| Interacting proteins: P55072 and P13569 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: P55072 and P13569 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: P55072 and P13569 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: P55072 and P13569 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: P55072 and P13569 |
Pubmed |
SVM Score :0.0 |
| The most common disease causing protein folding mutation , DeltaF 508 cystic fibrosis transmembrane regulator ( CFTR ) , is destroyed by ERAD to cause cystic fibrosis ( CF ) . p97 / valosin containing protein ( VCP ) physically interacts with gp78 / autocrine motility factor receptor to couple ubiquitination , retrotranslocation , and proteasome degradation of misfolded proteins . ^^^ Immunoprecipitation of DeltaF 508 CFTR from primary CF bronchial epithelial cells confirmed the interaction with VCP and associated chaperones in CF . ^^^ We conclude that VCP is an integral component of ERAD and cellular stress pathways induced by the unfolded protein response and may be central to the efficacy of CF drugs that target the ubiquitin proteasome pathway . . ^^^ We show here that p97 / VCP and gp 78 form complexes with CFTR during translocation from the ER for degradation by the cytosolic proteasome . ^^^ Interference in the VCP CFTR complex promoted accumulation of immature CFTR in the ER and partial rescue of functional chloride channels to the cell surface . ^^^ |
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