Pubmed abstracts for Protein-Protein Interaction search result :


Interacting proteins: P42858 and P42574 Pubmed SVM Score :0.0
Genetic analyses excluded SCA 1 , SCA 2 , SCA 3 , SCA 6 , SCA 7 , DRPLA , and huntingtin gene mutations . . ^^^
Interacting proteins: P42858 and P42574 Pubmed SVM Score :0.0
Wild type huntingtin protects from apoptosis upstream of caspase 3 . ^^^
Interacting proteins: P42858 and P42574 Pubmed SVM Score :0.0
Activation of caspase 3 by H2O2 was more pronounced in LD than in MD and HD . ^^^
Interacting proteins: P42858 and P42574 Pubmed SVM Score :0.0
This leads to overactivation of caspase 3 , which is capable of cleaving htt . ^^^
Interacting proteins: P42858 and P42574 Pubmed SVM Score :0.0
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Interacting proteins: P42858 and P42574 Pubmed SVM Score :0.0
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Interacting proteins: P42858 and P42574 Pubmed SVM Score :0.0
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Interacting proteins: P42858 and P42574 Pubmed SVM Score :0.0
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Interacting proteins: P42858 and P42574 Pubmed SVM Score :0.0
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Interacting proteins: P42858 and P42574 Pubmed SVM Score :0.0
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Interacting proteins: P42858 and P42574 Pubmed SVM Score :0.0
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Interacting proteins: P42858 and P42574 Pubmed SVM Score :0.0
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Interacting proteins: P42858 and P42574 Pubmed SVM Score :0.0
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Interacting proteins: P42858 and P42574 Pubmed SVM Score :0.0
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Interacting proteins: P42858 and P42574 Pubmed SVM Score :0.0
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Interacting proteins: P42858 and P42574 Pubmed SVM Score :0.0
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Interacting proteins: P42858 and P42574 Pubmed SVM Score :0.0
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Interacting proteins: P42858 and P42574 Pubmed SVM Score :0.0
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Interacting proteins: P42858 and P42574 Pubmed SVM Score :0.0
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Interacting proteins: P42858 and P42574 Pubmed SVM Score :0.0
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Interacting proteins: P42858 and P42574 Pubmed SVM Score :0.0
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Interacting proteins: P42858 and P42574 Pubmed SVM Score :0.0
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Interacting proteins: P42858 and P42574 Pubmed SVM Score :0.0
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Interacting proteins: P42858 and P42574 Pubmed SVM Score :0.0
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Interacting proteins: P42858 and P42574 Pubmed SVM Score :0.0
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Interacting proteins: P42858 and P42574 Pubmed SVM Score :0.0
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Interacting proteins: P42858 and P42574 Pubmed SVM Score :0.0
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Interacting proteins: P42858 and P42574 Pubmed SVM Score :0.0
Cleavage of huntingtin by apopain , a proapoptotic cysteine protease , is modulated by the polyglutamine tract . ^^^ Here we show that apoptotic extracts and apopain itself specifically cleave the HD gene product , huntingtin . ^^^
Interacting proteins: P42858 and P42574 Pubmed SVM Score :0.0
Huntingtin is specifically cleaved during apoptosis by a key cysteine protease , apopain , known to play a pivotal role in apoptotic cell death . ^^^
Interacting proteins: P42858 and P42574 Pubmed SVM Score :0.0
Caspase 3 ( apopain ) , a key cysteine protease known to play a seminal role in neural apoptosis , has also been demonstrated to specifically cleave huntingtin in a CAG length dependent manner . ^^^ Caspase 3 ( apopain ) , a key cysteine protease known to play a seminal role in neural apoptosis , has also been demonstrated to specifically cleave huntingtin in a CAG length dependent manner . ^^^
Interacting proteins: P42858 and P42574 Pubmed SVM Score :0.0
Huntingtin truncated at nucleotide 1955 , close to the caspase 3 cleavage site , forms perinuclear aggregates more readily than full length huntingtin and increases the susceptibility of cells to death following apoptotic stimuli . ^^^
Interacting proteins: P42858 and P42574 Pubmed SVM Score :0.0
Selective processing of the mutant , but not the wild type , full length huntingtin was observed at late time points , with appearance of a breakdown product corresponding to a predicted caspase 3 cleavage product . ^^^
Interacting proteins: P42858 and P42574 Pubmed SVM Score :0.0
Huntingtin interacts with the proteins GAPDH , HAP 1 , HIP 1 , HIP 2 , and calmodulin , and a mutant huntingtin is specifically cleaved by the proapoptotic enzyme caspase 3 . ^^^
Interacting proteins: P42858 and P42574 Pubmed SVM Score :0.0
Here we report that intranuclear huntingtin induces the activation of caspase 3 and the release of cytochrome c from mitochondria in cultured cells . ^^^ We show that intranuclear huntingtin increases the expression of caspase 1 , which may in turn activate caspase 3 and trigger apoptosis . ^^^
Interacting proteins: P42858 and P42574 Pubmed SVM Score :0.0
We also identify caspase 6 and caspase 3 cleavage sites in huntingtin and demonstrate that neuronal and non neuronal cells expressing a caspase resistant huntingtin with an expanded polyglutamine tract are less susceptible to apoptosis and aggregate formation . ^^^
Interacting proteins: P42858 and P42574 Pubmed SVM Score :0.0
Chaperones inhibit caspase 3 and caspase 9 activation mediated by mutant huntingtin , and this inhibition is independent of huntingtin aggregation . ^^^
Interacting proteins: P42858 and P42574 Pubmed SVM Score :0.0
Caspase 3 cleaved N terminal fragments of wild type and mutant huntingtin are present in normal and Huntington ' s disease brains , associate with membranes , and undergo calpain dependent proteolysis . ^^^ Here , we show that N htt fragments consistent with the size produced by caspase 3 cleavage in vitro are resident in the cortex , striatum , and cerebellum of normal and adult onset HD brain and are similar in size to the fragments seen after exogenous expression of human huntingtin in mouse clonal striatal neurons . ^^^ Compared with the full length huntingtin , the caspase 3 cleaved N htt fragments , especially the mutant fragment , preferentially segregated with the membrane fraction . ^^^ Results support the idea that sequential proteolysis by caspase 3 and calpain may regulate huntingtin function at membranes and produce N terminal mutant fragments that aggregate and cause cellular dysfunction in HD . . ^^^
Interacting proteins: P42858 and P42574 Pubmed SVM Score :0.0
However , double immunostaining for huntingtin and active caspase 3 revealed that the presence of aggregates did not correlate with the presence of active caspase 3 , indicating that aggregates do not contribute to the increase in apoptosis in the N 63 82Q cells . . ^^^
Interacting proteins: P42858 and P42574 Pubmed SVM Score :0.0
However , huntingtin fragments resembled products of calpain 1 , not caspase 3 , cleavage and turnover was accompanied by augmented levels of full length normal and mutant protein . ^^^ Thus , our findings do not support a role for increased apoptosis or caspase 3 cleavage in the mechanism by which mutant huntingtin triggers disease . ^^^
Interacting proteins: P42858 and P42574 Pubmed SVM Score :0.0
Regulation of the proapoptotic activity of huntingtin interacting protein 1 by Dyrk 1 and caspase 3 in hippocampal neuroprogenitor cells . ^^^
Interacting proteins: P42858 and P42574 Pubmed SVM Score :0.0
Huntingtin fragmentation and increased caspase 3 , 8 and 9 activities in lymphoblasts with heterozygous and homozygous Huntington ' s disease mutation . ^^^
Interacting proteins: P42858 and P42574 Pubmed SVM Score :0.0
Cultured striatal neurons from yeast artificial chromosome ( YAC ) 18 transgenic mice over expressing full length wild type huntingtin were dramatically protected from apoptosis and caspase 3 activation compared with cultured striatal neurons from non transgenic FVB / N littermates and YAC 72 mice expressing mutant human huntingtin . ^^^ NMDA receptor activation induced by intrastriatal injection of quinolinic acid initiated a form of apoptotic neurodegeneration within the striatum of mice that was associated with caspase 3 cleavage of huntingtin in neurons and astrocytes , decreased levels of full length huntingtin , and the generation of a specific N terminal caspase cleavage product of huntingtin . ^^^
Interacting proteins: P42858 and P42574 Pubmed SVM Score :0.0
Expanded triplets have been found in the genes causing six different neurological disorders : fragile 10 syndrome ( FRAXA ) , spinal and bulbar muscular atrophy ( SBMA ) , myotonic dystrophy ( DM ) , Huntington ' s disease ( HD ) , spinocerebellar ataxia type 1 ( SCA 1 ) , and dentato rubra pallidoluysian atrophy ( DRPLA ) . ^^^
Interacting proteins: P42858 and P42574 Pubmed SVM Score :0.0
Three neurodegenerative diseases , Huntington ' s disease ( HD ) , Kennedy ' s disease ( hereditary spinobulbar muscular atrophy , SBMA ) , and type 1 spinocerebellar ataxia ( SCA 1 ) have been found to share a common genetic defect : an unstable region of repeated CAG trinucleotides which are thought to be translated into a polyglutamine moiety . ^^^ The unstable repeat regions occur near the N termini of the predicted proteins for HD and SBMA , but the location of the CAG repeat region is not known for SCA 1 . ^^^ These products , in part consisting of abnormally large polyglutamine moieties , act to disturb the cellular and mitochondrial milieu such that energy metabolism is impaired , rendering specific regions of the nervous system vulnerable , and resulting in the clinical phenotypes of HD , SBMA , and SCA 1 . ^^^
Interacting proteins: P42858 and P42574 Pubmed SVM Score :0.0
Trinucleotide repeats at five disease loci ( DM , DRPLA , HD , SBMA and SCA 1 ) were surveyed in phenotypically normal individuals from three continental populations . ^^^
Interacting proteins: P42858 and P42574 Pubmed SVM Score :0.0
The size of CAG repeats in the AR gene is one of the determinant factors of the severity and progression rate of SBMA phenotypes , but the meiotic and somatic instability of CAG repeats is far more stable as compared with other diseases caused by trinucleotide repeat expansions such as HD , DRPLA , MJD and SCA 1 . ^^^
Interacting proteins: P42858 and P42574 Pubmed SVM Score :0.0
An analysis of genetic fitness was performed in Huntington ' s Disease ( HD ) and Spinocerebellar Ataxia 1 ( SCA 1 ) families . ^^^ Two partially overlapping samples were used : clinically defined HD and SCA 1 patients from families ascertained in definite geographical areas , and molecularly typed carriers of HD and SCA 1 mutations ( CAG trinucleotide expansions ) . ^^^ HD and SCA 1 patients born before 1915 20 had more children than normal controls . ^^^ Carriers of HD and SCA 1 mutations , all in the low / medium expansion range ( 37 49 and 47 54 CAG repeats respectively ) , had a higher number of children than controls up to more recent times ( 1935 1950 ) . ^^^
Interacting proteins: P42858 and P42574 Pubmed SVM Score :0.0
Several human disorders are now known to be caused by expansion of unstable trinucleotide repeat sequences , including fragile 10 syndrome ( FRAX ) , myotonic dystrophy ( DM ) , spinal and bulbar muscular atrophy ( SBMA , also known as Kennedy disease ) , Huntington disease ( HD ) , dentatorubral pallidoluysian atrophy ( DRPLA ) , spinocerebellar ataxia type 1 ( SCA 1 ) , Machado Joseph disease ( MJD ) , and Friedreich ataxia . ^^^
Interacting proteins: P42858 and P42574 Pubmed SVM Score :0.0
Recent progress in the generation and characterization of transgenic mice expressing the genes containing expanded repeats associated with spinal and bulbar muscular atrophy ( SBMA ) , spinocerebellar ataxia type 1 ( SCA 1 ) , Machado Joseph disease ( MJD / SCA3 ) , and Huntington ' s disease ( HD ) is beginning to provide insight into the underlying mechanisms of these neurodegenerative disorders . . ^^^
Interacting proteins: P42858 and P42574 Pubmed SVM Score :0.0
To investigate the mitotic stability of the repetitive element in the genes for SCA 1 , SCA 3 , HD , and DRPLA we extracted DNA from up to 13 tissue samples from four deceased individuals with progressive neurological disorders and neuropathological signs . ^^^
Interacting proteins: P42858 and P42574 Pubmed SVM Score :0.0
To date there have been no studies examining the role of the ICE / Ced 3 family of proteins , in particular CPP 32 , in HD . ^^^ We examined 24 cases of HD with a classical immunophenotype and 6 cases of nodular lymphocyte predominant HD ( NLPHD ) for the expression of CPP 32 in the RS cells and lymphohistiocytic ( L & H ) cells as detected by immunohistochemistry . ^^^ Twenty two of 24 cases ( 92 % ) of HD expressed the protein in the RS cells , whereas the L & H cells in all 6 cases of NLPHD lacked expression of CPP 32 . ^^^ The differential expression of the cell death protein CPP 32 may be an important factor contributing to the apparently different clinical behaviour of NLPHD in contrast to classical HD . ^^^ Further studies are required to elucidate the significance of CPP 32 in HD . . ^^^
Interacting proteins: P42858 and P42574 Pubmed SVM Score :0.0
We have improved the RED method by the use of 8 mer oligonucleotides and assessed its usefulness in 30 samples from patients with spinocerebellar ataxia type 1 ( SCA 1 ) , Huntington ' s disease ( HD ) , and Machado Joseph ' s disease ( MJD ) , for which the number of CAG / CTG repeats was determined by sequencing . ^^^
Interacting proteins: P42858 and P42574 Pubmed SVM Score :0.0
Experiments in HD and SCA 1 transgenic mice suggest a correlation between phenotypic severity and expression of the mutant transgene . ^^^
Interacting proteins: P42858 and P42574 Pubmed SVM Score :0.0
Those negative for HD were tested for dentatorubro pallidoluysian atrophy , SCA 1 , SCA 3 , SCA 2 , SCA 6 , and other conditions as indicated . ^^^
Interacting proteins: P42858 and P42574 Pubmed SVM Score :0.0
Molecular tests were performed including genetic analysis for SCA 1 , 2 , and 3 ( spinocerebellar ataxias ) , Huntington ' s disease ( HD ) and DRPLA , due to a possible overlapping in clinical presentation . ^^^
Interacting proteins: P42858 and P42574 Pubmed SVM Score :0.0
Trinucleotide repeat expansion mutations have been identified in a number of neurodegenerative diseases , including spinal and bulbar muscular atrophy ( SBMA ) , fragile 10 syndrome ( FRAXA and FRAXE ) , myotonic dystrophy ( DM ) , Huntington ' s disease ( HD ) , spinocerebellar ataxia types 1 , 2 , 3 , 6 , 7 ( SCA 1 , SCA 2 , SCA 3 , SCA 6 , SCA 7 ) , dentatorubral pallidoluysian atrophy ( DRPLA ) , Friedreich ' s ataxia ( FRDA ) and autosomal dominant pure spastic paraplegia ( ADPSP ) . ^^^
Interacting proteins: P42858 and P42574 Pubmed SVM Score :0.0
We now report that lymphoblasts derived from HD patients showed increased stress induced apoptotic cell death associated with caspase 3 activation . ^^^
Interacting proteins: P42858 and P42574 Pubmed SVM Score :0.0
In apparently healthy , unrelated Hungarians we examined triplet repeat length polymorphism at Huntington disease ( HD ) , spinal and bulbar muscular atrophy ( SBMA ) , spinocerebellar ataxia type 1 ( SCA 1 ) , dentatorubral pallidoluysian atrophy ( DRPLA ) , and myotonic dystrophy ( MD ) loci . ^^^
Interacting proteins: P42858 and P42574 Pubmed SVM Score :0.0
Huntington ' s disease ( HD ) , spinocerebellar ataxias types 1 and 3 ( SCA 1 , SCA 3 ) , and spinobulbar muscular atrophy ( SBMA ) are caused by CAG / polyglutamine expansion mutations . ^^^
Interacting proteins: P42858 and P42574 Pubmed SVM Score :0.0
At least nine disorders result from a CAG trinucleotide repeat expansion which is translated into a polyglutamine stretch in the respective proteins : Huntington ' s disease ( HD ) , dentatorubral pallidolysian atrophy ( DRPLA ) , spinal bulbar muscular atrophy ( SBMA ) , and several of the spinocerebellar ataxias ( SCA 1 , 2 , 3 , 6 , 7 and 12 ) . ^^^
Interacting proteins: P42858 and P42574 Pubmed SVM Score :0.0
To investigate the mechanism of FAS resistance , the expression of caspase 8 was analysed by immunohistochemistry , together with that of the substrates caspase 3 and ICAD , in 52 representative samples from non Hodgkin ' s lymphoma ( NHL ) , 12 from Hodgkin ' s disease ( HD ) , and eight benign lymphoid tissues . ^^^ Ten out of the 12 HD cases lacked significant cytoplasmic staining for caspase 3 and caspase 8 in the majority of Reed Sternberg cells . ^^^
Interacting proteins: P42858 and P42574 Pubmed SVM Score :0.0
Comparison of the number of triplets in SCA 1 , MJD / SCA3 , HD , SBMA , DRPLA , MD , FRAXA and FRDA genes in schizophrenic patients and a healthy population . ^^^
Interacting proteins: P42858 and P42574 Pubmed SVM Score :0.0
Here we report the results of the analysis of five trinucleotide repeats containing genes ( SCA 1 , MJD / SCA3 , DRPLA , FRDA and MD ) in HD patients and in a group of healthy controls . ^^^ Allelic frequency distributions for SCA 1 and FRDA genes were shifted toward larger alleles in the group of unrelated HD patients , compared to healthy controls . ^^^
Interacting proteins: P42858 and P42574 Pubmed SVM Score :0.0
We have demonstrated in a transgenic HD mouse model that caspase 1 and caspase 3 are transcriptionally up regulated and activated . ^^^
Interacting proteins: P42858 and P42574 Pubmed SVM Score :0.0
We have analyzed six trinucleotide repeat containing loci [ spinocerebellar ataxias ( SCA 1 , SCA 3 , SCA 8 ) , dentatorubral pallidoluysian atrophy ( DRPLA ) , Huntington chorea ( HD ) and fragile 10 syndrome ( FRAXA ) ] in myotonic dystrophy type 1 ( DM 1 ) patients ( n = 52 ) . ^^^
Interacting proteins: P42858 and P42574 Pubmed SVM Score :0.0
The authors performed linkage analysis to APP , PS 1 , PS 2 , FTDP 17 , BRI , PI 12 , FND , HD like , SCA 4 , SCA 5 , SCA 10 , SCA 11 , SCA 13 , PARK 1 , PARK 2 , PARK 3 loci ; direct mutation analysis of HD , DRPLA , SCA 1 , SCA 2 , SCA 3 , SCA 6 , SCA 7 , SCA 8 , SCA 12 , and PRNP genes ; and sequencing of the PRNP open reading frame . ^^^
Interacting proteins: P42858 and P42574 Pubmed SVM Score :0.0
We tested this hypothesis by quantifying active caspase 3 positive tumor cells in primary biopsy specimens of HD and compared these numbers to clinical outcomes . ^^^ In conclusion , high numbers of active caspase 3 positive neoplastic cells predict a highly favorable clinical outcome in HD patients , supporting the notion that an ( at least partially ) intact apoptosis cascade is essential for the cell killing effect of chemotherapy . . ^^^
Interacting proteins: P42858 and P42574 Pubmed SVM Score :0.0
In order to understand the dynamics of the expressed single tandem repeat trinucleotides ( most of them involved in pathological expansion ) , the diversity in 10 different loci ( SCA 1 , SCA 2 , SCA 3 , SCA 6 , SCA 8 , SCA 12 , DRPLA , HD , KCNN 3 , and NCOA 3 ) was analyzed in four major human groups ( Africans , Europeans , Indians , and East Asians ) . ^^^ SCA 2 and SCA 3 follow an unrestricted stepwise mutation model , while the rest of loci are found under allele size constrictions and a bias to expansion ( SCA 1 , SCA 6 , HD , and KCNN 3 ) , contraction ( SCA 12 , DRPLA , and NCOA 3 ) , or unbiased ( SCA 8 ) . . ^^^
Interacting proteins: P42858 and P42574 Pubmed SVM Score :0.0
HT and NE , but not HD and NI , caused rapid and transient induction of caspase 3 / CPP32 activity , but not caspase 1 activity , according to the cleavage of caspase 3 substrates poly ( ADP ribose ) polymerase and D 4 GDI proteins , the appearance of cleaved caspase 3 fragments detected in HT or NE , but not in HD or NI treated HL 60 cells . ^^^
Interacting proteins: P42858 and P42574 Pubmed SVM Score :0.0
CONCLUSION : HD causes activation of cytokines , which may mediate the increase in gene expression of caspase 3 , ubiquitin , and BCKAD E 2 in skeletal muscles . . ^^^
Interacting proteins: P42858 and P42574 Pubmed SVM Score :0.0
In the present study , we investigated the neuroprotective properties of a new , reversible , caspase 3 specific inhibitor , M 826 ( 3 ( [ ( 2S ) 2 [ 5 tert butyl 3 [ [ ( 4 methyl 1 , 2 , 5 oxadiazol 3 yl ) methyl ] amino ] 2 oxopyrazin 1 ( 2H ) yl ] butanoyl ] amino ) 5 [ hexyl ( methyl ) amino ] 4 oxopentanoic acid ) , in a rat malonate model of HD . 2 . ^^^
Interacting proteins: P42858 and P42574 Pubmed SVM Score :0.0
In a rat model of Huntington ' s disease ( HD ) in which an excitotoxin is unilaterally infused into the striatum , both long and short term pretreatment with lithium reduces DNA damage , caspase 3 activation , and loss of striatal neurons . ^^^
Interacting proteins: P42858 and P42574 Pubmed SVM Score :0.0
No significant differences were observed between D HD , HD and CRF sera on apoptosis rate , caspase 3 activity and phagocytic capacity of U 937 monocytes . ^^^
Interacting proteins: P42858 and P42574 Pubmed SVM Score :0.0
Caspase 3 , an executioner protease , was only found in four HD brains of different grades and was not expressed in controls . ^^^ However , the presence of late apoptotic events , such as enhanced PARP expression and many TUNEL positive cells accompanied with weak caspase 3 immunoreactivity in severely affected HD brains , suggests that caspase mediated neuronal death only plays a minor role in HD . . ^^^
Interacting proteins: P42858 and P42574 Pubmed SVM Score :0.0
In addition , upon exposure to toxic stimuli , increased mitochondrial release of cytochrome C and activation of caspase 9 and caspase 3 are found in HD cells and tissue . ^^^
Interacting proteins: P42858 and P42574 Pubmed SVM Score :0.0
To determine whether caspase cleavage of htt is a key event in the neuronal dysfunction and selective neurodegeneration in HD , we generated YAC mice expressing caspase 3 and caspase 6 resistant mutant htt . ^^^
Interacting proteins: P42858 and P42574 Pubmed SVM Score :0.0
Additionally , we define htt itself as an important caspase substrate by generating a site directed htt mutant that is resistant to caspase 3 cleavage at positions 513 and 530 and to caspase 6 cleavage at position 586 . ^^^