| Interacting proteins: Q9UMD9 and P16144 |
Pubmed |
SVM Score :0.0 |
| NA |
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| Interacting proteins: Q9UMD9 and P16144 |
Pubmed |
SVM Score :0.0 |
| Immunofluorescence analysis showed that the beta 4 subunit was absent in the skin of the PA JEB patient , whereas the alpha 6 subunit appeared to be normally distributed along the basement membrane zone , as were the other hemidesmosomal components BP 230 , BP 180 and HD 1 . ^^^ The few hemidesmosome like structures found in cultured keratinocytes from the PA JEB patient contained the hemidesmosomal components BP 230 , BP 180 and HD 1 , but not the integrin alpha 6 subunit . ^^^ |
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| Interacting proteins: Q9UMD9 and P16144 |
Pubmed |
SVM Score :0.0 |
| We report here that these cells possess HD 1 but not BP 230 or BP 180 ; two other hemidesmosomal constituents , and express only traces of the alpha 6 beta 4 integrin . ^^^ |
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| Interacting proteins: Q9UMD9 and P16144 |
Pubmed |
SVM Score :0.0 |
| Our results show that the mutated beta 4 subunit associates with integrin alpha 6 , but the resulting heterodimer does not induce nucleation of the bullous pemphigoid antigens BP 180 and BP 230 , and that of the inner plaque component plectin / HD1 , into hemidesmosomal structures . ^^^ The integrity of the cytoplasmic tail of integrin beta 4 seems to be essential to the targeting and stabilization of plectin / HD1 and BP 180 in HD , because transfection of a recombinant wild type B 4 cDNA in the delta 17 beta 4 PA JEB keratinocytes restores the synthesis of a functional alpha 6 / beta 4 heterodimer , which promotes the polarization of plectin / HD1 and BP 180 , to the basal aspect of the cells . ^^^ Because in the transfected keratinocytes the distribution of BP 230 remains diffuse in the cytoplasm , we suggest that the interaction between plectin / HD1 and integrin alpha 6 beta 4 , followed by the association with BP 180 , constitutes the first step in the nucleation of the HD . . ^^^ |
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| Interacting proteins: Q9UMD9 and P16144 |
Pubmed |
SVM Score :0.0 |
| We tested this notion by monitoring the fate of green fluorescent protein ( GFP ) tagged human integrin beta 4 subunit ( GFP hbeta 4 ) and GFP tagged 180 kD human bullous pemphigoid ( BP ) autoantigen ( GFP BP 180 ) in live cultures of 804G cells that assemble numerous mature hemidesmosomes . ^^^ In these migratory cells , which lack mature hemidesmosomes , integrin beta 4 subunit and BP 180 protein clusters progressively assemble and disassemble into linear and cat paw arrays . ^^^ |
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| Interacting proteins: Q9UMD9 and P16144 |
Pubmed |
SVM Score :0.0 |
| The 230 kD bullous pemphigoid antigen ( BPAG 1 ) gene expression was also reduced by RA , whereas transcript levels of integrin alpha 6 , integrin beta 4 , the 180 kD bullous pemphigoid antigen ( BPAG 2 ) , and laminin 5 were not affected . ^^^ |
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| Interacting proteins: Q9UMD9 and P16144 |
Pubmed |
SVM Score :0.0 |
| Previously , mutations in this condition have been described in the three genes for the anchoring filament protein laminin 5 ( LAMA 3 , LAMB 3 , and LAMC 2 ) , in the gene encoding the hemidesmosome associated beta 4 integrin ( ITGB 4 ) , and in the gene for the hemidesmosomal protein type 17 collagen ( COL17A1 / BPAG2 ) . ^^^ |
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