Plaque-type deposition of prion protein in the damaged white matter of sporadic Creutzfeldt-Jakob disease MM1 patients
Plaque-type deposition of prion protein (PrP) in the brain has been extremely rare in sporadic Creutzfeldt-Jakob disease patients with methionine homozygosity at polymorphic codon 129 of the PrP gene and type 1 abnormal isoform of PrP (sCJD-MM1). Here we report three sCJD-MM1 patients who showed pro...
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| Veröffentlicht in: | Acta neuropathologica 2008-11, Vol.116 (5), p.561-566 |
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| creator | Kobayashi, Atsushi Arima, Kunimasa Ogawa, Masafumi Murata, Miho Fukuda, Takahiro Kitamoto, Tetsuyuki |
| description | Plaque-type deposition of prion protein (PrP) in the brain has been extremely rare in sporadic Creutzfeldt-Jakob disease patients with methionine homozygosity at polymorphic codon 129 of the PrP gene and type 1 abnormal isoform of PrP (sCJD-MM1). Here we report three sCJD-MM1 patients who showed prominent PrP-positive amyloid plaques in the cerebral and cerebellar white matter. All three patients showed clinical courses of long duration (2 years ≤), particularly at the end-stage. The white matter of these patients was severely damaged because of the prolonged disease duration. Furthermore, Alzheimer’s amyloid precursor protein, which accumulates within the axonal swellings under pathological conditions, co-accumulated with the PrP-amyloid plaques. These findings suggest that the axonal damage reflecting the prolonged disease duration causes the deposition of PrP-amyloid plaques in the white matter. The present study shows that PrP-amyloid plaques can occur in the white matter of sCJD-MM1 cases. |
| doi_str_mv | 10.1007/s00401-008-0425-8 |
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Here we report three sCJD-MM1 patients who showed prominent PrP-positive amyloid plaques in the cerebral and cerebellar white matter. All three patients showed clinical courses of long duration (2 years ≤), particularly at the end-stage. The white matter of these patients was severely damaged because of the prolonged disease duration. Furthermore, Alzheimer’s amyloid precursor protein, which accumulates within the axonal swellings under pathological conditions, co-accumulated with the PrP-amyloid plaques. These findings suggest that the axonal damage reflecting the prolonged disease duration causes the deposition of PrP-amyloid plaques in the white matter. The present study shows that PrP-amyloid plaques can occur in the white matter of sCJD-MM1 cases.</description><identifier>ISSN: 0001-6322</identifier><identifier>EISSN: 1432-0533</identifier><identifier>DOI: 10.1007/s00401-008-0425-8</identifier><identifier>PMID: 18751990</identifier><language>eng</language><publisher>Berlin/Heidelberg: Springer-Verlag</publisher><subject>Aged ; Blotting, Western ; Brain - metabolism ; Brain - pathology ; Creutzfeldt-Jakob Syndrome - genetics ; Creutzfeldt-Jakob Syndrome - metabolism ; Creutzfeldt-Jakob Syndrome - pathology ; DNA Mutational Analysis ; Female ; Humans ; Male ; Medicine ; Medicine & Public Health ; Middle Aged ; Neurons - metabolism ; Neurons - pathology ; Neurosciences ; Original Paper ; Pathology ; Plaque, Amyloid - genetics ; Plaque, Amyloid - metabolism ; Plaque, Amyloid - pathology ; Prions - genetics ; Prions - metabolism</subject><ispartof>Acta neuropathologica, 2008-11, Vol.116 (5), p.561-566</ispartof><rights>Springer-Verlag 2008</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-c400t-f73e9ae375791f27b242630e99c3a076a8e729c401a6acea75e72c6c75f1f7363</citedby><cites>FETCH-LOGICAL-c400t-f73e9ae375791f27b242630e99c3a076a8e729c401a6acea75e72c6c75f1f7363</cites></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://link.springer.com/content/pdf/10.1007/s00401-008-0425-8$$EPDF$$P50$$Gspringer$$H</linktopdf><linktohtml>$$Uhttps://link.springer.com/10.1007/s00401-008-0425-8$$EHTML$$P50$$Gspringer$$H</linktohtml><link.rule.ids>314,780,784,27922,27923,41486,42555,51317</link.rule.ids><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/18751990$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Kobayashi, Atsushi</creatorcontrib><creatorcontrib>Arima, Kunimasa</creatorcontrib><creatorcontrib>Ogawa, Masafumi</creatorcontrib><creatorcontrib>Murata, Miho</creatorcontrib><creatorcontrib>Fukuda, Takahiro</creatorcontrib><creatorcontrib>Kitamoto, Tetsuyuki</creatorcontrib><title>Plaque-type deposition of prion protein in the damaged white matter of sporadic Creutzfeldt-Jakob disease MM1 patients</title><title>Acta neuropathologica</title><addtitle>Acta Neuropathol</addtitle><addtitle>Acta Neuropathol</addtitle><description>Plaque-type deposition of prion protein (PrP) in the brain has been extremely rare in sporadic Creutzfeldt-Jakob disease patients with methionine homozygosity at polymorphic codon 129 of the PrP gene and type 1 abnormal isoform of PrP (sCJD-MM1). Here we report three sCJD-MM1 patients who showed prominent PrP-positive amyloid plaques in the cerebral and cerebellar white matter. All three patients showed clinical courses of long duration (2 years ≤), particularly at the end-stage. The white matter of these patients was severely damaged because of the prolonged disease duration. Furthermore, Alzheimer’s amyloid precursor protein, which accumulates within the axonal swellings under pathological conditions, co-accumulated with the PrP-amyloid plaques. These findings suggest that the axonal damage reflecting the prolonged disease duration causes the deposition of PrP-amyloid plaques in the white matter. The present study shows that PrP-amyloid plaques can occur in the white matter of sCJD-MM1 cases.</description><subject>Aged</subject><subject>Blotting, Western</subject><subject>Brain - metabolism</subject><subject>Brain - pathology</subject><subject>Creutzfeldt-Jakob Syndrome - genetics</subject><subject>Creutzfeldt-Jakob Syndrome - metabolism</subject><subject>Creutzfeldt-Jakob Syndrome - pathology</subject><subject>DNA Mutational Analysis</subject><subject>Female</subject><subject>Humans</subject><subject>Male</subject><subject>Medicine</subject><subject>Medicine & Public Health</subject><subject>Middle Aged</subject><subject>Neurons - metabolism</subject><subject>Neurons - pathology</subject><subject>Neurosciences</subject><subject>Original Paper</subject><subject>Pathology</subject><subject>Plaque, Amyloid - genetics</subject><subject>Plaque, Amyloid - metabolism</subject><subject>Plaque, Amyloid - pathology</subject><subject>Prions - genetics</subject><subject>Prions - metabolism</subject><issn>0001-6322</issn><issn>1432-0533</issn><fulltext>true</fulltext><rsrctype>article</rsrctype><creationdate>2008</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><recordid>eNqFkVFrFTEQhYMo9lr9Ab5I8MG31EmySTaP5VK10qIP7XPI3Z1tt-5utkm2Un-9We6FglCEQDLMN2c4OYS853DCAcznBFABZwA1g0ooVr8gG15JwUBJ-ZJsAEpXSyGOyJuU7kolTKVekyNeG8WthQ15-Dn4-wVZfpyRtjiH1Oc-TDR0dI7rY44hYz_RcvJtQfzob7Clv2_7jHT0OWNc4TSH6Nu-oduIS_7T4dBm9t3_Cjva9gl9Qnp5yensc49TTm_Jq84PCd8d7mNy_eXsavuNXfz4er49vWBNBZBZZyRaj9IoY3knzE5UQktAaxvpwWhfoxG2sNxr36A3qtSNbozqeJnV8ph82usWG8Vmym7sU4PD4CcMS3LaaqVrKf4Lcluwql4VP_4D3oUlTsWEE5zXWkmrCsT3UBNDShE7Vz5z9PHRcXBrdG4fnSvRuTU6V5eZDwfhZTdi-zRxyKoAYg-k0ppuMD5tfl71L7M5o9k</recordid><startdate>20081101</startdate><enddate>20081101</enddate><creator>Kobayashi, Atsushi</creator><creator>Arima, Kunimasa</creator><creator>Ogawa, Masafumi</creator><creator>Murata, Miho</creator><creator>Fukuda, Takahiro</creator><creator>Kitamoto, Tetsuyuki</creator><general>Springer-Verlag</general><general>Springer Nature B.V</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>3V.</scope><scope>7TK</scope><scope>7U9</scope><scope>7X7</scope><scope>7XB</scope><scope>88E</scope><scope>88G</scope><scope>8AO</scope><scope>8FI</scope><scope>8FJ</scope><scope>8FK</scope><scope>ABUWG</scope><scope>AFKRA</scope><scope>AZQEC</scope><scope>BENPR</scope><scope>CCPQU</scope><scope>DWQXO</scope><scope>FYUFA</scope><scope>GHDGH</scope><scope>GNUQQ</scope><scope>H94</scope><scope>K9.</scope><scope>M0S</scope><scope>M1P</scope><scope>M2M</scope><scope>PQEST</scope><scope>PQQKQ</scope><scope>PQUKI</scope><scope>PRINS</scope><scope>PSYQQ</scope><scope>Q9U</scope><scope>7T7</scope><scope>8FD</scope><scope>C1K</scope><scope>FR3</scope><scope>P64</scope><scope>7X8</scope></search><sort><creationdate>20081101</creationdate><title>Plaque-type deposition of prion protein in the damaged white matter of sporadic Creutzfeldt-Jakob disease MM1 patients</title><author>Kobayashi, Atsushi ; Arima, Kunimasa ; Ogawa, Masafumi ; Murata, Miho ; Fukuda, Takahiro ; Kitamoto, Tetsuyuki</author></sort><facets><frbrtype>5</frbrtype><frbrgroupid>cdi_FETCH-LOGICAL-c400t-f73e9ae375791f27b242630e99c3a076a8e729c401a6acea75e72c6c75f1f7363</frbrgroupid><rsrctype>articles</rsrctype><prefilter>articles</prefilter><language>eng</language><creationdate>2008</creationdate><topic>Aged</topic><topic>Blotting, Western</topic><topic>Brain - metabolism</topic><topic>Brain - pathology</topic><topic>Creutzfeldt-Jakob Syndrome - genetics</topic><topic>Creutzfeldt-Jakob Syndrome - metabolism</topic><topic>Creutzfeldt-Jakob Syndrome - pathology</topic><topic>DNA Mutational Analysis</topic><topic>Female</topic><topic>Humans</topic><topic>Male</topic><topic>Medicine</topic><topic>Medicine & Public Health</topic><topic>Middle Aged</topic><topic>Neurons - metabolism</topic><topic>Neurons - pathology</topic><topic>Neurosciences</topic><topic>Original Paper</topic><topic>Pathology</topic><topic>Plaque, Amyloid - genetics</topic><topic>Plaque, Amyloid - metabolism</topic><topic>Plaque, Amyloid - pathology</topic><topic>Prions - genetics</topic><topic>Prions - metabolism</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>Kobayashi, Atsushi</creatorcontrib><creatorcontrib>Arima, Kunimasa</creatorcontrib><creatorcontrib>Ogawa, Masafumi</creatorcontrib><creatorcontrib>Murata, Miho</creatorcontrib><creatorcontrib>Fukuda, Takahiro</creatorcontrib><creatorcontrib>Kitamoto, Tetsuyuki</creatorcontrib><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>ProQuest Central (Corporate)</collection><collection>Neurosciences Abstracts</collection><collection>Virology and AIDS Abstracts</collection><collection>Health & Medical Collection</collection><collection>ProQuest Central (purchase pre-March 2016)</collection><collection>Medical Database (Alumni Edition)</collection><collection>Psychology Database (Alumni)</collection><collection>ProQuest Pharma Collection</collection><collection>Hospital Premium Collection</collection><collection>Hospital Premium Collection (Alumni Edition)</collection><collection>ProQuest Central (Alumni) (purchase pre-March 2016)</collection><collection>ProQuest Central (Alumni Edition)</collection><collection>ProQuest Central UK/Ireland</collection><collection>ProQuest Central Essentials</collection><collection>ProQuest Central</collection><collection>ProQuest One Community College</collection><collection>ProQuest Central Korea</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>ProQuest Health & Medical Complete (Alumni)</collection><collection>Health & Medical Collection (Alumni Edition)</collection><collection>Medical Database</collection><collection>Psychology 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>ProQuest One Psychology</collection><collection>ProQuest Central Basic</collection><collection>Industrial and Applied Microbiology Abstracts (Microbiology A)</collection><collection>Technology Research Database</collection><collection>Environmental Sciences and Pollution Management</collection><collection>Engineering Research Database</collection><collection>Biotechnology and BioEngineering Abstracts</collection><collection>MEDLINE - Academic</collection><jtitle>Acta neuropathologica</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>Kobayashi, Atsushi</au><au>Arima, Kunimasa</au><au>Ogawa, Masafumi</au><au>Murata, Miho</au><au>Fukuda, Takahiro</au><au>Kitamoto, Tetsuyuki</au><format>journal</format><genre>article</genre><ristype>JOUR</ristype><atitle>Plaque-type deposition of prion protein in the damaged white matter of sporadic Creutzfeldt-Jakob disease MM1 patients</atitle><jtitle>Acta neuropathologica</jtitle><stitle>Acta Neuropathol</stitle><addtitle>Acta Neuropathol</addtitle><date>2008-11-01</date><risdate>2008</risdate><volume>116</volume><issue>5</issue><spage>561</spage><epage>566</epage><pages>561-566</pages><issn>0001-6322</issn><eissn>1432-0533</eissn><abstract>Plaque-type deposition of prion protein (PrP) in the brain has been extremely rare in sporadic Creutzfeldt-Jakob disease patients with methionine homozygosity at polymorphic codon 129 of the PrP gene and type 1 abnormal isoform of PrP (sCJD-MM1). Here we report three sCJD-MM1 patients who showed prominent PrP-positive amyloid plaques in the cerebral and cerebellar white matter. All three patients showed clinical courses of long duration (2 years ≤), particularly at the end-stage. The white matter of these patients was severely damaged because of the prolonged disease duration. Furthermore, Alzheimer’s amyloid precursor protein, which accumulates within the axonal swellings under pathological conditions, co-accumulated with the PrP-amyloid plaques. These findings suggest that the axonal damage reflecting the prolonged disease duration causes the deposition of PrP-amyloid plaques in the white matter. The present study shows that PrP-amyloid plaques can occur in the white matter of sCJD-MM1 cases.</abstract><cop>Berlin/Heidelberg</cop><pub>Springer-Verlag</pub><pmid>18751990</pmid><doi>10.1007/s00401-008-0425-8</doi><tpages>6</tpages></addata></record> |
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| subjects | Aged Blotting, Western Brain - metabolism Brain - pathology Creutzfeldt-Jakob Syndrome - genetics Creutzfeldt-Jakob Syndrome - metabolism Creutzfeldt-Jakob Syndrome - pathology DNA Mutational Analysis Female Humans Male Medicine Medicine & Public Health Middle Aged Neurons - metabolism Neurons - pathology Neurosciences Original Paper Pathology Plaque, Amyloid - genetics Plaque, Amyloid - metabolism Plaque, Amyloid - pathology Prions - genetics Prions - metabolism |
| title | Plaque-type deposition of prion protein in the damaged white matter of sporadic Creutzfeldt-Jakob disease MM1 patients |
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