Anti transglutaminase antibodies cause ataxia in mice.

<h4>Background</h4>Celiac disease (CD) is an autoimmune gastrointestinal disorder characterized by the presence of anti-transglutaminase 2 (TG2) and anti-gliadin antibodies. Amongst the neurological dysfunctions associated with CD, ataxia represents the most common one.<h4>Methods&...

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Main Authors: Sabrina Boscolo, Andrea Lorenzon, Daniele Sblattero, Fiorella Florian, Marco Stebel, Roberto Marzari, Tarcisio Not, Daniel Aeschlimann, Alessandro Ventura, Marios Hadjivassiliou, Enrico Tongiorgi
Format: Article
Language:English
Published: Public Library of Science (PLoS) 2010-03-01
Series:PLoS ONE
Online Access:https://journals.plos.org/plosone/article/file?id=10.1371/journal.pone.0009698&type=printable
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author Sabrina Boscolo
Andrea Lorenzon
Daniele Sblattero
Fiorella Florian
Marco Stebel
Roberto Marzari
Tarcisio Not
Daniel Aeschlimann
Alessandro Ventura
Marios Hadjivassiliou
Enrico Tongiorgi
author_facet Sabrina Boscolo
Andrea Lorenzon
Daniele Sblattero
Fiorella Florian
Marco Stebel
Roberto Marzari
Tarcisio Not
Daniel Aeschlimann
Alessandro Ventura
Marios Hadjivassiliou
Enrico Tongiorgi
author_sort Sabrina Boscolo
collection DOAJ
description <h4>Background</h4>Celiac disease (CD) is an autoimmune gastrointestinal disorder characterized by the presence of anti-transglutaminase 2 (TG2) and anti-gliadin antibodies. Amongst the neurological dysfunctions associated with CD, ataxia represents the most common one.<h4>Methods</h4>We analyzed by immunohistochemistry, the anti-neural reactivity of the serum from 20 CD patients. To determine the role of anti-TG2 antibodies in ataxia, two anti-TG2 single chain variable fragments (scFv), isolated from a phage-display IgA antibody library, were characterized by immunohistochemistry and ELISA, and injected in mice to study their effects on motor coordination. We found that 75% of the CD patient population without evidence of neurological involvement, has circulating anti-neural IgA and/or IgG antibodies. Two anti-TG2 scFvs, cloned from one CD patient, stained blood vessels but only one reacted with neurons. This anti-TG2 antibody showed cross reactivity with the transglutaminase isozymes TG3 and TG6. Intraventricular injection of the anti-TG2 or the anti-TG2/3/6 cross-reactive scFv provoked transient, equally intensive ataxia in mice.<h4>Conclusion</h4>The serum from CD patients contains anti-TG2, TG3 and TG6 antibodies that may potentially cause ataxia.
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spelling doaj-art-e4344625fbe0478c8dbc601a5230d38b2025-08-20T02:31:51ZengPublic Library of Science (PLoS)PLoS ONE1932-62032010-03-0153e969810.1371/journal.pone.0009698Anti transglutaminase antibodies cause ataxia in mice.Sabrina BoscoloAndrea LorenzonDaniele SblatteroFiorella FlorianMarco StebelRoberto MarzariTarcisio NotDaniel AeschlimannAlessandro VenturaMarios HadjivassiliouEnrico Tongiorgi<h4>Background</h4>Celiac disease (CD) is an autoimmune gastrointestinal disorder characterized by the presence of anti-transglutaminase 2 (TG2) and anti-gliadin antibodies. Amongst the neurological dysfunctions associated with CD, ataxia represents the most common one.<h4>Methods</h4>We analyzed by immunohistochemistry, the anti-neural reactivity of the serum from 20 CD patients. To determine the role of anti-TG2 antibodies in ataxia, two anti-TG2 single chain variable fragments (scFv), isolated from a phage-display IgA antibody library, were characterized by immunohistochemistry and ELISA, and injected in mice to study their effects on motor coordination. We found that 75% of the CD patient population without evidence of neurological involvement, has circulating anti-neural IgA and/or IgG antibodies. Two anti-TG2 scFvs, cloned from one CD patient, stained blood vessels but only one reacted with neurons. This anti-TG2 antibody showed cross reactivity with the transglutaminase isozymes TG3 and TG6. Intraventricular injection of the anti-TG2 or the anti-TG2/3/6 cross-reactive scFv provoked transient, equally intensive ataxia in mice.<h4>Conclusion</h4>The serum from CD patients contains anti-TG2, TG3 and TG6 antibodies that may potentially cause ataxia.https://journals.plos.org/plosone/article/file?id=10.1371/journal.pone.0009698&type=printable
spellingShingle Sabrina Boscolo
Andrea Lorenzon
Daniele Sblattero
Fiorella Florian
Marco Stebel
Roberto Marzari
Tarcisio Not
Daniel Aeschlimann
Alessandro Ventura
Marios Hadjivassiliou
Enrico Tongiorgi
Anti transglutaminase antibodies cause ataxia in mice.
PLoS ONE
title Anti transglutaminase antibodies cause ataxia in mice.
title_full Anti transglutaminase antibodies cause ataxia in mice.
title_fullStr Anti transglutaminase antibodies cause ataxia in mice.
title_full_unstemmed Anti transglutaminase antibodies cause ataxia in mice.
title_short Anti transglutaminase antibodies cause ataxia in mice.
title_sort anti transglutaminase antibodies cause ataxia in mice
url https://journals.plos.org/plosone/article/file?id=10.1371/journal.pone.0009698&type=printable
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