Catalog No.
AID00701
Description
Bullous pemphigoid is the result of an attack on the basement membrane of the epidermis by IgG +/- IgE immunoglobulins (antibodies) and activated T lymphocytes (white blood cells). The target is the protein BP180 (also called Type XVII collagen), or less frequently BP230 (a plakin). These proteins are within the NC16A domain of collagen XVII. They are associated with the hemidesmosomes, structures that ensure the epidermal keratinocyte cells stick to the dermis to make a waterproof seal. The binding of the autoantibodies to the proteins and release of cytokines from the T cells lead to complement activation, recruitment of neutrophils (acute inflammatory cells) and the release of proteolytic enzymes. These destroy the hemidesmosomes and cause the formation of subepidermal blisters.
Expression system
Confidential
Species
Homo sapiens (Human)
Protein length
Confidential
Nature
Recombinant
Endotoxin level
Please contact with the lab for this information.
Purity
>95% as determined by SDS-PAGE.
Accession
Q9UMD9
Applications
As a diagnostic tool for bullous pemphigoid.
Form
Lyophilized
Storage buffer
Lyophilized from a solution in PBS pH 7.4, 0.02% NLS, 1mM EDTA, 4% Trehalose, 1% Mannitol.
Reconstitution
Reconstitute in sterile water for a stock solution.
Shipping
In general, proteins are provided as lyophilized powder/frozen liquid. They are shipped out with dry ice/blue ice unless customers require otherwise.
Stability and Storage
Use a manual defrost freezer and avoid repeated freeze thaw cycles. Store at 2 to 8°C for frequent use. Store at -20 to -80°C for twelve months from the date of receipt.
Alternative Names
Linear IgA disease antigen 1, COL17A1, 180 kDa bullous pemphigoid antigen 2, LAD-1, Linear IgA bullous disease antigen of 97 kDa, 120 kDa linear IgA dermatosis antigen, 97-LAD, Collagen alpha-1(XVII) chain, Bullous pemphigoid antigen 2, 97 kDa linear IgA bullous dermatosis antigen, LABD97, 97 kDa LAD antigen, BPAG2, BP180
Aberrant glycosylation patterns as potential biomarkers for diagnosis and disease progression in bullous pemphigoid., PMID:40491922
Dupilumab in the Treatment of Pemphigoid Gestationis., PMID:40315477
[A Case of Bullous Pemphigoid after Combined Chemotherapy of Durvalumab, Gemcitabine and Cisplatin for Intrahepatic Cholangiocarcinoma]., PMID:40073898
Clinical Outcomes and Prognostic Factors in Bullous Pemphigoid Patients: A 15-Year Review in China., PMID:40059256
Localization of Lesions in Autoimmune Blistering Diseases Is Independent of Site-Specific Target Antigen Expression., PMID:40003627
Periostin in Bullous Pemphigoid: A Potential Biomarker of Disease Activity and Severity., PMID:39989295
Bullous pemphigoid., PMID:39979318
Research in practice: Immune checkpoint inhibitor related autoimmune bullous dermatosis., PMID:39945070
Diagnostic methods and strategies for autoimmune bullous diseases., PMID:39930606
Correlation of BP180, BP230, and type VII collagen antibody titers in serum, blister fluid, erosion, and saliva in pemphigoid diseases., PMID:39916594
Linear IgA bullous dermatosis-a fifty year experience of Warsaw Center of bullous diseases., PMID:39877369
Clinical characteristics of bullous pemphigoid patients of different ages and the possible mechanism., PMID:39821084
C4d immunostaining facilitates differentiation of pemphigoid nodularis from prurigo nodularis., PMID:39755047
Improvement of immunological tests for detecting autoantibodies in patients with lamina lucida-type linear IgA bullous dermatosis., PMID:39732612
Dupilumab, corticosteroids and their combination for the treatment of bullous pemphigoid., PMID:39694724
Bullous pemphigoid and mucous membrane pemphigoid humoral responses differ in reactivity towards BP180 midportion and BP230., PMID:39676877
Use of multivariant enzyme-linked immunosorbent assay (ELISA) in the diagnosis of autoimmune bullous disorders in a resource-limited setting: A single-center experience., PMID:39635793
Enriched class II HLA inherence in patients with checkpoint inhibitor-associated bullous pemphigoid., PMID:39592443
Ocular Mucous Membrane Pemphigoid Demonstrates a Distinct Autoantibody Profile from Those of Other Autoimmune Blistering Diseases: A Preliminary Study., PMID:39584991
The "entanglement" between bullous pemphigoid and diabetes mellitus: a comprehensive review and expert recommendations., PMID:39521622
Eosinophil extracellular traps drive T follicular helper cell differentiation via VIRMA-dependent MAF stabilization in bullous pemphigoid., PMID:39490750
Clinical manifestation variability of bullous pemphigoid., PMID:39487837
Serum interleukin-10 level is increased and correlated positively with disease severity in patients with dipeptidyl peptidase-4 inhibitor-related bullous pemphigoid., PMID:39428667
Pathophysiology of Bullous Pemphigoid: Role of Type 2 Inflammation and Emerging Treatment Strategies (Narrative Review)., PMID:39425892
A Case of Acquired Factor V Inhibitor During Bullous Pemphigoid Treatment., PMID:39391459
Perinatal Outcome of Pemphigoid Gestationis: A Report of Three Cases and Review of the Literature., PMID:39371863
Serum inflammatory biomarkers associated with disease severity and response to dupilumab treatment in bullous pemphigoid: A cluster analysis., PMID:39358056
Bullous Pemphigoid Severity and Levels of Antibodies to BP180 and BP230: A Systematic Review and Meta-Analysis., PMID:39356527
The possible and intriguing relationship between bullous pemphigoid and melanoma: speculations on significance and clinical relevance., PMID:39267741
Polarization of circulating follicular helper T cells correlates with bullous pemphigoid severity., PMID:39255051
The Role of Serology in the Diagnosis of Ocular Predominant Mucous Membrane Pemphigoid and the Search for an Ocular-Specific Autoantigen., PMID:39241171
Isolation and analysis of the exosomal membrane proteins in bullous pemphigoid., PMID:39230197
Autoantigen-specific CD4+ T cells acquire an exhausted phenotype and persist in human antigen-specific autoimmune diseases., PMID:39226901
Diagnostic challenge: Juvenile bullous pemphigoid with a negative BP180 ELISA., PMID:39168501
Serum Biomarkers in Bullous Pemphigoid: A Systematic Review., PMID:39075716
Single-cell transcriptomics analysis of bullous pemphigoid unveils immune-stromal crosstalk in type 2 inflammatory disease., PMID:39009587
Reactivity against the BP180 ectodomain in patients with bullous pemphigoid, mucous membrane pemphigoid, multiple sclerosis and Parkinson disease., PMID:38946225
Pemphigus vegetans with autoantibodies against desmocollin and BP180., PMID:38907556
State-of-the-art diagnosis of autoimmune blistering diseases., PMID:38903493
Absence of Epidermal Antibodies in Stevens-Johnson Syndrome/Toxic Epidermal Necrolysis Patients but Beware of Single Positive Results., PMID:38803350
Acute myeloid leukemia with paraneoplastic pemphigus successfully treated with a personalized antileukemic and immunosuppressive strategy., PMID:38780802
Collagen 17A1 in the Urothelium Regulates Epithelial Cell Integrity and Local Immunologic Responses in Obstructive Uropathy., PMID:38768778
Study of original B cells producing pathogenic IgG and IgA autoantibodies in anti-BP180-type mucous membrane pemphigoid., PMID:38734797
Potential correlation between anti-laminin 332 autoantibodies and malignant tumours in anti-BP180-type mucous membrane pemphigoid., PMID:38634807
Anti-BP230 IgE autoantibodies in bullous pemphigoid intraindividually correlate with disease activity., PMID:38582700
Targeting antibody-mediated complement-independent mechanism in bullous pemphigoid with diacerein., PMID:38508975
A case of bullous pemphigoid following administration of anti-IL-31 receptor A antibody., PMID:38507442
IgE anti-BP180 NC16A autoantibody in both serum and blister fluid samples does not correlate with disease activity of bullous pemphigoid., PMID:38465539
Oral mucous membrane pemphigoid: updates in diagnosis and management., PMID:38388600
Expression of Serum Anti-BP180/230 Antibodies in Bullous Pemphigoid Patients Combined with Nervous System Diseases and Relevant Factor Analysis., PMID:38342861
For research use only. Not for human or drug use.
