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Kawasaki disease (KD) is an acute febrile disorder characterized by systemic vasculitis primarily occurring in coronary arteries. Matrix metalloproteinases (MMPs) have been considered to play pathophysiologic roles in the development of coronary artery lesions (CALs); therefore, an evaluation of the genetic contributions of the
MMP
genes to the development of CALs in KD patients would be beneficial for the prediction of CAL formation. We focused on the known functional single nucleotide polymorphisms (SNPs) in the
MMP
genes (
MMP-2
-735C>T,
MMP-3
-1612 5A/6A,
MMP-9
-1562C>T,
MMP-12
-82A>G, and
MMP-13
-77A>G) and performed the association study between these SNPs and CAL formation in KD. The study population consisted of 44 KD patients with CALs and 92 without CALs and 175 healthy controls. As a result, allele and genotype frequencies of
MMP-13
-77A>G showed significant differences between KD patients with CALs and without CALs (
p
= 0.00989 and
p
= 0.00551, respectively). The estimated frequencies of the G-C haplotype in the
MMP-13
gene promoter were significantly lower in KD patients with CALs than in those without CALs. There was no association between other
MMP
genes and CAL formation. In conclusion, the genetic evaluation by association study demonstrated that the
MMP-13
gene, at least in part, contributed to the development of CALs in KD.