<i>HLA-DPA1*02:01~B1*01:01</i> is a risk haplotype for primary sclerosing cholangitis mediating activation of NKp44+ NK cells.

The HLA-DP haplotype analysis revealed that HLA-DPA1*02:01~DPB1*01:01 was highly significantly associated with an increased risk of PSC (OR 1.99, p=6.69×10 −50 ), whereas HLA-DPA1*01:03~DPB1*03:01 was protective (OR 0.78, p=3.18×10 −9 ) ( table 1 , online supplemental table 8 ).

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