| Interacting proteins: P10515 and Q02218 |
Pubmed |
SVM Score :0.0 |
| We have used recombinant or purified preparations from the 2 oxo acid dehydrogenase enzyme complexes , the major mitochondrial autoantigens in PBC ( PDC E 2 , BCOADC E 2 , OGDC , protein 10 and PDC E 1 alpha ) to compare the reactivity of sera from either similarly staged sera from Japanese ( n = 23 ) or American Caucasian patients ( n = 39 ) with PBC . ^^^ |
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| Interacting proteins: P10515 and Q02218 |
Pubmed |
SVM Score :0.0 |
| This was shown as follows : ( 1 ) PBC MoAb , like PBC AMA , completely inhibited PDH enzyme activity and reacted weakly with OGDH ; ( 2 ) PBC MoAb bound strongly to the E 2 subunit in western blots , with weaker binding to a doublet of about 56 kDa ; and ( 3 ) in immunosorbent experiments , PBC MoAb absorbed most ( greater than 95 % ) of the AMA reactive material found in solubilized mitochondria . ^^^ |
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| Interacting proteins: P10515 and Q02218 |
Pubmed |
SVM Score :0.0 |
| Anti M 2 antibodies in primary biliary cirrhosis ( PBC ) have been shown to react with the alpha ketoacid dehydrogenase complex of the inner mitochondrial membrane consisting of six epitopes ( E 2 subunit of the pyruvate dehydrogenase complex ( PDC ) , 70 kD ; protein 10 of the PDC , 56 kD ; alpha ketoglutarate dehydrogenase complex , 52 kD ; branched chain alpha ketoacid dehydrogenase , 52 kD ; E 1 alpha subunit of PDC , 45 kD ; and E 1 beta subunit of PDC , 36 kD ) . ^^^ |
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| Interacting proteins: P10515 and Q02218 |
Pubmed |
SVM Score :0.0 |
| We established one Epstein Barr virus transformed B cell hybrid clone producing human mAb of the IgG class to the 2 oxoglutarate dehydrogenase complex ( OGDC ) for the first time from the peripheral B lymphocytes of a patient with primary biliary cirrhosis ( PBC ) . ^^^ This human mAb to OGDC , which was established for the first time from a patient with PBC and characterized at the molecular level , would be a valuable tool to study the B cell autoepitopes of OGDC , to clone as yet undetermined full length cDNA encoding OGDC and to dissect the autoimmune response to mitochondrial antigens in PBC . . ^^^ |
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| Interacting proteins: P10515 and Q02218 |
Pubmed |
SVM Score :0.0 |
| Five different target mitochondrial autoantigens recognized by sera from patients with primary biliary cirrhosis ( PBC ) have been identified as subunits of the following 2 oxo acid dehydrogenase complexes : the pyruvate dehydrogenase complex ( PDC ) , the branched chain 2 oxo acid dehydrogenase complex ( BCOADC ) , and the 2 oxoglutarate dehydrogenase complex ( OGDC ) . ^^^ Unlike the E 2 subunits of PDC ( PDC E 2 ) and BCOADC ( BCOADC E 2 ) , the E 2 subunits of OGDC ( OGDC E 2 ) reactivity of PBC sera and the reactive epitope of OGDC D 2 have not hitherto been studied in detail . ^^^ Eighty of 268 ( 29 . 9 % ) PBC patient sera but none of the 45 controls reacted with recombinant OGDC E 2 . ^^^ The recombinant OGDC E 2 was judged to express the immunodominant epitope , because when sera from patients with PBC were preabsorbed with the recombinant fusion protein , such sera were depleted of reactivity against 48 kD OGDC E 2 when probed on beef heart mitochondria ( BHM ) but retained reactivity toward PDC E 2 and / or BCOADC E 2 . ^^^ Furthermore , affinity purified PBC sera against recombinant OGDC E 2 reacted only with native OGDC E 2 and not with any other enzyme components of the 2 oxo acid dehydrogenase complex . ^^^ |
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| Interacting proteins: P10515 and Q02218 |
Pubmed |
SVM Score :0.0 |
| Although 80 % to 90 % of sera from patients with PBC react to PDC E 2 , approximately 10 % patients with PBC react only to BCOADC E 2 and / or OGDC E 2 . ^^^ Of 34 PBC sera that did not react with recombinant PDC E 2 , 18 sera contained BCOADC E 2 specific AMA and 1 serum possessed only OGDC E 2 specific AMA . ^^^ |
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| Interacting proteins: P10515 and Q02218 |
Pubmed |
SVM Score :0.0 |
| Primary biliary cirrhosis ( PBC ) is associated with a high incidence of autoantibodies directed at mitochondrial autoantigens ( the antimito chondrial antibodies ) , identified as the E 2 components of PDC , OGDC , and BCOADC , together with protein 10 and the E 1 alpha and E 1 beta subunits of PDC . ^^^ |
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| Interacting proteins: P10515 and Q02218 |
Pubmed |
SVM Score :0.0 |
| Anti mitochondrial antibodies ( AMA ) , including antibodies against pyruvate dehydrogenase complex , branched chain alpha ketoacid dehydrogenase complex and alpha ketoglutarate dehydrogenase complex , were not detected by enzyme linked immunosorbent assay in five of the 113 ( 4 . 4 % ) patients with PBC . ^^^ |
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| Interacting proteins: P10515 and Q02218 |
Pubmed |
SVM Score :0.0 |
| The porcine PDC E 2 and the dihydrolipoamide succinyltransferase ( OGDC E 2 ) component of the porcine 2 oxoglutarate dehydrogenase complex ( OGDC ) were identified as mitochondrial autoantigen with sera from patients with primary biliary cirrhosis ( PBC ) . ^^^ Enzymatic delipoylation and relipoylation of the complexed and free PDC E 2 and OGDC E 2 components did not influence immunoreactivity with PBC sera . . ^^^ |
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| Interacting proteins: P10515 and Q02218 |
Pubmed |
SVM Score :0.0 |
| The target autoantigens for the AMA were identified as pyruvate dehydrogenase , branched chain alpha ketoacid dehydrogenase , alpha ketoglutarate dehydrogenase , protein 10 and pyruvate dehydrogenase E 1 alpha in all 5 cases , PBC was confirmed by means a liver biopsy and endoscopic retrograde cholangioscopy . ^^^ |
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| Interacting proteins: P10515 and Q02218 |
Pubmed |
SVM Score :0.0 |
| The characteristic serological feature of primary biliary cirrhosis ( PBC ) is the presence of antimitochondrial antibodies ( AMAs ) , and the major proteins recognized by AMAs are subunits of the 2 oxo acid dehydrogenase complexes ( 2 OADC ) , including the E 2 components of the pyruvate dehydrogenase complex ( PDC ) , the 2 oxo glutarate dehydrogenase complex ( OGDC ) , the branched chain 2 oxoacid dehydrogenase complex ( BCOADC ) , the E 3 binding protein ( E3BP or protein 10 ) and the E1a component of mammalian PDC . ^^^ To address this issue , we performed in situ hybridization for all of the major 2 OADC components at the mRNA level , including PDC E 2 , BCOADC E 2 , OGDC E 2 , PDC E1a , BCOADC E1a , OGDC E 1 , and E3BP using 13 PBC and 9 control livers using 7 mitochondrial antisense probes . ^^^ |
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| Interacting proteins: P10515 and Q02218 |
Pubmed |
SVM Score :0.0 |
| These include the E 2 components of PBC , BCKD and OGDC . ^^^ |
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| Interacting proteins: P10515 and Q02218 |
Pubmed |
SVM Score :0.0 |
| The anti mitochondrial antibody response in primary biliary cirrhosis ( PBC ) is primarily directed at E 2 components of PDC , OGDC , and BCOADC , and E3BP . ^^^ |
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| Interacting proteins: P10515 and Q02218 |
Pubmed |
SVM Score :0.0 |
| For the development of a `` complete ' ' or `` gold standard ' ' diagnostic assay for PBC , similar assays of the enzyme inhibition for anti 2 oxoglutarate dehydrogenase complex ( OGDC ) and anti branched chain oxo acid dehydrogenase complex ( BCOADC ) antibodies will be needed in future . . ^^^ |
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| Interacting proteins: P10515 and Q02218 |
Pubmed |
SVM Score :0.0 |
| The sera were assayed by immunofluorescence on frozen tissue sections , immunoblotting on bovine heart mitochondria and recombinant E 2 subunit of branched chain oxo acid dehydrogenase complex ( BCOADC E 2 ) , ELISA using recombinant E 2 subunit of human pyruvate dehydrogenase complex ( PDC E 2 ) and purified porcine 2 oxoglutarate dehydrogenase complex ( OGDC ) , and enzyme inhibition assay using procine PDC and OGDC . ^^^ |
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| Interacting proteins: P10515 and Q02218 |
Pubmed |
SVM Score :0.0 |
| Four of the 13 mAbs recognized the inner lipoyl domain of PDC E 2 , 2 of 13 recognized the entire PDC E 2 molecule , 4 of 13 recognized PDC E 2 and OGDC E 2 , 1 of 13 recognized OGDC only , 1 recognized BCOADC E 2 only , and 1 recognized an unidentified 100 kd mitochondrial protein . ^^^ |
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| Interacting proteins: P10515 and Q02218 |
Pubmed |
SVM Score :0.0 |
| One infant also reacted with outer lipoyl domain PDC E 2 ( 35 49 ) , and 2 oxoglutarate dehydrogenase complex ( OGDC ) E 2 ( 99 113 ) . ^^^ |
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| Interacting proteins: P10515 and Q02218 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: P10515 and Q02218 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: P10515 and Q02218 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: P10515 and Q02218 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: P10515 and Q02218 |
Pubmed |
SVM Score :0.0 |
| NA |
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