Inhibitor of HGF activator. Also inhibits plasmin, plasma and tissue kallikrein, and factor XIa.
Expressed in placenta, kidney, pancreas, prostate, testis, thymus, and trachea.
Defects in SPINT2 are the cause of diarrhea type 3 (DIAR3) [MIM:270420]; also known as congenital sodium diarrhea (CSD). DIAR3 is a rare, inherited diarrhea of infancy. A diagnosis of DIAR3 is made on the findings of a life-threatening secretory diarrhea, severe metabolic acidosis, and hyponatremia secondary to extraordinarily high fecal losses of sodium, with low or normal excretion of urinary sodium, in the absence of infectious, autoimmune, and endocrine causes.