HLA-DR4
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| Image:DR Illustration.PNG | |
| human major histocompatibility complex, class II, DR4
| |
| Haplotypes groups | DRA*01:DRB1*0401 to DRA*01:DRB1*0413 |
| Structure (See HLA-DR) | |
| Identifiers | alpha *0101
|
| Symbol(s) | HLA-DRA |
| EBI-HLA | DRA*0101 |
| Identifiers | beta 1 *0401 to *0413
|
| Symbol(s) | HLA-DRB1 |
| EBI-HLA | DRB1*0401 |
| EBI-HLA | DRB1*0402 |
| EBI-HLA | DRB1*0403 |
| EBI-HLA | DRB1*0404 |
| EBI-HLA | DRB1*0405 |
| EBI-HLA | DRB1*0406 |
| EBI-HLA | DRB1*0407 |
| EBI-HLA | DRB1*0408 |
| EBI-HLA | DRB1*0409 |
| EBI-HLA | DRB1*0410 |
| EBI-HLA | DRB1*0411 |
| EBI-HLA | DRB1*0412 |
| EBI-HLA | DRB1*0413 |
| Shared data | |
| Locus | chr.6 6p21.31 |
HLA-DR4 (DR4) is a HLA-DR serotype that recognizes the DRB1*04 gene products. The DR4 serogroup is large and has a number of moderate frequency alleles spread over large regions of the world.
Serology
| DRB1* | DR4 | Sample |
| allele | % | size (N) |
| 0401 | 98 | 5118 |
| 0402 | 92 | 1029 |
| 0403 | 92 | 748 |
| 0404 | 89 | 2164 |
| 0405 | 91 | 505 |
| 0406 | 89 | 83 |
| 0407 | 86 | 846 |
| 0408 | 86 | 258 |
| 0409 | 95 | 13 |
| 0410 | 84 | 29 |
| 0411 | 93 | 76 |
| 0412 | >50 | 1 |
| 0413 | >30 | 3 |
The serological identification of DR4 is good. The serology of DRB1*0417 to *0460
is unknown.
Disease associations
By serotype
DR4 is associated extraarticular[1]rheumatoid arthritis[1], hydralazine-induced female systemic lupus erythematosus[1],pemphigoid gestationis[1], phemphigus foliaceus[1], obstructive hypertrophic cardiomyopathy[1], IgA nephropathy[1] and 'shared syndrome'-systemic schlerosis/rheumatoid arthritis.[1]
By allele
DRB1*04 is associated with increased risk for alopecia areata.[1]
DRB1*0401 is associated with multiple sclerosis[1], rheumatoid arthritis[1], type 1 diabetes[1][1], lyme disease induced arthritis[1]
DRB1*0402: drug-triggered[1]/idiopathic pemphigus vulgaris[1][1], type 1 diabetes[1], SLE associated anti-cardiolipin and anti-β2 glycoprotein I[1]
DRB1*0403: polycystic ovary syndrome[1], SLE associated anti-cardiolipin and anti-β2 glycoprotein I[1]
DRB1*0404: anti-citrullinated fibrinogen[1] in rheumatoid arthritis[1], autoimmune hepatitis[1]
DRB1*0405: rheumatoid arthritis[1], Autoimmune hepatitis[1], type 1 diabetes[1][1]
DRB1*0406: caspase-8 autoantibodies silicosis-systemic sclerosis (SSc)-systemic lupus erythematosus (SLE)[1]
DRB1*0409: T. cruzi infection with cardiomyopathy[1]
By haplotype
DR4-DQ8 is a risk factor for papillary thyroid carcinoma.[1]
DRB1*04:DQA1*0303-DQB1*0401 haplotype: type III autoimmune polyglandular syndrome[1], autoimmune hepatitis [1], autoimmune pancreatitis[1]
DRB1*04:DQA1*03-DQB1*0302 haplotype: Type 1 diabetes[1][1] with DRB1*0401, *0405, *0402 increasing risk when DQ8 is present.[1]
By genotype
DRB1*0101/*0404 and *0101/*0401 increases risk of mortality in rheumatoid arthritis, with eschemic heart disease and smoking.[1] these same genotypes are associated with rheumatoid vasculitis.[1]
By haplotype
HLA DR4-DQ8 juvenile diabetes, coeliac disease, rheumatoid arthritis[1]
DRB1*04 allele group 68 Alleles: 60 proteins DR4 Serotype: *0401 to *0416 Serotype unknown: *0417 to *460
Genetic Linkage
| DR4 Haplotypes
| ||||
| Serotypes | DRA | DRB1 | DRB4 | |
|---|---|---|---|---|
| DR4-DR53 | *0101 | *0401 | *0103 | |
| *0101 | *0402 | *01 | ||
| *0101 | *0403 | *01 | ||
| *0101 | *0404 | *01 | ||
| *0101 | *0405 | *01 | ||
| Serotypes | DQA1 | DQB1 | DRB1 | |
| DR4-DQ7 (7.3, 3) | *0303 | *0301 | *0401 | |
| DR4-DQ7 (8.1, 3) | *0301 | *0302 | *0401 | |
| *0301 | *0302 | *0402 | ||
| *0301 | *0302 | *0404 | ||
| DR4-DQ4 (4.3) | *0303 | *0401 | *0405 | |
| Serotypes | HLA-A | HLA-C | HLA-B | DRB1 |
| A2-Cw10-B62(15)-DR4 | *0201 | *0304 | *1501 | *0401 |
| A2-Cw10-B60(40)-DR4 | *0201 | *0304 | *4001 | *0402 |
| A2-Cw5-B44(16)-DR4 | *0201 | *0501 | *4402 | *0401 |
References
HLA DR Serotypes |
|---|
| DR - DR1 - DR2 - DR3 - DR4 - DR5 - DR6 - DR7 - DR8 - DR9 - DR10 - DR11 - DR12 - DR13 - DR14 - DR15 - DR16 - DR17 - DR18 - DR51 - DR52 - DR53 |
Acknowledgement and Attribution Regarding Sources of Content
Some of the initial content on this page may be incorporated in part from copyleft sources in the public domain including wikis such as Wikipedia and AskDrWiki. Drug information for patients came from the The National Library of Medicine. Infectious disease information may have come from the Centers for Disease Control (CDC). Differential Diagnoses are drawn from clinicians as well as an amalgamation of 3 sources: 1.The Disease Database; 2. Kahan, Scott, Smith, Ellen G. In A Page: Signs and Symptoms. Malden, Massachusetts: Blackwell Publishing, 2004:3; 3. Sailer, Christian, Wasner, Susanne. Differential Diagnosis Pocket. Hermosa Beach, CA: Borm Bruckmeir Publishing LLC, 2002:7 .
