| Interacting proteins: O00515 and Q9UMD9 |
Pubmed |
SVM Score :0.0 |
| In contrast to bullous pemphigoid , in which autoantibodies recognize transmembrane type 17 collagen ( BP 180 , BPAG 2 ) , LAD is associated with skin fixed and circulating IgA autoantibodies that target LAD 1 , the processed extracellular form of type 17 collagen . ^^^ |
|
| Interacting proteins: O00515 and Q9UMD9 |
Pubmed |
SVM Score :0.0 |
| With the combined use of the soluble BP 180 ectodomain ( LAD 1 ) and recombinant BP 180 NC16A , 16 of these 19 CP sera ( 84 % ) targeted BP 180 . ^^^ |
|
| Interacting proteins: O00515 and Q9UMD9 |
Pubmed |
SVM Score :0.0 |
| Immunoblotting showed that the patient ' s serum reacted with the NC 1 domain of type 7 collagen ( 290 kDa epidermolysis bullosa acquisita antigen ) as well as the 120 kDa linear IgA bullous dermatosis antigen , LAD 1 . ^^^ |
|
| Interacting proteins: O00515 and Q9UMD9 |
Pubmed |
SVM Score :0.0 |
| The 97 kDa linear IgA bullous dermatosis antigen is not expressed in a patient with generalized atrophic benign epidermolysis bullosa with a novel homozygous G258X mutation in COL17A1 . ^^^ The nature and expression pattern of the 97 kDa linear IgA bullous dermatosis antigen ( LAD 1 ) and its role in epidermolysis bullosa have not been fully elucidated . ^^^ |
|
| Interacting proteins: O00515 and Q9UMD9 |
Pubmed |
SVM Score :0.0 |
| To this end , 44 patients with a variety of subtypes of JEB were analyzed by indirect immunofluorescence microscopy with antibodies to LAD 1 , BP 180 , and laminin 5 . ^^^ Patients who showed LAD 1 defects also showed abnormal expression of BP 180 . ^^^ One culture demonstrated defects of BP 180 and LAD 1 expression ( which was also verified by radioimmunoprecipitation assay ) , and one culture showed decreased laminin 5 expression but normal BP 180 and LAD 1 expression . ^^^ Thus , these studies demonstrate that : ( 1 ) LAD 1 and BP 180 are normally expressed in all subtypes of JEB except GABEB , ( 2 ) the majority of GABEB patients show absent or near absent expression of both LAD 1 and BP 180 but normal expression of laminin 5 , and ( 3 ) a smaller subset of GABEB patients show normal LAD 1 and BP 180 expression but express persistent but reduced levels of laminin 5 . . ^^^ |
|
| Interacting proteins: O00515 and Q9UMD9 |
Pubmed |
SVM Score :0.0 |
| Type 17 collagen ( BP 180 ) and LAD 1 are present as separate trimeric complexes . ^^^ The 120 kDa linear IgA dermatosis antigen ( LAD 1 ) is an collagenous anchoring filament protein with homology to the extracellular collagenous part of BP 180 . ^^^ Two dimensional immunoblotting showed that LAD 1 , as BP 180 , is also present as a high molecular mass complex and does not form mixed complexes with BP180 . . ^^^ |
|
| Interacting proteins: O00515 and Q9UMD9 |
Pubmed |
SVM Score :0.0 |
| Eight of the linear IgA disease sera that were reactive with NC16A also recognized LAD 1 secreted by the SCC 25 cell line , and five sera recognized BP 180 extracted from keratinocytes . ^^^ Linear IgA disease sera depleted of reactivity to NC16A by immunoadsorption continued to react with both the LAD 1 antigen and BP 180 by immunoblotting and with the basement membrane zone by indirect immunofluorescence microscopy . ^^^ |
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| Interacting proteins: O00515 and Q9UMD9 |
Pubmed |
SVM Score :0.0 |
| By immunoblot analysis , the patient ' s IgA reacted with the soluble ectodomain of BP 180 ( LAD 1 ) . ^^^ |
|
| Interacting proteins: O00515 and Q9UMD9 |
Pubmed |
SVM Score :0.0 |
| Our patient ' s IgG and IgA autoantibodies also reacted with full length BP 180 derived from epidermal extracts and the ectodomain of BP 180 ( LAD 1 ) derived from culture supernatant of keratinocytes . ^^^ |
|
| Interacting proteins: O00515 and Q9UMD9 |
Pubmed |
SVM Score :0.0 |
| Finally , the epidermal 97 kDa and the keratinoctye derived 120 kDa autoantigens of LAD ( LABD 97 and LAD 1 , respectively ) have recently been identified as portions of the BP 180 ectodomain . ^^^ |
|
| Interacting proteins: O00515 and Q9UMD9 |
Pubmed |
SVM Score :0.0 |
| In addition , we found strong reactivity with recombinant NC16A , an immunodominant region of BP 180 targeted in the majority of BP sera , whereas no antibodies against the keratinocyte derived soluble BP 180 ectodomain ( LAD 1 ) or the recombinant intracellular domain of BP 180 were detected . ^^^ |
|
| Interacting proteins: O00515 and Q9UMD9 |
Pubmed |
SVM Score :0.0 |
| A normal proteolytic processing event results in the shedding of the BP 180 extracellular domain ( LAD 1 ) from the keratinocyte cell surface . ^^^ |
|
| Interacting proteins: O00515 and Q9UMD9 |
Pubmed |
SVM Score :0.0 |
| RESULTS : Immunoblot assay revealed that the majority of MMP patients had IgG ( 93 of 124 , 75 % ) and / or IgA autoantibodies ( 63 of 124 , 51 % ) to BP 180 ( including its soluble ectodomains , 120 kDa LAD 1 and 97 kDa LABD 97 antigens ) . ^^^ |
|
| Interacting proteins: O00515 and Q9UMD9 |
Pubmed |
SVM Score :0.0 |
| We found reactivity to the extracellular fragment of BP 180 ( LAD 1 ) in 3 patients , 2 of them via IgA and 1 with IgG . ^^^ |
|
| Interacting proteins: O00515 and Q9UMD9 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: O00515 and Q9UMD9 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: O00515 and Q9UMD9 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: O00515 and Q9UMD9 |
Pubmed |
SVM Score :0.0 |
| NA |
|