Literature summary for 3.4.21.76 extracted from

Hajjar, E.; Broemstrup, T.; Kantari, C.; Witko-Sarsat, V.; Reuter, N.
Structures of human proteinase 3 and neutrophil elastase - so similar yet so different (2010), FEBS J., 277, 2238-2254.

Search for more literature for 3.4.21.76

Activating Compound

Activating Compound	Comment	Organism	Structure
additional information	bilayer-bound PR3 has a reduced catalytic efficiency	Homo sapiens

Cloned(Commentary)

Cloned (Comment)	Organism
mast cell lines transfected with PR3	Homo sapiens

Crystallization (Commentary)

Crystallization (Comment)	Organism
adopts a fold consisting of two beta-barrels made each of six anti-parallel beta-sheets. Crystallizes as a tetramer, which can be regarded as a dimer of dimers: two monomers in a dimer are oriented so that their active sites face each other, preventing the binding of large substrates. The hole in the middle of the tetramer is lined with hydrophobic residues. Contains four disulfide bridges between cysteine pairs 42-58, 136-201, 168-182 and 191-220. No X-ray structure of PR3 with a substrate in its active site available	Homo sapiens

Crystallization (Comment)

Organism

adopts a fold consisting of two beta-barrels made each of six anti-parallel beta-sheets. Crystallizes as a tetramer, which can be regarded as a dimer of dimers: two monomers in a dimer are oriented so that their active sites face each other, preventing the binding of large substrates. The hole in the middle of the tetramer is lined with hydrophobic residues. Contains four disulfide bridges between cysteine pairs 42-58, 136-201, 168-182 and 191-220. No X-ray structure of PR3 with a substrate in its active site available

Homo sapiens

Inhibitors

Inhibitors	Comment	Organism
alpha1-antitrypsin	inhibition of bilayer-bound PR3 is more important than that observed for the soluble form of the enzyme	Homo sapiens
alpha1-proteinase	does not inhibit when PR3 is bound to the outer cell surface of neutrophils	Homo sapiens
elafin	does not inhibit when PR3 is bound to the outer cell surface of neutrophils	Homo sapiens

Localization

Localization	Comment	Organism	GeneOntology No.	Textmining
azurophil granule	major pool of intracellular PR3 is within the azurophilic granules	Homo sapiens	42582	-
membrane	behaves as a peripheral membrane protein	Homo sapiens	16020	-
membrane	behaves as a peripheral membrane protein. The mouse form is globally more electronegative than the human form. As a consequence of differences in sequence and structural properties, mouse PR3 is likely to be able to bind to the plasma membrane using the same region as human PR3, although less strongly and specifically	Mus musculus	16020	-
soluble	the extragranular pool of PR3, which is externalized during apoptosis, may be functionally very important in the pathophysiology of vasculitis	Homo sapiens	-	-

Organism

Organism	UniProt	Comment	Textmining
Homo sapiens	-	-	-
Mus musculus	-	-	-

Source Tissue

Source Tissue	Comment	Organism	Textmining
polymorphonuclear neutrophil	-	Mus musculus	-
polymorphonuclear neutrophil	-	Homo sapiens	-

Substrates and Products (Substrate)

Substrates	Comment Substrates	Organism	Products	Comment (Products)	Rev.
additional information	the peptide sequence VADVKDR is highly specific for PR3	Homo sapiens	?	-	?
additional information	unlike human PR3, mouse PR3 is unlikely to bind substrates with acidic groups (Asp, Glu) on the S side. Efficient substrates of human PR3 may be poor substrates of mouse PR3	Mus musculus	?	-	?
procaspase 3 + H2O	PR3 can cleave membrane-associated procaspase 3 into a 22 kDa fragment	Homo sapiens	?	-	?

Subunits

Subunits	Comment	Organism
tetramer	crystallography	Homo sapiens

Synonyms

Synonyms	Comment	Organism
myeloblastin	-	Mus musculus
myeloblastin	-	Homo sapiens
p29b	-	Mus musculus
p29b	-	Homo sapiens
PR3	-	Mus musculus
PR3	-	Homo sapiens
proteinase 3	-	Mus musculus
proteinase 3	-	Homo sapiens

Expression

Organism	Comment	Expression
Homo sapiens	expression of membrane PR3 and CD177 on the same subset of neutrophils	up

General Information

General Information	Comment	Organism
physiological function	involvement in the regulation of intracellular functions such as proliferation or apoptosis. Its membrane expression is a risk factor in chronic inflammatory diseases. PR3 is the preferred target antigen in Wegener's granulomatosis. Colocalization of PR3 with the integrin CD11b/CD18 (b2 integrin), the Fcgamma receptor FcgRIIIb and the p22phox subunit of cytochrome b558 in the membrane. Both PR3 and phospholipid scramblase 1 are associated within the same protein complex	Homo sapiens