Additional examples include case 09DG01414 with a homozygous truncating variant in TMPRSS15 (NM_002772.2:c.2808_2809insATCA p.(Ser937Ilefs*4)) who died in infancy, an unusual presentation of enterokinase deficiency. Here, TMPRSS15 is linked to congenital enteropathy due to enteropeptidase deficiency.