major histocompatibility complex, class II, DR53 | ||
Haplotypes groups | DRA*01:DRB3*01 DRA*01:DRB3*02 DRA*01:DRB3*03 | |
Structure (See HLA-DR) | ||
Identifiers | alpha *01:01 | |
Symbol(s) | HLA-DRA [ permanent dead link ] | |
EBI-HLA | DRA*01:01 | |
Identifiers | beta 3 *01*02*03 | |
Symbol(s) | HLA-DRB3 [ permanent dead link ] | |
EBI-HLA | DRB3*01 | |
EBI-HLA | DRB3*02 | |
EBI-HLA | DRB3*03 Archived 2011-05-15 at the Wayback Machine | |
Shared data | ||
Locus | chr.6 6p21.31 | |
HLA-DR52 is an HLA-DR serotype that recognizes gene products of HLA-DRB3 locus. Three allele groups can produce 35 isoforms.
DRB3, DRB4, and DRB5 are minor DR beta-encoding loci, and they have been recognized as having distinct evolution, [1] having diverged from DRB1 around 4 million years ago. [2]
The DRB3 locus is only apparent in a small subset of DR haplotypes, and most individuals lack DRB3.
DRB3* | DR52 | ? | Sample |
allele | % | % | size (N) |
01:01 | 95 | 62 | |
01:02 | 99 | 1 | |
02:01 | 99 | 2 | |
02:02 | 95 | 100 | |
02:03 | 95 | 1 | |
03:01 | 95 | 36 | |
DR52 serotype is positively associated with systemic sclerosis, [4] inflammatory myopathies, [5] inclusion body myositis, [6]
DRB3*01 is positively associated with sarcoidosis, [7] Grave's Disease, [8] [9] pulmonary sarcoidosis, [10]
DRB3*01:01:DRB1*03:01 is linked to Lofgren's syndrome [11]
DRB3*02:02 is also linked to Grave's disease, [9] serum IgG antibodies to Chlamydia pneumoniae with essential hypertension, [12] acute necrotizing encephalopathy [13]
DRB3*03:01 is weakly associated with anticardiolipin antibodies in SLE [14]
DRB3*03:01:DRB1*13:02 may be associated with Crohn's disease [15]
HLA-DRB3 is linked to these HLA-DR serotypes and DRB1 allele groups:
HLA-DR3 - DRB1*03
HLA-DR5 -
HLA-DR6 -
HLA-DR52 - Sjögren syndrome
The human leukocyte antigen (HLA) system or complex is a complex of genes on chromosome 6 in humans which encode cell-surface proteins responsible for regulation of the immune system. The HLA system is also known as the human version of the major histocompatibility complex (MHC) found in many animals.
HLA-DR is an MHC class II cell surface receptor encoded by the human leukocyte antigen complex on chromosome 6 region 6p21.31. The complex of HLA-DR and peptide, generally between 9 and 30 amino acids in length, constitutes a ligand for the T-cell receptor (TCR). HLA were originally defined as cell surface antigens that mediate graft-versus-host disease. Identification of these antigens has led to greater success and longevity in organ transplant.
HLA-DQ2 (DQ2) is a serotype group within HLA-DQ (DQ) serotyping system. The serotype is determined by the antibody recognition of β2 subset of DQ β-chains. The β-chain of DQ is encoded by HLA-DQB1 locus and DQ2 are encoded by the HLA-DQB1*02 allele group. This group currently contains two common alleles, DQB1*0201 and DQB1*0202. HLA-DQ2 and HLA-DQB1*02 are almost synonymous in meaning. DQ2 β-chains combine with α-chains, encoded by genetically linked HLA-DQA1 alleles, to form the cis-haplotype isoforms. These isoforms, nicknamed DQ2.2 and DQ2.5, are also encoded by the DQA1*0201 and DQA1*0501 genes, respectively.
HLA-DQ4 (DQ4) is a serotype subgroup within HLA-DQ(DQ) serotypes. The serotype is determined by the antibody recognition of β4 subset of DQ β-chains. The β-chain of DQ is encoded by HLA-DQB1 locus and DQ4 are encoded by the HLA-DQB1*04 allele group. This group currently contains 2 common alleles, DQB1*0401 and DQB1*0402. HLA-DQ4 and HLA-DQB1*04 are almost synonymous in meaning. DQ4 β-chains combine with α-chains, encoded by genetically linked HLA-DQA1 alleles, to form the cis-haplotype isoforms. These isoforms, nicknamed DQ4.3 and DQ4.4, are also encoded by the DQA1*0303 and DQA1*0401 genes, respectively.
HLA-DQ1 is a serotype that covers a broad range of HLA-DQ haplotypes. Historically it was identified as a DR-like alpha chain called DC1; later, it was among 3 types DQw1, DQw2 and DQw3. Of these three serotyping specificities only DQw1 recognized DQ alpha chain. The serotype is positive in individuals who bear the DQA1*01 alleles. The most frequently found within this group are: DQA1*0101, *0102, *0103, and *0104. In the illustration on the right, DQ1 serotyping antibodies recognizes the DQ α (magenta), where antibodies to DQA1* gene products bind variable regions close to the peptide binding pocket.
HLA-DR53 is an HLA-DR serotype that recognizes gene products of HLA-DRB4 locus. There are 13 alleles at this locus that encode 7 proteins.
HLA-DR51 is a HLA-DR serotype that recognizes the antigens encoded by the minor DR locus HLA-DRB5.
HLA-DR17 (DR17) is an HLA-DR serotype that recognizes the DRB1*0301 and *0304 gene products. DR17 is found at high frequency in Western Europe. DR17 is part of the broader antigen group HLA-DR3 and is very similar to the group HLA-DR18.
HLA-DR15 (DR15) is a HLA-DR serotype that recognizes the DRB1*1501 to *1505 and *1507 gene products. DR15 is found at high levels from Ireland to Central Asia. DR15 is part of the older HLA-DR2 serotype group which also contains the similar HLA-DR16 antigens.
HLA-DR13(DR13) is a HLA-DR serotype that recognizes the DRB1*1301 to *13082, *1310 and some other *13 gene products. DR13 serotype is a split antigen of the older HLA-DR6 serotype group which also contains the similar HLA-DR14 antigens.
HLA-DR14(DR14) is a HLA-DR serotype that recognizes the DRB1*1401 to *1408, *1410 to *1418, and other *14 gene products. DR14 serotype is a split antigen of the older HLA-DR6 serotype group which also contains the similar HLA-DR13 antigens.
HLA-DR11 (DR11) is a HLA-DR serotype that recognizes the DRB1*1101 to *1110. DR11 serotype is a split antigen of the older HLA-DR5 serotype group which also contains the similar HLA-DR12 antigens.
HLA-DR12(DR12) is a HLA-DR serotype that recognizes the DRB1*1201 to *1203, *1206. DR12 serotype is a split antigen of the older HLA-DR5 serotype group which also contains the similar HLA-DR11 antigens.
HLA-DR8 (DR8) is a HLA-DR serotype that recognizes the DRB1*0801 to *0807, and *0810 to *0812 gene products.
HLA-DR6 (DR6) is a broad-antigen serotype that is further split into HLA-DR13 and HLA-DR14 antigen serotypes.
HLA-DR5 (DR5) is a broad-antigen serotype that is further split into HLA-DR11 and HLA-DR12 antigen serotypes.
HLA-DR2 (DR2) of the HLA-DR serotype system, is a broad antigen serotype that is now preferentially covered by HLA-DR15 and HLA-DR16 serotype group. This serotype primarily recognizes gene products of the HLA-DRB1*15 and HLA-DRB1*16 allele groups.
HLA-DR3 is composed of the HLA-DR17 and HLA-DR18 split 'antigens' serotypes. DR3 is a component gene-allele of the AH8.1 haplotype in Northern and Western Europeans. Genes between B8 and DR3 on this haplotype are frequently associated with autoimmune disease. Type 1 diabetes mellitus is associated with HLA-DR3 or HLA-DR4. Nearly half the US population has either DR3 or DR4, yet only a small percentage of these individuals will develop type 1 diabetes.
HLA-DR4 (DR4) is an 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.
In autoimmune disease, anti-apolipoprotein H (AAHA) antibodies, also called anti-β2 glycoprotein I antibodies, comprise a subset of anti-cardiolipin antibodies and lupus anticoagulant. These antibodies are involved in sclerosis and are strongly associated with thrombotic forms of lupus. As a result AAHA are strongly implicated in autoimmune deep vein thrombosis.