| Interacting proteins: P21980 and P42858 |
Pubmed |
SVM Score :0.54360001 |
| Our laboratory previously demonstrated that huntingtin protein colocalizes with transglutaminase 2 and its product , the epsilon ( gamma glutamyl ) lysine bond in intranuclear inclusions in HD frontal cortex . 0.54360001^^^ |
|
| Interacting proteins: P21980 and P42858 |
Pubmed |
SVM Score :0.0 |
| In the presence of tissue transglutaminase , purified glyceraldehyde 3 phosphate dehydrogenase ( a key glycolytic enzyme that binds tightly to the polyglutamine domains of both huntingtin and dentatorubral pallidoluysian atrophy proteins ) is covalently attached to polyglutamine peptides in vitro , resulting in the formation of high M ( r ) aggregates . ^^^ |
|
| Interacting proteins: P21980 and P42858 |
Pubmed |
SVM Score :0.0 |
| In this report immunohistochemical and biochemical methods were used to demonstrate that tissue transglutaminase expression and transglutaminase activity are elevated in HD brains in a grade dependent manner . ^^^ In the striatum , tissue transglutaminase activity was significantly increased in the grade 3 HD cases compared with controls . ^^^ When normalized to the neuronal marker calbindin D28k , immunoblot analysis revealed that in the striatum the levels of tissue transglutaminase were significantly increased in all HD cases compared with controls . ^^^ Immunohistochemical staining of the HD striatum revealed that tissue transglutaminase immunoreactivity was markedly increased in all grades as compared with controls . ^^^ In the superior frontal cortex , tissue transglutaminase activity was significantly higher in all HD cases as compared with controls . ^^^ |
|
| Interacting proteins: P21980 and P42858 |
Pubmed |
SVM Score :0.0 |
| Tissue transglutaminase ( tTG ) is a transamidating enzyme that is elevated in Huntington ' s disease ( HD ) brain and may be involved in the etiology of the disease . ^^^ |
|
| Interacting proteins: P21980 and P42858 |
Pubmed |
SVM Score :0.0 |
| Because in vitro expanded polyglutamine repeats are glutaminyl donor substrates of tissue transglutaminase ( tTG ) , it has been hypothesized that tTG may contribute to the formation of these aggregates in HD . ^^^ Tissue transglutaminase does not contribute to the formation of mutant huntingtin aggregates . ^^^ |
|
| Interacting proteins: P21980 and P42858 |
Pubmed |
SVM Score :0.0 |
| Pathological length polyglutamine ( Q ( n ) ) expansions , such as those that occur in the huntingtin protein ( htt ) in Huntington ' s disease ( HD ) , are excellent substrates for tissue transglutaminase in vitro , and transglutaminase activity is increased in post mortem HD brain . ^^^ However , direct evidence for the participation of tissue transglutaminase ( or other transglutaminases ) in HD patients in vivo is scarce . ^^^ |
|
| Interacting proteins: P21980 and P42858 |
Pubmed |
SVM Score :0.0 |
| Proteolytic products of the huntingtin protein can contribute to toxic cellular aggregates that may be formed in part by tissue transglutaminase ( Tgase ) . ^^^ |
|
| Interacting proteins: P21980 and P42858 |
Pubmed |
SVM Score :0.0 |
| In order to determine the role of tissue transglutaminase ( tTG ) in HD aggregate formation and disease progression , we cross bred the R6 / 2 HD mouse model with a tTG knockout mouse line . ^^^ |
|
| Interacting proteins: P21980 and P42858 |
Pubmed |
SVM Score :0.0 |
| Tissue transglutaminase ( tTG ) is a multifunctional enzyme that contributes to disease progression in mouse models of Huntington ' s disease ( HD ) , an inherited neurodegenerative disease that shows an age related onset . ^^^ |
|
| Interacting proteins: P21980 and P42858 |
Pubmed |
SVM Score :0.0 |
| Furthermore , the transglutaminase 2 isoform colocalizes with both huntingtin protein and epsilon ( gamma glutamyl ) lysine covalent cross links in HD intranuclear inclusions . ^^^ Transient transfection of N terminally truncated huntingtin with an expanded glutamine domain ( htt N 63 148Q myc ) with and without and transglutaminase 2 into HEK 293T cells resulted in an increase in cross linked huntingtin in the insoluble formic acid treated pellet in comparison to transfection of N terminally truncated huntingtin with normal length glutamine domain ( htt N 63 18Q myc ) . ^^^ Transfection with both htt N 63 148Q myc and transglutaminase 2 resulted in high molecular weight huntingtin in the insoluble fraction . ^^^ |
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| Interacting proteins: P21980 and P42858 |
Pubmed |
SVM Score :0.0 |
| We demonstrated previously that transglutaminase 2 cross links mutant huntingtin in cells in culture and demonstrated the presence of transglutaminase catalyzed cross links in the HD cortex that colocalize with transglutaminase 2 and huntingtin . ^^^ We found that calmodulin colocalizes at the confocal level with transglutaminase 2 and with huntingtin in HD intranuclear inclusions . ^^^ Calmodulin regulates transglutaminase 2 cross linking of huntingtin . ^^^ Calmodulin coimmunoprecipitates with transglutaminase 2 and huntingtin in cells transfected with myc tagged N terminal huntingtin fragments containing 148 polyglutamine repeats ( htt N 63 148Q myc ) and transglutaminase 2 but not in cells transfected with myc tagged N terminal huntingtin fragments containing 18 polyglutamine repeats . ^^^ Our previous studies demonstrated that transfection with both htt N 63 148Q myc and transglutaminase 2 resulted in cross linking of mutant huntingtin protein fragments and the formation of insoluble high molecular weight aggregates of huntingtin protein fragments . ^^^ |
|
| Interacting proteins: P21980 and P42858 |
Pubmed |
SVM Score :0.0 |
| PATIENTS AND METHODS : Three hundred nineteen boys and 259 girls younger than 18 years with previously untreated HD , enrolled onto the study between 1990 and 1995 , were allocated to treatment group ( TG ) 1 ( early stages ) , TG 2 ( intermediate stages ) , or TG 3 ( advanced stages ) . ^^^ |
|
| Interacting proteins: P21980 and P42858 |
Pubmed |
SVM Score :0.0 |
| By crossing Huntington ' s disease ( HD ) R6 / 1 transgenic mice with ' tissue ' transglutaminase ( TG 2 ) knock out mice , we have demonstrated that this multifunctional enzyme plays an important role in the neuronal death characterising this disorder in vivo . ^^^ |
|
| Interacting proteins: P21980 and P42858 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: P21980 and P42858 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: P21980 and P42858 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: P21980 and P42858 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: P21980 and P42858 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: P21980 and P42858 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: P21980 and P42858 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: P21980 and P42858 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: P21980 and P42858 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: P21980 and P42858 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: P21980 and P42858 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: P21980 and P42858 |
Pubmed |
SVM Score :0.0 |
| NA |
|