SPINT2

SPINT2 is inhibitor of HGF activator. Also SPINT2 inhibits plasmin, plasma and tissue kallikrein, and factor XIa.

SPINT2 Related Areas

Enzyme>>Protease & Regulator>>Serine Protease & Regulator>>SPINT2/HAI-2

SPINT2 Alternative Names

SPINT2, HAI-2, HAI2, DIAR3, FLJ45571, Kop, PB [Homo sapiens]

Spint2, HAI-2, AL024025, C76321 [Mus musculus]

Summaries for SPINT2

Entrez Gene summary for SPINT2:

SPINT2 gene encodes a transmembrane protein with two extracellular Kunitz domains that inhibits a variety of serine proteases. The protein inhibits HGF activator which prevents the formation of active hepatocyte growth factor. SPINT2 gene is a putative tumor suppressor, and mutations in this gene result in congenital sodium diarrhea. Multiple transcript variants encoding different isoforms have been found for SPINT2 gene. [provided by RefSeq, Oct 2009]

Wikipedia summary for SPINT2:

Kunitz-type protease inhibitor 2 is an enzyme that in humans is encoded by the SPINT2 gene.

Human SPINT2 Protein General Information

 

• Protein names: Kunitz-type protease inhibitor 2
  Short name=SPINT2
• Sequence length: 252 AA.
• Domain: This inhibitor contains two inhibitory domains.
• Sequence similarities: Contains 2 BPTI/Kunitz inhibitor domains.
• Subcellular location: Membrane; Single-pass type I membrane protein
• Tissue specificity: Expressed in placenta, kidney, pancreas, prostate, testis, thymus, and trachea.
• Involvement in disease: Defects in SPINT2 are the cause of diarrhea type 3 (DIAR3) ; 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.

General information above from UniProt

Function for SPINT2 Protein

UniProtKB:

SPINT2 is inhibitor of HGF activator. Also SPINT2 inhibits plasmin, plasma and tissue kallikrein, and factor XIa.

Genatlas:

• SPINT2 is kunitz-type serine-protease inhibitor, potent inhibitor of a number of serine proteases involved in blood coagulation and fibrinolysis such as pancreatic trypsin, plasmin, kallikrein-11 and hepatocyte growth factor activator
• SPINT2 plays an important regulatory role in pericellular activation of hepatocyte growth factor/scatter factor (HGF/SF), critically involved in the development and regeneration of various tissues
• SPINT2 may play a role in the process of spermatogenesis
• SPINT2 may regulate ST14 activity

Homology for human SPINT2

• homolog to SPINT1

Phenotype Information for SPINT2

• Gene/Locus: SPINT2, HAI2, DIAR3
• Phenotype: Diarrhea 3, secretory sodium, congenital, syndromic

Phenotype Information for SPINT2 from OMIM (Online Mendelian Inheritance in Man)