Complement activation in human prion disease
The central event in the neuropathological process of prion diseases (PrD) is the accumulation of abnormal prion protein accompanied by severe neuronal loss. Despite the infectious nature of these diseases, no prominent immune response has been detected yet. However, recent studies have shown that c...
Saved in:
| Published in | Neurobiology of disease Vol. 15; no. 1; pp. 21 - 28 |
|---|---|
| Main Authors | , , , , , , , |
| Format | Journal Article |
| Language | English |
| Published |
United States
Elsevier Inc
01.02.2004
Elsevier |
| Subjects | |
| Online Access | Get full text |
Cover
Loading…
| Abstract | The central event in the neuropathological process of prion diseases (PrD) is the accumulation of abnormal prion protein accompanied by severe neuronal loss. Despite the infectious nature of these diseases, no prominent immune response has been detected yet. However, recent studies have shown that complement, a component of the innate immune system, is involved in the early pathogenesis of experimental prion infection. Here we demonstrate, in the diseased human brains, the presence of active compounds of the complement system, like C1q and C3b, in extracellular disease-associated prion protein deposits and the membrane attack complex in neurons. The neuronal localization of the membrane attack complex correlates well with the severity of disease-specific pathology and TUNEL labeling of neurons, irrespective of genotype or molecular phenotype of human prion diseases. |
|---|---|
| AbstractList | The central event in the neuropathological process of prion diseases (PrD) is the accumulation of abnormal prion protein accompanied by severe neuronal loss. Despite the infectious nature of these diseases, no prominent immune response has been detected yet. However, recent studies have shown that complement, a component of the innate immune system, is involved in the early pathogenesis of experimental prion infection. Here we demonstrate, in the diseased human brains, the presence of active compounds of the complement system, like C1q and C3b, in extracellular disease-associated prion protein deposits and the membrane attack complex in neurons. The neuronal localization of the membrane attack complex correlates well with the severity of disease-specific pathology and TUNEL labeling of neurons, irrespective of genotype or molecular phenotype of human prion diseases. The central event in the neuropathological process of prion diseases (PrD) is the accumulation of abnormal prion protein accompanied by severe neuronal loss. Despite the infectious nature of these diseases, no prominent immune response has been detected yet. However, recent studies have shown that complement, a component of the innate immune system, is involved in the early pathogenesis of experimental prion infection. Here we demonstrate, in the diseased human brains, the presence of active compounds of the complement system, like C1q and C3b, in extracellular disease-associated prion protein deposits and the membrane attack complex in neurons. The neuronal localization of the membrane attack complex correlates well with the severity of disease-specific pathology and TUNEL labeling of neurons, irrespective of genotype or molecular phenotype of human prion diseases.The central event in the neuropathological process of prion diseases (PrD) is the accumulation of abnormal prion protein accompanied by severe neuronal loss. Despite the infectious nature of these diseases, no prominent immune response has been detected yet. However, recent studies have shown that complement, a component of the innate immune system, is involved in the early pathogenesis of experimental prion infection. Here we demonstrate, in the diseased human brains, the presence of active compounds of the complement system, like C1q and C3b, in extracellular disease-associated prion protein deposits and the membrane attack complex in neurons. The neuronal localization of the membrane attack complex correlates well with the severity of disease-specific pathology and TUNEL labeling of neurons, irrespective of genotype or molecular phenotype of human prion diseases. |
| Author | Ironside, James W Lindeck-Pozza, Elisabeth Strohschneider, Michaela Kovacs, Gabor G Ströbel, Thomas Budka, Herbert Gasque, Philippe Guentchev, Marin |
| Author_xml | – sequence: 1 givenname: Gabor G surname: Kovacs fullname: Kovacs, Gabor G organization: Institute of Neurology, University of Vienna, Vienna, Austria – sequence: 2 givenname: Philippe surname: Gasque fullname: Gasque, Philippe organization: Medical Biochemistry Department, College of Medicine, University of Wales, Cardiff, UK – sequence: 3 givenname: Thomas surname: Ströbel fullname: Ströbel, Thomas organization: Institute of Neurology, University of Vienna, Vienna, Austria – sequence: 4 givenname: Elisabeth surname: Lindeck-Pozza fullname: Lindeck-Pozza, Elisabeth organization: Institute of Neurology, University of Vienna, Vienna, Austria – sequence: 5 givenname: Michaela surname: Strohschneider fullname: Strohschneider, Michaela organization: Institute of Neurology, University of Vienna, Vienna, Austria – sequence: 6 givenname: James W surname: Ironside fullname: Ironside, James W organization: National CJD Surveillance Unit, University of Edinburgh, Western General Hospital, EH4 2XU Edinburgh, Scotland, UK – sequence: 7 givenname: Herbert surname: Budka fullname: Budka, Herbert email: H.Budka@akh-wien.ac.at organization: Institute of Neurology, University of Vienna, Vienna, Austria – sequence: 8 givenname: Marin surname: Guentchev fullname: Guentchev, Marin organization: Institute of Neurology, University of Vienna, Vienna, Austria |
| BackLink | https://www.ncbi.nlm.nih.gov/pubmed/14751767$$D View this record in MEDLINE/PubMed |
| BookMark | eNqFkUtvFDEQhC0URDaBH8AF7YkTM7Rn_BiLE1pBiBSJC0jcLD_a4GXGs9izkfLv8WaTHHJITu226iu1qs7ISZoTEvKWQkuBio_bNlnfdgB9C6oFCi_IioLijeL9rxOyAiVUo5Sgp-SslC0ApVzJV-SUMsmpFHJFPmzmaTfihGlZG7fEa7PEOa1jWv_ZTyatd_mw-ljQFHxNXgYzFnxzN8_Jz69ffmy-NVffLy43n68ax5RYGmOdtYOEDgfD-iA4Auf1JTrshqGHIFRQXrKBeWG5tcFBx1DwwDwPGFx_Ti6Pvn42W11PmEy-0bOJ-vZjzr-1yUt0I-oeUBhLFePgGVI-GOgllTx4FBB6Vr3eH712ef63x7LoKRaH42gSzvuiB6CdUkw8K-yA90IxWYXv7oR7O6F_uO8-1CqQR4HLcykZg3Zxuc11ySaOmoI-1Ke3utanD_VpULrWV0n6iHwwf4L5dGSwNnIdMeviIiaHPmZ0S40sPkmrR7QbY4rOjH_x5hn2PzIew14 |
| CitedBy_id | crossref_primary_10_1016_j_mehy_2006_06_017 crossref_primary_10_1016_j_coi_2004_07_002 crossref_primary_10_1007_s12031_012_9854_x crossref_primary_10_2353_ajpath_2008_070442 crossref_primary_10_1038_nrmicro1492 crossref_primary_10_1172_JCI90605 crossref_primary_10_1007_s00702_017_1792_x crossref_primary_10_1097_NEN_0b013e31820805b9 crossref_primary_10_1007_s12035_014_8915_2 crossref_primary_10_1186_s40249_016_0128_7 crossref_primary_10_1016_j_amc_2007_10_062 crossref_primary_10_1371_journal_pone_0239537 crossref_primary_10_1007_s00430_019_00641_6 crossref_primary_10_3390_ijms10030976 crossref_primary_10_1016_j_imlet_2006_03_001 crossref_primary_10_1007_s00018_016_2418_4 crossref_primary_10_3390_ijms22042196 crossref_primary_10_1016_j_ajpath_2018_11_009 crossref_primary_10_1016_j_virol_2011_11_017 crossref_primary_10_1016_j_mehy_2004_07_024 crossref_primary_10_1074_jbc_M506579200 crossref_primary_10_1016_j_imbio_2004_06_003 crossref_primary_10_1371_journal_pone_0149792 crossref_primary_10_1155_2013_342931 crossref_primary_10_1007_s12035_018_1421_1 crossref_primary_10_1007_s00401_004_0953_9 crossref_primary_10_3390_biom10060908 crossref_primary_10_1016_j_brainresrev_2005_12_002 crossref_primary_10_1021_bi047370a crossref_primary_10_1021_sb400168u crossref_primary_10_1007_s10875_012_9767_z crossref_primary_10_1016_j_nbd_2005_01_005 crossref_primary_10_1007_s12035_022_02817_3 crossref_primary_10_1016_j_imlet_2010_03_012 crossref_primary_10_1016_j_ddmec_2004_11_001 crossref_primary_10_1146_annurev_pathmechdis_3_121806_154326 crossref_primary_10_1016_j_yexmp_2005_12_007 crossref_primary_10_1097_01_jnen_0000218445_30535_6f crossref_primary_10_1016_j_virol_2016_05_014 crossref_primary_10_1016_j_imbio_2007_04_001 crossref_primary_10_1016_j_molimm_2008_02_023 crossref_primary_10_1007_s12026_011_8239_5 crossref_primary_10_1111_j_1460_9568_2006_04801_x crossref_primary_10_1186_s12864_022_08306_0 crossref_primary_10_1196_annals_1332_010 crossref_primary_10_1016_j_jneuroim_2011_05_006 crossref_primary_10_1016_j_it_2008_11_003 crossref_primary_10_3389_fncel_2021_710390 |
| Cites_doi | 10.1097/00005072-199904000-00002 10.1038/86567 10.1172/JCI112372 10.1006/exnr.1999.7170 10.1111/j.1750-3639.2002.tb00417.x 10.1212/WNL.53.4.699 10.1006/nbdi.2002.0477 10.1097/00005072-199805000-00009 10.1074/jbc.273.13.7691 10.1016/S0002-9440(10)64753-0 10.1073/pnas.95.23.13363 10.1161/hq0701.092160 10.1074/jbc.M206448200 10.1002/ana.410430409 10.1002/glia.10146 10.1016/S0161-5890(99)00116-9 10.1016/S0002-9440(10)64604-4 10.1097/00005072-199710000-00005 10.1615/CritRevImmunol.v19.i3.10 10.1523/JNEUROSCI.20-20-07505.2000 10.1016/S0006-8993(99)01514-0 10.1038/86562 10.1016/S0162-3109(97)00059-3 10.1016/S0162-3109(00)80302-1 10.1002/1531-8249(199908)46:2<224::AID-ANA12>3.0.CO;2-W |
| ContentType | Journal Article |
| Copyright | 2003 Elsevier Inc. |
| Copyright_xml | – notice: 2003 Elsevier Inc. |
| DBID | AAYXX CITATION CGR CUY CVF ECM EIF NPM 7TK 7X8 DOA |
| DOI | 10.1016/j.nbd.2003.09.010 |
| DatabaseName | CrossRef Medline MEDLINE MEDLINE (Ovid) MEDLINE MEDLINE PubMed Neurosciences Abstracts MEDLINE - Academic DOAJ Directory of Open Access Journals |
| DatabaseTitle | CrossRef MEDLINE Medline Complete MEDLINE with Full Text PubMed MEDLINE (Ovid) Neurosciences Abstracts MEDLINE - Academic |
| DatabaseTitleList | MEDLINE - Academic Neurosciences Abstracts MEDLINE |
| Database_xml | – sequence: 1 dbid: DOA name: DOAJ Directory of Open Access Journals url: https://www.doaj.org/ sourceTypes: Open Website – sequence: 2 dbid: NPM name: PubMed url: https://proxy.k.utb.cz/login?url=http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=PubMed sourceTypes: Index Database – sequence: 3 dbid: EIF name: MEDLINE url: https://proxy.k.utb.cz/login?url=https://www.webofscience.com/wos/medline/basic-search sourceTypes: Index Database |
| DeliveryMethod | fulltext_linktorsrc |
| Discipline | Medicine Anatomy & Physiology |
| EISSN | 1095-953X |
| EndPage | 28 |
| ExternalDocumentID | oai_doaj_org_article_30e6ab19450d4e158a037175fde60f34 14751767 10_1016_j_nbd_2003_09_010 S0969996103001748 |
| Genre | Research Support, Non-U.S. Gov't Journal Article |
| GroupedDBID | --- --K --M .1- .55 .FO .GJ .~1 0R~ 123 1B1 1P~ 1~. 1~5 29N 4.4 457 4G. 53G 5RE 5VS 7-5 71M 8P~ 9JM AABNK AAEDT AAEDW AAIKJ AAKOC AALRI AAOAW AAQFI AAQXK AAXLA AAXUO AAYWO ABBQC ABCQJ ABFNM ABFRF ABJNI ABMAC ABMZM ABTEW ABWVN ABXDB ACDAQ ACGFO ACGFS ACIEU ACRLP ACRPL ACVFH ADBBV ADCNI ADEZE ADFGL ADMUD ADNMO ADVLN ADXHL AEBSH AEFWE AEIPS AEKER AENEX AEUPX AEVXI AFJKZ AFPUW AFRHN AFTJW AFXIZ AGCQF AGHFR AGQPQ AGUBO AGWIK AGYEJ AIEXJ AIGII AIKHN AITUG AJRQY AJUYK AKBMS AKRWK AKYEP ALMA_UNASSIGNED_HOLDINGS AMRAJ ANKPU ANZVX APXCP ASPBG AVWKF AXJTR AZFZN BKOJK BLXMC BNPGV CAG COF CS3 DM4 DU5 EBS EFBJH EFKBS EJD EO8 EO9 EP2 EP3 F5P FDB FEDTE FGOYB FIRID FNPLU G-Q GBLVA GROUPED_DOAJ HVGLF HZ~ IHE J1W K-O KOM M41 MO0 MOBAO N9A O-L O9- OAUVE OK1 OP~ OZT P-8 P-9 P2P PC. Q38 R2- ROL RPZ SCC SDF SDG SDP SES SEW SSH SSN SSZ T5K X7M XPP Z5R ZGI ZMT ZU3 ~G- 0SF 6I. AACTN AADPK AAIAV ABLVK ABYKQ AFCTW AFKWA AHPSJ AJBFU AJOXV AMFUW EFLBG LCYCR NCXOZ RIG AAYXX AGRNS CITATION CGR CUY CVF ECM EIF NPM PKN 7TK 7X8 |
| ID | FETCH-LOGICAL-c496t-abcbb8702e8a43f65e055a4362e28830f69f9d7484d6b5bbfc024e65f4d5fefc3 |
| IEDL.DBID | DOA |
| ISSN | 0969-9961 |
| IngestDate | Wed Aug 27 01:33:31 EDT 2025 Fri Jul 11 09:47:59 EDT 2025 Tue Aug 05 10:17:33 EDT 2025 Wed Feb 19 01:44:01 EST 2025 Thu Apr 24 23:44:16 EDT 2025 Tue Jul 01 03:07:08 EDT 2025 Fri Feb 23 02:29:34 EST 2024 Tue Aug 26 17:15:20 EDT 2025 |
| IsDoiOpenAccess | true |
| IsOpenAccess | true |
| IsPeerReviewed | true |
| IsScholarly | true |
| Issue | 1 |
| Keywords | Membrane attack complex Prion disease Complement Creutzfeldt–Jakob disease |
| Language | English |
| License | https://www.elsevier.com/tdm/userlicense/1.0 |
| LinkModel | DirectLink |
| MergedId | FETCHMERGED-LOGICAL-c496t-abcbb8702e8a43f65e055a4362e28830f69f9d7484d6b5bbfc024e65f4d5fefc3 |
| Notes | ObjectType-Article-2 SourceType-Scholarly Journals-1 ObjectType-Feature-1 content type line 23 ObjectType-Article-1 ObjectType-Feature-2 |
| OpenAccessLink | https://doaj.org/article/30e6ab19450d4e158a037175fde60f34 |
| PMID | 14751767 |
| PQID | 20536947 |
| PQPubID | 23462 |
| PageCount | 8 |
| ParticipantIDs | doaj_primary_oai_doaj_org_article_30e6ab19450d4e158a037175fde60f34 proquest_miscellaneous_80129946 proquest_miscellaneous_20536947 pubmed_primary_14751767 crossref_citationtrail_10_1016_j_nbd_2003_09_010 crossref_primary_10_1016_j_nbd_2003_09_010 elsevier_sciencedirect_doi_10_1016_j_nbd_2003_09_010 elsevier_clinicalkey_doi_10_1016_j_nbd_2003_09_010 |
| ProviderPackageCode | CITATION AAYXX |
| PublicationCentury | 2000 |
| PublicationDate | 2004-02-01 |
| PublicationDateYYYYMMDD | 2004-02-01 |
| PublicationDate_xml | – month: 02 year: 2004 text: 2004-02-01 day: 01 |
| PublicationDecade | 2000 |
| PublicationPlace | United States |
| PublicationPlace_xml | – name: United States |
| PublicationTitle | Neurobiology of disease |
| PublicationTitleAlternate | Neurobiol Dis |
| PublicationYear | 2004 |
| Publisher | Elsevier Inc Elsevier |
| Publisher_xml | – name: Elsevier Inc – name: Elsevier |
| References | Prusiner (BIB19) 1998; 95 Yasojima, McGeer, McGeer (BIB29) 1999; 833 Adler, Baker, Johnson, Ochi, Pritzl, Couser (BIB1) 1986; 77 Ironside (BIB10) 2000; 16 Klein, Kaeser, Schwarz, Weyd, Xenarios, Zinkernagel, Carroll, Verbeek, Botto, Walport, Molina, Kalinke, Acha-Orbea, Aguzzi (BIB12) 2001; 7 Stadelmann, Bruck, Bancher, Jellinger, Lassmann (BIB24) 1998; 57 Yang, Li, Meri, Rogers, Shen (BIB28) 2000; 20 Budka (BIB2) 2000; 16 Guentchev, Siedlak, Jarius, Tagliavini, Castellani, Perry, Smith, Budka (BIB9) 2002; 9 Ishii, Haga, Yagishita, Tateishi (BIB11) 1984; 2 Eikelenboom, Bate, Van Gool, Hoozemans, Rozemuller, Veerhuis, Williams (BIB5) 2002; 40 Stoltzner, Grenfell, Mori, Wisniewski, Wisniewski, Selkoe, Lemere (BIB25) 2000; 156 Parchi, Giese, Capellari, Brown, Schulz-Schaeffer, Windl, Zerr, Budka, Kopp, Piccardo, Poser, Rojiani, Streichemberger, Julien, Vital, Ghetti, Gambetti, Kretzschmar (BIB18) 1999; 46 Whitney, Andrews, McNamara (BIB27) 1999; 53 Dandoy-Dron, Guillo, Benboudjema, Deslys, Lasmezas, Dormont, Tovey, Dron (BIB4) 1998; 273 Gasque, Dean, McGreal, VanBeek, Morgan (BIB6) 2000; 49 Morgan, Gasque, Singhrao, Piddlesden (BIB16) 1997; 38 Makrides (BIB15) 1998; 50 Singhrao, Neal, Rushmere, Morgan, Gasque (BIB23) 2000; 157 Kovacs, Head, Hegyi, Bunn, Flicker, Hainfellner, McCardle, Laszlo, Jarius, Ironside, Budka (BIB13) 2002; 12 Morgan (BIB17) 1999; 19 Storch, Piddlesden, Haltia, Iivanainen, Morgan, Lassmann (BIB26) 1998; 43 Yasojima, Schwab, McGeer, McGeer (BIB30) 2001; 21 Guentchev, Hainfellner, Trabattoni, Budka (BIB8) 1997; 56 Collard, Lekowski, Jordan, Agah, Stahl (BIB3) 1999; 36 Rostagno, Revesz, Lashley, Tomidokoro, Magnotti, Braendgaard, Plant, Bojsen-Moller, Holton, Frangione, Ghiso (BIB20) 2002; 277 Gray, Chretien, Adle-Biassette, Dorandeu, Ereau, Delisle, Kopp, Ironside, Vital (BIB7) 1999; 58 Mabbott, Bruce, Botto, Walport, Pepys (BIB14) 2001; 7 Singhrao, Neal, Morgan, Gasque (BIB21) 1999; 159 Singhrao, Neal, Rushmere, Morgan, Gasque (BIB22) 1999; 79 Stadelmann (10.1016/j.nbd.2003.09.010_BIB24) 1998; 57 Klein (10.1016/j.nbd.2003.09.010_BIB12) 2001; 7 Yasojima (10.1016/j.nbd.2003.09.010_BIB30) 2001; 21 Rostagno (10.1016/j.nbd.2003.09.010_BIB20) 2002; 277 Eikelenboom (10.1016/j.nbd.2003.09.010_BIB5) 2002; 40 Guentchev (10.1016/j.nbd.2003.09.010_BIB8) 1997; 56 Budka (10.1016/j.nbd.2003.09.010_BIB2) 2000; 16 Singhrao (10.1016/j.nbd.2003.09.010_BIB21) 1999; 159 Gasque (10.1016/j.nbd.2003.09.010_BIB6) 2000; 49 Ironside (10.1016/j.nbd.2003.09.010_BIB10) 2000; 16 Storch (10.1016/j.nbd.2003.09.010_BIB26) 1998; 43 Singhrao (10.1016/j.nbd.2003.09.010_BIB22) 1999; 79 Yang (10.1016/j.nbd.2003.09.010_BIB28) 2000; 20 Stoltzner (10.1016/j.nbd.2003.09.010_BIB25) 2000; 156 Guentchev (10.1016/j.nbd.2003.09.010_BIB9) 2002; 9 Ishii (10.1016/j.nbd.2003.09.010_BIB11) 1984; 2 Morgan (10.1016/j.nbd.2003.09.010_BIB16) 1997; 38 Makrides (10.1016/j.nbd.2003.09.010_BIB15) 1998; 50 Collard (10.1016/j.nbd.2003.09.010_BIB3) 1999; 36 Dandoy-Dron (10.1016/j.nbd.2003.09.010_BIB4) 1998; 273 Adler (10.1016/j.nbd.2003.09.010_BIB1) 1986; 77 Morgan (10.1016/j.nbd.2003.09.010_BIB17) 1999; 19 Gray (10.1016/j.nbd.2003.09.010_BIB7) 1999; 58 Yasojima (10.1016/j.nbd.2003.09.010_BIB29) 1999; 833 Singhrao (10.1016/j.nbd.2003.09.010_BIB23) 2000; 157 Prusiner (10.1016/j.nbd.2003.09.010_BIB19) 1998; 95 Whitney (10.1016/j.nbd.2003.09.010_BIB27) 1999; 53 Kovacs (10.1016/j.nbd.2003.09.010_BIB13) 2002; 12 Parchi (10.1016/j.nbd.2003.09.010_BIB18) 1999; 46 Mabbott (10.1016/j.nbd.2003.09.010_BIB14) 2001; 7 |
| References_xml | – volume: 79 start-page: 1247 year: 1999 end-page: 1259 ident: BIB22 article-title: Differential expression of individual complement regulators in the brain and choroid plexus publication-title: Lab. Invest. – volume: 50 start-page: 59 year: 1998 end-page: 87 ident: BIB15 article-title: Therapeutic inhibition of the complement system publication-title: Pharmacol. Rev. – volume: 43 start-page: 465 year: 1998 end-page: 471 ident: BIB26 article-title: Multiple sclerosis: in situ evidence for antibody- and complement-mediated demyelination publication-title: Ann. Neurol. – volume: 159 start-page: 362 year: 1999 end-page: 376 ident: BIB21 article-title: Increased complement biosynthesis by microglia and complement activation on neurons in Huntington's disease publication-title: Exp. Neurol. – volume: 46 start-page: 224 year: 1999 end-page: 233 ident: BIB18 article-title: Classification of sporadic Creutzfeldt–Jakob disease based on molecular and phenotypic analysis of 300 subjects publication-title: Ann. Neurol. – volume: 277 start-page: 49782 year: 2002 end-page: 49790 ident: BIB20 article-title: Complement activation in chromosome 13 dementias. Similarities with Alzheimer's disease publication-title: J. Biol. Chem. – volume: 21 start-page: 1214 year: 2001 end-page: 1219 ident: BIB30 article-title: Complement components, but not complement inhibitors, are upregulated in atherosclerotic plaques publication-title: Arterioscler., Thromb., Vasc. Biol. – volume: 36 start-page: 941 year: 1999 end-page: 948 ident: BIB3 article-title: Complement activation following oxidative stress publication-title: Mol. Immunol. – volume: 95 start-page: 13363 year: 1998 end-page: 13383 ident: BIB19 article-title: Prions publication-title: Proc. Natl. Acad. Sci. U. S. A. – volume: 58 start-page: 321 year: 1999 end-page: 328 ident: BIB7 article-title: Neuronal apoptosis in Creutzfeldt–Jakob disease publication-title: J. Neuropathol. Exp. Neurol. – volume: 9 start-page: 275 year: 2002 end-page: 281 ident: BIB9 article-title: Oxidative damage to nucleic acids in human prion disease publication-title: Neurobiol Dis. – volume: 273 start-page: 7691 year: 1998 end-page: 7697 ident: BIB4 article-title: Gene expression in scrapie. Cloning of a new scrapie-responsive gene and the identification of increased levels of seven other mRNA transcripts publication-title: J. Biol. Chem. – volume: 19 start-page: 173 year: 1999 end-page: 198 ident: BIB17 article-title: Regulation of the complement membrane attack pathway publication-title: Crit. Rev. Immunol. – volume: 7 start-page: 485 year: 2001 end-page: 487 ident: BIB14 article-title: Temporary depletion of complement component C3 or genetic deficiency of C1q significantly delays onset of scrapie publication-title: Nat. Med. – volume: 12 start-page: 1 year: 2002 end-page: 11 ident: BIB13 article-title: Immunohistochemistry for the prion protein: comparison of different monoclonal antibodies in human prion disease subtypes publication-title: Brain Pathol. – volume: 157 start-page: 905 year: 2000 end-page: 918 ident: BIB23 article-title: Spontaneous classical pathway activation and deficiency of membrane regulators render human neurons susceptible to complement lysis publication-title: Am. J. Pathol. – volume: 53 start-page: 699 year: 1999 end-page: 708 ident: BIB27 article-title: Immunoglobulin G and complement immunoreactivity in the cerebral cortex of patients with Rasmussen's encephalitis publication-title: Neurology – volume: 16 start-page: 135 year: 2000 end-page: 142 ident: BIB2 article-title: Histopathology and immunohistochemistry of human transmissible spongiform encephalopathies (TSEs) publication-title: Arch. Virol., Suppl. – volume: 49 start-page: 171 year: 2000 end-page: 186 ident: BIB6 article-title: Complement components of the innate immune system in health and disease in the CNS publication-title: Immunopharmacology – volume: 156 start-page: 489 year: 2000 end-page: 499 ident: BIB25 article-title: Temporal accrual of complement proteins in amyloid plaques in Down's syndrome with Alzheimer's disease publication-title: Am. J. Pathol. – volume: 833 start-page: 297 year: 1999 end-page: 301 ident: BIB29 article-title: Complement regulators C1 inhibitor and CD59 do not significantly inhibit complement activation in Alzheimer disease publication-title: Brain Res. – volume: 77 start-page: 762 year: 1986 end-page: 767 ident: BIB1 article-title: Complement membrane attack complex stimulates production of reactive oxygen metabolites by cultured rat mesangial cells publication-title: J. Clin. Invest. – volume: 56 start-page: 1119 year: 1997 end-page: 1124 ident: BIB8 article-title: Distribution of parvalbumin-immunoreactive neurons in brain correlates with hippocampal and temporal cortical pathology in Creutzfeldt–Jakob disease publication-title: J. Neuropathol. Exp. Neurol. – volume: 16 start-page: 143 year: 2000 end-page: 151 ident: BIB10 article-title: Pathology of variant Creutzfeldt–Jakob disease publication-title: Arch. Virol., Suppl. – volume: 2 start-page: 370 year: 1984 end-page: 379 ident: BIB11 article-title: The presence of complements in amyloid plaques of Creutzfeldt–Jakob disease and Gerstmann–Straussler–Scheinker disease publication-title: Appl. Pathol. – volume: 20 start-page: 7505 year: 2000 end-page: 7509 ident: BIB28 article-title: Deficiency of complement defense protein CD59 may contribute to neurodegeneration in Alzheimer's disease publication-title: J. Neurosci. – volume: 38 start-page: 43 year: 1997 end-page: 50 ident: BIB16 article-title: The role of complement in disorders of the nervous system publication-title: Immunopharmacology – volume: 7 start-page: 488 year: 2001 end-page: 492 ident: BIB12 article-title: Complement facilitates early prion pathogenesis publication-title: Nat. Med. – volume: 57 start-page: 456 year: 1998 end-page: 464 ident: BIB24 article-title: Alzheimer disease: DNA fragmentation indicates increased neuronal vulnerability, but not apoptosis publication-title: J. Neuropathol. Exp. Neurol. – volume: 40 start-page: 9 year: 2002 end-page: 232 ident: BIB5 article-title: Neuroinflammation in Alzheimer's disease and prion disease publication-title: Glia – volume: 16 start-page: 135 year: 2000 ident: 10.1016/j.nbd.2003.09.010_BIB2 article-title: Histopathology and immunohistochemistry of human transmissible spongiform encephalopathies (TSEs) publication-title: Arch. Virol., Suppl. – volume: 58 start-page: 321 year: 1999 ident: 10.1016/j.nbd.2003.09.010_BIB7 article-title: Neuronal apoptosis in Creutzfeldt–Jakob disease publication-title: J. Neuropathol. Exp. Neurol. doi: 10.1097/00005072-199904000-00002 – volume: 7 start-page: 488 year: 2001 ident: 10.1016/j.nbd.2003.09.010_BIB12 article-title: Complement facilitates early prion pathogenesis publication-title: Nat. Med. doi: 10.1038/86567 – volume: 77 start-page: 762 year: 1986 ident: 10.1016/j.nbd.2003.09.010_BIB1 article-title: Complement membrane attack complex stimulates production of reactive oxygen metabolites by cultured rat mesangial cells publication-title: J. Clin. Invest. doi: 10.1172/JCI112372 – volume: 159 start-page: 362 year: 1999 ident: 10.1016/j.nbd.2003.09.010_BIB21 article-title: Increased complement biosynthesis by microglia and complement activation on neurons in Huntington's disease publication-title: Exp. Neurol. doi: 10.1006/exnr.1999.7170 – volume: 12 start-page: 1 year: 2002 ident: 10.1016/j.nbd.2003.09.010_BIB13 article-title: Immunohistochemistry for the prion protein: comparison of different monoclonal antibodies in human prion disease subtypes publication-title: Brain Pathol. doi: 10.1111/j.1750-3639.2002.tb00417.x – volume: 53 start-page: 699 year: 1999 ident: 10.1016/j.nbd.2003.09.010_BIB27 article-title: Immunoglobulin G and complement immunoreactivity in the cerebral cortex of patients with Rasmussen's encephalitis publication-title: Neurology doi: 10.1212/WNL.53.4.699 – volume: 16 start-page: 143 year: 2000 ident: 10.1016/j.nbd.2003.09.010_BIB10 article-title: Pathology of variant Creutzfeldt–Jakob disease publication-title: Arch. Virol., Suppl. – volume: 9 start-page: 275 year: 2002 ident: 10.1016/j.nbd.2003.09.010_BIB9 article-title: Oxidative damage to nucleic acids in human prion disease publication-title: Neurobiol Dis. doi: 10.1006/nbdi.2002.0477 – volume: 79 start-page: 1247 year: 1999 ident: 10.1016/j.nbd.2003.09.010_BIB22 article-title: Differential expression of individual complement regulators in the brain and choroid plexus publication-title: Lab. Invest. – volume: 57 start-page: 456 year: 1998 ident: 10.1016/j.nbd.2003.09.010_BIB24 article-title: Alzheimer disease: DNA fragmentation indicates increased neuronal vulnerability, but not apoptosis publication-title: J. Neuropathol. Exp. Neurol. doi: 10.1097/00005072-199805000-00009 – volume: 273 start-page: 7691 year: 1998 ident: 10.1016/j.nbd.2003.09.010_BIB4 article-title: Gene expression in scrapie. Cloning of a new scrapie-responsive gene and the identification of increased levels of seven other mRNA transcripts publication-title: J. Biol. Chem. doi: 10.1074/jbc.273.13.7691 – volume: 156 start-page: 489 year: 2000 ident: 10.1016/j.nbd.2003.09.010_BIB25 article-title: Temporal accrual of complement proteins in amyloid plaques in Down's syndrome with Alzheimer's disease publication-title: Am. J. Pathol. doi: 10.1016/S0002-9440(10)64753-0 – volume: 95 start-page: 13363 year: 1998 ident: 10.1016/j.nbd.2003.09.010_BIB19 article-title: Prions publication-title: Proc. Natl. Acad. Sci. U. S. A. doi: 10.1073/pnas.95.23.13363 – volume: 21 start-page: 1214 year: 2001 ident: 10.1016/j.nbd.2003.09.010_BIB30 article-title: Complement components, but not complement inhibitors, are upregulated in atherosclerotic plaques publication-title: Arterioscler., Thromb., Vasc. Biol. doi: 10.1161/hq0701.092160 – volume: 277 start-page: 49782 year: 2002 ident: 10.1016/j.nbd.2003.09.010_BIB20 article-title: Complement activation in chromosome 13 dementias. Similarities with Alzheimer's disease publication-title: J. Biol. Chem. doi: 10.1074/jbc.M206448200 – volume: 43 start-page: 465 year: 1998 ident: 10.1016/j.nbd.2003.09.010_BIB26 article-title: Multiple sclerosis: in situ evidence for antibody- and complement-mediated demyelination publication-title: Ann. Neurol. doi: 10.1002/ana.410430409 – volume: 40 start-page: 9 year: 2002 ident: 10.1016/j.nbd.2003.09.010_BIB5 article-title: Neuroinflammation in Alzheimer's disease and prion disease publication-title: Glia doi: 10.1002/glia.10146 – volume: 36 start-page: 941 year: 1999 ident: 10.1016/j.nbd.2003.09.010_BIB3 article-title: Complement activation following oxidative stress publication-title: Mol. Immunol. doi: 10.1016/S0161-5890(99)00116-9 – volume: 50 start-page: 59 year: 1998 ident: 10.1016/j.nbd.2003.09.010_BIB15 article-title: Therapeutic inhibition of the complement system publication-title: Pharmacol. Rev. – volume: 157 start-page: 905 year: 2000 ident: 10.1016/j.nbd.2003.09.010_BIB23 article-title: Spontaneous classical pathway activation and deficiency of membrane regulators render human neurons susceptible to complement lysis publication-title: Am. J. Pathol. doi: 10.1016/S0002-9440(10)64604-4 – volume: 2 start-page: 370 year: 1984 ident: 10.1016/j.nbd.2003.09.010_BIB11 article-title: The presence of complements in amyloid plaques of Creutzfeldt–Jakob disease and Gerstmann–Straussler–Scheinker disease publication-title: Appl. Pathol. – volume: 56 start-page: 1119 year: 1997 ident: 10.1016/j.nbd.2003.09.010_BIB8 article-title: Distribution of parvalbumin-immunoreactive neurons in brain correlates with hippocampal and temporal cortical pathology in Creutzfeldt–Jakob disease publication-title: J. Neuropathol. Exp. Neurol. doi: 10.1097/00005072-199710000-00005 – volume: 19 start-page: 173 year: 1999 ident: 10.1016/j.nbd.2003.09.010_BIB17 article-title: Regulation of the complement membrane attack pathway publication-title: Crit. Rev. Immunol. doi: 10.1615/CritRevImmunol.v19.i3.10 – volume: 20 start-page: 7505 year: 2000 ident: 10.1016/j.nbd.2003.09.010_BIB28 article-title: Deficiency of complement defense protein CD59 may contribute to neurodegeneration in Alzheimer's disease publication-title: J. Neurosci. doi: 10.1523/JNEUROSCI.20-20-07505.2000 – volume: 833 start-page: 297 year: 1999 ident: 10.1016/j.nbd.2003.09.010_BIB29 article-title: Complement regulators C1 inhibitor and CD59 do not significantly inhibit complement activation in Alzheimer disease publication-title: Brain Res. doi: 10.1016/S0006-8993(99)01514-0 – volume: 7 start-page: 485 year: 2001 ident: 10.1016/j.nbd.2003.09.010_BIB14 article-title: Temporary depletion of complement component C3 or genetic deficiency of C1q significantly delays onset of scrapie publication-title: Nat. Med. doi: 10.1038/86562 – volume: 38 start-page: 43 year: 1997 ident: 10.1016/j.nbd.2003.09.010_BIB16 article-title: The role of complement in disorders of the nervous system publication-title: Immunopharmacology doi: 10.1016/S0162-3109(97)00059-3 – volume: 49 start-page: 171 year: 2000 ident: 10.1016/j.nbd.2003.09.010_BIB6 article-title: Complement components of the innate immune system in health and disease in the CNS publication-title: Immunopharmacology doi: 10.1016/S0162-3109(00)80302-1 – volume: 46 start-page: 224 year: 1999 ident: 10.1016/j.nbd.2003.09.010_BIB18 article-title: Classification of sporadic Creutzfeldt–Jakob disease based on molecular and phenotypic analysis of 300 subjects publication-title: Ann. Neurol. doi: 10.1002/1531-8249(199908)46:2<224::AID-ANA12>3.0.CO;2-W |
| SSID | ssj0011597 |
| Score | 2.0159254 |
| Snippet | The central event in the neuropathological process of prion diseases (PrD) is the accumulation of abnormal prion protein accompanied by severe neuronal loss.... |
| SourceID | doaj proquest pubmed crossref elsevier |
| SourceType | Open Website Aggregation Database Index Database Enrichment Source Publisher |
| StartPage | 21 |
| SubjectTerms | Adolescent Adult Aged Aged, 80 and over Antibodies - immunology Antibody Specificity Brain - immunology Brain - physiopathology Complement Complement C1q - immunology Complement C3b - immunology Complement Membrane Attack Complex - immunology Complement System Proteins - immunology Creutzfeldt–Jakob disease Female Humans Immunity, Innate - immunology Immunohistochemistry Male Membrane attack complex Middle Aged Neurons - immunology Prion disease Prion Diseases - immunology PrPSc Proteins - immunology |
| SummonAdditionalLinks | – databaseName: Elsevier SD Freedom Collection Journals [SCFCJ] dbid: AIKHN link: http://utb.summon.serialssolutions.com/2.0.0/link/0/eLvHCXMwnV3dT9swED91RUJ7mUbZR2FseZj2MBE1bWwneSwI1IHgZSDxZtnxeeq0pVVXHvjvuXOcMh5g0t4Sx06s8_nunLv7HcBnowzZFaVPEZWlAwqthfW1TzMx9sIIVZaOE5wvLtXsWpzdyJseHHe5MBxWGWV_K9ODtI4to0jN0XI-H30n45utda6TRWwlyhewNcmprQ9b02_ns8uNM4E0dsiapmcpD-icmyHMq7EtXmhAO-U82r_UU0Dxf6SlnrJCgzY6fQ2vohmZTNuZ7kAPmwHsThs6Qv--S74kIbAz_DEfwPZF9J_vwiFv_zZgPOGMhvZ_bDJvklCrL1mu-DY6bd7A9enJ1fEsjfUS0lpUap0aW1tL-2-CpRG5VxIzKelKTZBrCmdeVb5yDB7qlJWW1oQUNCrphZMefZ2_hX6zaPA9JKTDjEGUyIB93jmDNYkCdK6WREDphpB1ZNJ1BBPnmha_dBc19lMTZbnIZa6zShNlh_B1M2TZImk81_mIab_pyCDYoWGx-qEjF2iakDJ2XAmZOYFjWRrGHyykd6gyn4shTLqV012eKUlGetH8uS-LzaBHjPivYZ861tC0QdnrYhpc3P6hTjJXlSie7sFGQlUJNYR3LU890EcUclyoYu__JrUPL9tAI46--QD99eoWD8iGWtuPcY_cA9l-F5U priority: 102 providerName: Elsevier |
| Title | Complement activation in human prion disease |
| URI | https://www.clinicalkey.com/#!/content/1-s2.0-S0969996103001748 https://dx.doi.org/10.1016/j.nbd.2003.09.010 https://www.ncbi.nlm.nih.gov/pubmed/14751767 https://www.proquest.com/docview/20536947 https://www.proquest.com/docview/80129946 https://doaj.org/article/30e6ab19450d4e158a037175fde60f34 |
| Volume | 15 |
| hasFullText | 1 |
| inHoldings | 1 |
| isFullTextHit | |
| isPrint | |
| link | http://utb.summon.serialssolutions.com/2.0.0/link/0/eLvHCXMwrV3NT90wDLcGkxAXxOf2-HjrYdphWlnfa5K2xwcCPdhAaBoStyhpHIkJCoLHgQt_O3bSPuAAXHbqh5K2cuzaju2fAb4aZciuKH2KqCw5KLQW1tc-zcTACyNUWToucD46VuNTcXgmz561-uKcsAgPHAn3M89QGUuutsycwIEsDYPMFdI7VJnPAxIo6bzOmWrjB6Skiy6GGbK5GhthQQOoKZfLPtNCAaz_hTJ6zdgMSmd_ERZaazEZxa9cgg_YLMPKqCFP-fI--ZaE_M2wMb4Mc0dtmHwFfrCUx7zwhAsX4rZrct4koSVfcn3Dl21sZhVO9_f-7o7Tti1CWotKTVJja2tJzIZYGpF7JTGTks7UELl1cOZV5SvHGKFOWWmJ9KSHUUkvnPTo63wNZpurBj9DQqrKGESJjMvnnTNYk8Sjc7WsyNRxPcg6Mum6xQzn1hUXuksO-6eJstzLMtdZpYmyPfg-nXIdATPeGrzDtJ8OZKzrcIM4QLccoN_jgB4Mu5XTXTkp_QDpQedvvVlMJ7W2RrQh3pv2pWMNTXLIwRXT4NXdLQ2SuapE8foItgWqSqgefIo89UQfUchBoYr1_0GODZiP2UWccrMJs5ObO9wiw2li-zCz_TDow8fR7p_fJ3w8-DU-7gfJeQR6YxfH |
| linkProvider | Directory of Open Access Journals |
| linkToHtml | http://utb.summon.serialssolutions.com/2.0.0/link/0/eLvHCXMwnV1LT9wwEB5RkNpeEI-2LFDIoeqharrZje0kR4pAS8tyKUjcLDseV1uV7Gq7HHrpb2fGcbblAJW4JdE4scbzcjzzDcA7owzFFaVPEZWlDQqthfW1TzMx8MIIVZaOC5zHF2p0Jb5cy-sVOO5qYTitMtr-1qYHax2f9CM3-7PJpP-Ngm-O1rlPFomVKJ_BmiD1Ze389GeZ50ERT-iwwtQpk3dHmyHJq7EtWmjAOuUq2n-cU8Dwv-ejHopBgy863YD1GEQmR-08N2EFmy3YPmpoA33zO3mfhLTO8L98C56P4-n5Nnxk5W_TxROuZ2j_xiaTJgmd-pLZnG_jkc0ruDo9uTwepbFbQlqLSi1SY2trSfuGWBqReyUxk5Ku1BC5o3DmVeUrx9ChTllpaUXIPaOSXjjp0df5a1htpg3uQEIezBhEiQzX550zWJMhQOdqSQyUrgdZxyZdRyhx7mjxU3c5Yz80cZZbXOY6qzRxtgcflkNmLY7GY8SfmfdLQobADg-m8-86yoCmCSljB5WQmRM4kKVh9MFCeocq87nowbBbOd1VmZJdpBdNHvuyWA66J4b_G3bYiYYm9eQzF9Pg9PYXEclcVaJ4mIJDhKoSqgdvWpn6yx9RyEGhit2nTeoQXowux-f6_Ozi6x68bFOOOA9nH1YX81t8S9HUwh4EbbkDsIEZVw |
| openUrl | ctx_ver=Z39.88-2004&ctx_enc=info%3Aofi%2Fenc%3AUTF-8&rfr_id=info%3Asid%2Fsummon.serialssolutions.com&rft_val_fmt=info%3Aofi%2Ffmt%3Akev%3Amtx%3Ajournal&rft.genre=article&rft.atitle=Complement+activation+in+human+prion+disease&rft.jtitle=Neurobiology+of+disease&rft.au=Gabor+G+Kovacs&rft.au=Philippe+Gasque&rft.au=Thomas+Str%C3%B6bel&rft.au=Elisabeth+Lindeck-Pozza&rft.date=2004-02-01&rft.pub=Elsevier&rft.eissn=1095-953X&rft.volume=15&rft.issue=1&rft.spage=21&rft.epage=28&rft_id=info:doi/10.1016%2Fj.nbd.2003.09.010&rft.externalDBID=DOA&rft.externalDocID=oai_doaj_org_article_30e6ab19450d4e158a037175fde60f34 |
| thumbnail_l | http://covers-cdn.summon.serialssolutions.com/index.aspx?isbn=/lc.gif&issn=0969-9961&client=summon |
| thumbnail_m | http://covers-cdn.summon.serialssolutions.com/index.aspx?isbn=/mc.gif&issn=0969-9961&client=summon |
| thumbnail_s | http://covers-cdn.summon.serialssolutions.com/index.aspx?isbn=/sc.gif&issn=0969-9961&client=summon |