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Illustration of an HLA-DR (Human MHC class II) antigen receptor with bound antigen
major histocompatibility complex, class II, DR3
Haplotypes groups DRA*01:DRB1*0301 DRA*01:DRB1*0302 DRA*01:DRB1*0303 DRA*01:DRB1*0304
Structure (See HLA-DR)
alpha *0101
Symbol(s) HLA-DRA
beta 1 *0301 *0301 *0302 *0303 *0304
Symbol(s) HLA-DRB1
Shared data
Locus chr.6 6p21.31

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. Insulin dependent diabetes mellitus (IDDM) is strongly associated with HLA-DR3 or HLA-DR4.

Further information: B8-DR3 in autoimmune diseases


DR17 and DR3 recognition of some DRB1*03 alleles[1]
DRB1* DR3 DR17 DR18 Sample
allele  %  %  % size (N)
0301 33 64 0 9698
0302 24 3 66 317
0303 40 60 5
0304 60 20 5
0307 >50 1

Some DR3 also react with HLA-DR17 and/or HLA-DR18. The DRB1*0304 primarily reacts with DR3. The serotypes of *0305, *0306, *0308 to *0331 are unknown.

Disease associations[edit]

By serotype[edit]

HLA-DR3 is associated with early-age onset myasthenia gravis, Hashimoto's thyroiditis (along with DR5), primary sclerosing cholangitis,[2] and opportunistic infections in AIDS,[3] but lowered risk for cancers.[4]

By allele[edit]

DRB1*0301 (see HLA-DR17)

DRB1*0302 (See HLA-DR18)

DRB1*0304 is associated it graves disease[5]

By haplotypes[edit]

DR3 and/or DQ2 is associated with Moreen's ulceration,[6] "bout onset" multiple sclerosis,[7] Grave's disease[8] and systemic lupus erythematosus[9]

DR3-DQ2 linkage is associated with coeliac disease, dermatitis herpetiformis, Diabetes mellitus type 1. DR3-DR2 is the serological marker for HLA-DQ2.5cis isoform. Although it cannot identify the alpha ".5" chain of HLA DQ, DQA1*0501 gene is almost always found within the DR3-DQ2 haplotype Eurasia (however is older studies DQA1*0505 is commonly confused with DQA1*0501)

Genetic linkage[edit]

DR3 is genetically linked to HLA-DR52, DRB3*0202 allele, and HLA-DQ2 (DQ2.5).


  1. ^ derived from IMGT/HLA
  2. ^ Wiencke K, Karlsen TH, Boberg KM et al. (2007). "Primary sclerosing cholangitis is associated with extended HLA-DR3 and HLA-DR6 haplotypes". Tissue Antigens 69 (2): 161–9. doi:10.1111/j.1399-0039.2006.00738.x. PMID 17257319. 
  3. ^ Pollack MS, Gold J, Metroka CE, Safai B, Dupont B (1984). "HLA-A,B,C and DR antigen frequencies in acquired immunodeficiency syndrome (AIDS) patients with opportunistic infections". Hum. Immunol. 11 (2): 99–103. doi:10.1016/0198-8859(84)90048-X. PMID 6333416. 
  4. ^ Mann DL, Murray C, O'Donnell M, Blattner WA, Goedert JJ (1990). "HLA antigen frequencies in HIV-1-related Kaposi's sarcoma". J. Acquir. Immune Defic. Syndr. 3 Suppl 1: S51–5. PMID 2395088. 
  5. ^ Heward, Jm; Allahabadia, A; Sheppard, Mc; Barnett, Ah; Franklyn, Ja; Gough, Sc (Jul 1999). "Association of the large multifunctional proteasome (LMP2) gene with Graves' disease is a result of linkage disequilibrium with the HLA haplotype DRB1*0304-DQB1*02-DQA1*0501". Clinical endocrinology 51 (1): 115–8. doi:10.1046/j.1365-2265.1999.00755.x. ISSN 0300-0664. PMID 10468973. 
  6. ^ Taylor C, Smith S, Morgan C, Stephenson S, Key T, Srinivasan M, Cunningham E, Watson P (2000). "HLA and Mooren's ulceration". Br J Ophthalmol 84 (1): 72–5. doi:10.1136/bjo.84.1.72. PMC 1723219. PMID 10611103. 
  7. ^ Weinshenker B, Santrach P, Bissonet A, McDonnell S, Schaid D, Moore S, Rodriguez M (1998). "Major histocompatibility complex class II alleles and the course and outcome of MS: a population-based study". Neurology 51 (3): 742–7. doi:10.1212/wnl.51.3.742. PMID 9748020. 
  8. ^ Ratanachaiyavong S, Lloyd L, Darke C, McGregor A (1993). "MHC-extended haplotypes in families of patients with Graves' disease". Hum Immunol 36 (2): 99–111. doi:10.1016/0198-8859(93)90112-E. PMID 8096501. 
  9. ^ Tjernström F, Hellmer G, Nived O, Truedsson L, Sturfelt G (1999). "Synergetic effect between interleukin-1 receptor antagonist allele (IL1RN*2) and MHC class II (DR17,DQ2) in determining susceptibility to systemic lupus erythematosus". Lupus 8 (2): 103–8. doi:10.1191/096120399678847560. PMID 10192503. 
  10. ^ Pera C, Delfino L, Longo A, Pistillo MP, Ferrara GB (March 2000). "Novel associations among HLA-DQA1 and -DQB1 alleles, revealed by high-resolution sequence-based typing (SBT)". Tissue Antigens 55 (3): 275–9. doi:10.1034/j.1399-0039.2000.550313.x. PMID 10777105.