Kunitz-type protease inhibitor 2

This inhibitor contains two inhibitory domains.

Inhibitor of HGFAC (PubMed:9346890). Also inhibits plasmin, and plasma and tissue kallikrein (PubMed:9115294). Inhibits serine protease activity of TMPRSS13 (PubMed:20977675, PubMed:28710277). Inhibits serine protease activity of ST14/matriptase and PRSS8/prostasin in vitro (PubMed:28710277, PubMed:30445423).

Diarrhea 3, secretory sodium, congenital, with or without other congenital anomalies

DIAR3

A disease characterized by life-threatening secretory diarrhea, severe metabolic acidosis and hyponatremia. Hyponatremia is secondary to extraordinarily high fecal sodium loss, with low or normal excretion of urinary sodium, in the absence of infectious, autoimmune and endocrine causes.

None

The disease is caused by variants affecting the gene represented in this entry.

Expressed in placenta, kidney, pancreas, prostate, testis, thymus, and trachea.

• Cell membrane
• Single-pass type I membrane protein
• Cytoplasm

HAI2, KOP, SPINT2, Kunitz-type protease inhibitor 2, Hepatocyte growth factor activator inhibitor type 2, Placental bikunin, HAI-2

• entrezGene:10653
• omim:605124
• swissprot:O43291

Proteins

Oncology

28228Da