Abstract |
Pemphigoid (Pg) is an autoimmune subepidermal blistering disease that affects the elderly population. The phenotype can be Bullous Pemphigoid (BP), which primarily involves the skin, or Mucous Membrane Pemphigoid ( MMP), which primarily involves mucus membranes. Ocular Cicatricial Pemphigoid (OCP) and Oral Pemphigoid (OP) are subsets of MMP. The known antigens in BP are Bullous Pemphigoid Antigen 1 (BPAG1, also known as BP230), Bullous Pemphigoid Antigen 2 (BPAG2, also known as BP180), and subunits of human integrins α6 and β4. The Human Leukocyte Antigen (HLA) allele HLA-DQβ1*0301 has been reported to be associated with enhanced susceptibility to all of these subsets. Sera of patients with the four subsets are characterized by the presence of anti-Basement Membrane Zone (anti-BMZ) antibodies. In this manuscript, we present a model in which relevant portions of the four different antigens involved in pemphigoid have potential sites that could be presented by an antigen presenting cell (APC) in conjunction with DQβ1*0301 to a T cell receptor to initiate the process that results in anti-BMZ antibody production. Thus, this model provides a hypothetical computer-based mechanism to explain how a single HLA allele can be associated with the production of antibodies to four different antigens that result in four different subsets of a disease with four different clinical profiles and prognoses.
|
Authors | L R Zakka, P Reche, A R Ahmed |
Journal | Autoimmunity reviews
(Autoimmun Rev)
Vol. 11
Issue 1
Pg. 40-7
(Nov 2011)
ISSN: 1873-0183 [Electronic] Netherlands |
PMID | 21782980
(Publication Type: Journal Article, Review)
|
Copyright | Copyright © 2011 Elsevier B.V. All rights reserved. |
Chemical References |
- Autoantibodies
- Autoantigens
- Carrier Proteins
- Cytoskeletal Proteins
- DST protein, human
- Dystonin
- HLA-DQ Antigens
- HLA-DQ7 antigen
- Nerve Tissue Proteins
- Non-Fibrillar Collagens
- collagen type XVII
|
Topics |
- Amino Acid Sequence
- Autoantibodies
(blood)
- Autoantigens
(immunology, metabolism)
- Binding Sites
- Carrier Proteins
(immunology, metabolism)
- Cytoskeletal Proteins
(immunology, metabolism)
- Dystonin
- Enzyme-Linked Immunosorbent Assay
- Genes, MHC Class II
(genetics)
- HLA-DQ Antigens
(genetics, metabolism)
- Humans
- Molecular Sequence Data
- Nerve Tissue Proteins
(immunology, metabolism)
- Non-Fibrillar Collagens
(immunology, metabolism)
- Pemphigoid, Benign Mucous Membrane
(genetics, immunology, pathology)
- Pemphigoid, Bullous
(genetics, immunology, pathology)
|