Catecholamines may contribute to the cause of sudden infant death syndrome (SIDS). TH01, a tetrameric short tandem repeat marker in the tyrosine hydroxylase gene, regulates gene expression and catecholamine production.We investigated TH01 in 172 German Caucasian SIDS cases and 390 sex- and age-matched control subjects.The *9.3 alleles were more frequent in patients with SIDS than in control subjects (40.12% vs 31.15%; P = .006). For homozygotes the odds ratio was 1.83 (95% confidence interval: 1.09-3.05), for carriers 1.58 (1.09-2.28). Moreover, *9.3 alleles were significantly more frequent during the winter (47.73% vs 35.38% in the warmer seasons), and the frequency of *9.3 alleles varied significantly with the age at death (weeks 7 to 12: 49.04% vs 29.63% within the first 6 weeks). Other risk factors (sleeping position, gestation, smoking) had no significant impact on the frequency of *9.3.Our results indicate a relationship between SIDS and TH01 genotype, presumably caused by an impairment of breathing regulation or arousal. We propose that noradrenalinergic neuronal activity contributes to the cause of a major subset of SIDS victims. Moreover, the results further stress that SIDS is a highly heterogenic group.