineering Collection</collection><collection>ProQuest Central (Alumni Edition)</collection><collection>ProQuest One Sustainability</collection><collection>ProQuest Central UK/Ireland</collection><collection>Advanced Technologies & Aerospace Collection</collection><collection>Agricultural & Environmental Science Collection</collection><collection>ProQuest Central Essentials</collection><collection>Biological Science Collection</collection><collection>ProQuest Central</collection><collection>Technology Collection</collection><collection>Natural Science Collection</collection><collection>Environmental Sciences and Pollution Management</collection><collection>ProQuest One Community College</collection><collection>ProQuest Materials Science Collection</collection><collection>ProQuest Central Korea</collection><collection>Engineering Research Database</collection><collection>Health Research Premium Collection</collection><collection>Health Research Premium Collection (Alumni)</collection><collection>ProQuest Central Student</collection><collection>AIDS and Cancer Research Abstracts</collection><collection>SciTech Premium Collection</collection><collection>ProQuest Health & Medical Complete (Alumni)</collection><collection>Materials Science Database</collection><collection>Nursing & Allied Health Database (Alumni Edition)</collection><collection>Meteorological & Geoastrophysical Abstracts - Academic</collection><collection>ProQuest Engineering Collection</collection><collection>ProQuest Biological Science Collection</collection><collection>Agricultural Science Database</collection><collection>Health & Medical Collection (Alumni Edition)</collection><collection>Medical Database</collection><collection>Algology Mycology and Protozoology Abstracts (Microbiology C)</collection><collection>Biological Science Database</collection><collection>Engineering Database</collection><collection>Nursing & Allied Health Premium</collection><collection>Advanced Technologies & Aerospace Database</collection><collection>ProQuest Advanced Technologies & Aerospace Collection</collection><collection>Biotechnology and BioEngineering Abstracts</collection><collection>Environmental Science Database</collection><collection>Materials Science Collection</collection><collection>Publicly Available Content Database</collection><collection>ProQuest One Academic Eastern Edition (DO NOT USE)</collection><collection>ProQuest One Academic</collection><collection>ProQuest One Academic UKI Edition</collection><collection>ProQuest Central China</collection><collection>Engineering Collection</collection><collection>Environmental Science Collection</collection><collection>Genetics Abstracts</collection><collection>MEDLINE - Academic</collection><collection>PubMed Central (Full Participant titles)</collection><collection>DOAJ Directory of Open Access Journals</collection><jtitle>PloS one</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Satzer, David</au><au>DiBartolomeo, Christina</au><au>Ritchie, Michael M</au><au>Storino, Christine</au><au>Liimatainen, Timo</au><au>Hakkarainen, Hanne</au><au>Idiyatullin, Djaudat</au><au>Mangia, Silvia</au><au>Michaeli, Shalom</au><au>Parr, Ann M</au><au>Low, Walter C</au><au>Cercignani, Mara</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Assessment of dysmyelination with RAFFn MRI: application to murine MPS I</atitle><jtitle>PloS one</jtitle><addtitle>PLoS One</addtitle><date>2015-02-13</date><risdate>2015</risdate><volume>10</volume><issue>2</issue><spage>e0116788</spage><epage>e0116788</epage><pages>e0116788-e0116788</pages><issn>1932-6203</issn><eissn>1932-6203</eissn><abstract>Type I mucopolysaccharidosis (MPS I) is an autosomal recessive lysosomal storage disorder with neurological features. Humans and laboratory animals with MPS I exhibit various white matter abnormalities involving the corpus callosum and other regions. In this study, we first validated a novel MRI technique, entitled Relaxation Along a Fictitious Field in the rotating frame of rank n (RAFFn), as a measure of myelination and dysmyelination in mice. We then examined differences between MPS I mice and heterozygotes using RAFF5 and histology. RAFF5 (i.e., RAFFn with n = 5) relaxation time constants were highly correlated with histological myelin density (R2 = 0.68, P<0.001), and RAFF5 clearly distinguished between the hypomyelinated and dysmyelinated shiverer mouse and the wild-type mouse. Bloch-McConnell theoretical analysis revealed slower exchange correlation times and smaller exchange-induced relaxation rate constants for RAFF4 and RAFF5 compared to RAFF1-3, T1ρ, and T2ρ. These data suggest that RAFF5 may assess methylene protons in myelin lipids and proteins, though other mechanisms (e.g. detection of myelin-bound water) may also explain the sensitivity of RAFF5 to myelin. In MPS I mice, mean RAFF5 relaxation time constants were significantly larger for the striatum (P = 0.004) and internal capsule (P = 0.039), and marginally larger for the fornix (P = 0.15). Histological assessment revealed no differences between MPS I mice and heterozygotes in myelin density or corpus callosum thickness. Taken together, these findings support subtle dysmyelination in the brains of mice with MPS I. Dysmyelination may result from myelin lipid abnormalities caused by the absence of α-L-iduronidase. Our findings may help to explain locomotor and cognitive deficits seen in mice with MPS I.</abstract><cop>United States</cop><pub>Public Library of Science</pub><pmid>25680196</pmid><doi>10.1371/journal.pone.0116788</doi><oa>free_for_read</oa></addata></record> |
| fulltext | fulltext |
| identifier | ISSN: 1932-6203 |
| ispartof | PloS one, 2015-02, Vol.10 (2), p.e0116788-e0116788 |
| issn | 1932-6203 1932-6203 |
| language | eng |
| recordid | cdi_plos_journals_1656698792 |
| source | MEDLINE; DOAJ Directory of Open Access Journals; Public Library of Science (PLoS) Journals Open Access; EZB-FREE-00999 freely available EZB journals; PubMed Central; Free Full-Text Journals in Chemistry |
| subjects | Abnormalities Animals Bound water Brain Brain research Cognitive ability Corpus callosum Corpus Callosum - pathology Correlation analysis Data processing Female Fornix Hereditary diseases Heterozygote Heterozygotes Histology Iduronidase - genetics L-Iduronidase Laboratory animals Laboratory tests Lipids Magnetic resonance imaging Magnetic Resonance Imaging - methods Male Mice Mucopolysaccharidosis Mucopolysaccharidosis I - diagnosis Mucopolysaccharidosis I - genetics Mucopolysaccharidosis I - pathology Mucopolysaccharidosis I - physiopathology Myelin Myelin Sheath - physiology Myelination Neostriatum Nervous system Neurosurgery Organ Size Proteins Protons Rate constants Relaxation time Rotation Substantia alba Theoretical analysis Time Factors |
| title | Assessment of dysmyelination with RAFFn MRI: application to murine MPS I |
| url | https://sfx.bib-bvb.de/sfx_tum?ctx_ver=Z39.88-2004&ctx_enc=info:ofi/enc:UTF-8&ctx_tim=2025-01-10T22%3A06%3A31IST&url_ver=Z39.88-2004&url_ctx_fmt=infofi/fmt:kev:mtx:ctx&rfr_id=info:sid/primo.exlibrisgroup.com:primo3-Article-gale_plos_&rft_val_fmt=info:ofi/fmt:kev:mtx:journal&rft.genre=article&rft.atitle=Assessment%20of%20dysmyelination%20with%20RAFFn%20MRI:%20application%20to%20murine%20MPS%20I&rft.jtitle=PloS%20one&rft.au=Satzer,%20David&rft.date=2015-02-13&rft.volume=10&rft.issue=2&rft.spage=e0116788&rft.epage=e0116788&rft.pages=e0116788-e0116788&rft.issn=1932-6203&rft.eissn=1932-6203&rft_id=info:doi/10.1371/journal.pone.0116788&rft_dat=%3Cgale_plos_%3EA422026196%3C/gale_plos_%3E%3Curl%3E%3C/url%3E&disable_directlink=true&sfx.directlink=off&sfx.report_link=0&rft_id=info:oai/&rft_pqid=1656698792&rft_id=info:pmid/25680196&rft_galeid=A422026196&rft_doaj_id=oai_doaj_org_article_a539876289f14389a72d1ce954280534&rfr_iscdi=true |