SERPINB1

SERPINB1
Available structures
PDB	Ortholog search: PDBe RCSB
List of PDB id codes
	4GA7
Identifiers
Aliases	SERPINB1, EI, ELANH2, HEL57, LEI, M/NEI, MNEI, PI-2, PI2, HEL-S-27, serpin family B member 1
External IDs	OMIM: 130135; MGI: 1913472; HomoloGene: 133768; GeneCards: SERPINB1; OMA:SERPINB1 - orthologs
Gene location (Human)
Chr.	Chromosome 6 (human)
End	2,841,959 bp
Gene location (Mouse)
Chr.	Chromosome 13 (mouse)
End	33,035,168 bp
RNA expression pattern
	Top expressed in
	mucosa of pharynx; ; oral cavity; ; bone marrow; ; monocyte; ; palpebral conjunctiva; ; body of pancreas; ; jejunal mucosa; ; trabecular bone; ; bone marrow cells; ; duodenum;
	Top expressed in
	granulocyte; ; left colon; ; jejunum; ; duodenum; ; mucous cell of stomach; ; esophagus; ; tibiofemoral joint; ; epithelium of stomach; ; ileum; ; Paneth cell;
	More reference expression data
	; ;
	More reference expression data
Gene ontology
Molecular function	peptidase inhibitor activity; serine-type endopeptidase inhibitor activity; protein binding;
Cellular component	cytoplasm; extracellular exosome; membrane; extracellular space; secretory granule lumen; extracellular matrix; extracellular region; collagen-containing extracellular matrix;
Biological process	negative regulation of peptidase activity; negative regulation of endopeptidase activity; neutrophil degranulation; type B pancreatic cell proliferation;
	Sources:Amigo / QuickGO
Orthologs
	1992
	66222
	ENSG00000021355
	ENSMUSG00000044734
	P30740
	Q9D154
	NM_030666
	NM_025429
	NP_109591
	NP_079705
	Wikidata
View/Edit Human	View/Edit Mouse

Leukocyte elastase inhibitor (LEI) also known as serpin B1 is a protein that in humans is encoded by the SERPINB1 gene. It is a member of the clade B serpins or ov-serpins (ovalbumin related serpins) founded by ovalbumin.^[5]^[6]^[7]

MNEI (monocyte/neutrophil elastase inhibitor) is the mouse orthologue of human SerpinB1.^[7]

Function

SerpinB1 is a cytoplasmic serine protease inhibitor of polymorphonuclear neutrophils. Among other serine proteases, it specifically inhibits neutrophil elastase, PR3 and cathepsin G, all found in neutrophil granules, by a suicide inhibition mechanism. SerpinB1 was found to reduce tissue damage caused by the mentioned proteases during inflammation and has a role in neutrophil homeostasis in mice. In various infection models (e.g. pneumonia) correlation of SerpinB1 absence and lack of microbial clearance have been shown. Different knockout strains serve as model to investigate the role of SerpinB1 in vivo.^[8]^[9]^[10]

References

^ ^a ^b ^c GRCh38: Ensembl release 89: ENSG00000021355 – Ensembl, May 2017
^ ^a ^b ^c GRCm38: Ensembl release 89: ENSMUSG00000044734 – Ensembl, May 2017
^ "Human PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
^ "Mouse PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
^ Remold-O'Donnell E, Chin J, Alberts M (Jul 1992). "Sequence and molecular characterization of human monocyte/neutrophil elastase inhibitor". Proc Natl Acad Sci U S A. 89 (12): 5635–9. Bibcode:1992PNAS...89.5635R. doi:10.1073/pnas.89.12.5635. PMC 49347. PMID 1376927.
^ "Entrez Gene: SERPINB1 serpin peptidase inhibitor, clade B (ovalbumin), member 1".
^ ^a ^b Benarafa C, Remold-O'Donnell E (August 2005). "The ovalbumin serpins revisited: perspective from the chicken genome of clade B serpin evolution in vertebrates". Proc. Natl. Acad. Sci. U.S.A. 102 (32): 11367–72. Bibcode:2005PNAS..10211367B. doi:10.1073/pnas.0502934102. PMC 1183561. PMID 16055559.
^ Gong D, Farley K, White M, Hartshorn KL, Benarafa C, Remold-O'Donnell E (August 2011). "Critical role of serpinB1 in regulating inflammatory responses in pulmonary influenza infection". J. Infect. Dis. 204 (4): 592–600. doi:10.1093/infdis/jir352. PMC 3144176. PMID 21791661.
^ Benarafa C, LeCuyer TE, Baumann M, Stolley JM, Cremona TP, Remold-O'Donnell E (July 2011). "SerpinB1 protects the mature neutrophil reserve in the bone marrow". J. Leukoc. Biol. 90 (1): 21–9. doi:10.1189/jlb.0810461. PMC 3114599. PMID 21248149.
^ Benarafa C, Priebe GP, Remold-O'Donnell E (August 2007). "The neutrophil serine protease inhibitor serpinb1 preserves lung defense functions in Pseudomonas aeruginosa infection". J. Exp. Med. 204 (8): 1901–9. doi:10.1084/jem.20070494. PMC 2118684. PMID 17664292.

External links

The MEROPS online database for peptidases and their inhibitors: I04.006
SERPINB1+protein,+human at the U.S. National Library of Medicine Medical Subject Headings (MeSH)

This article incorporates text from the United States National Library of Medicine, which is in the public domain.

This article on a gene on human chromosome 6 is a stub. You can help Wikipedia by expanding it.

[refGRCh38Ensembl-1] GRCh38: Ensembl release 89: ENSG00000021355 – Ensembl, May 2017

[refGRCm38Ensembl-2] GRCm38: Ensembl release 89: ENSMUSG00000044734 – Ensembl, May 2017

[3] "Human PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.

[4] "Mouse PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.

[pmid1376927-5] Remold-O'Donnell E, Chin J, Alberts M (Jul 1992). "Sequence and molecular characterization of human monocyte/neutrophil elastase inhibitor". Proc Natl Acad Sci U S A. 89 (12): 5635–9. Bibcode:1992PNAS...89.5635R. doi:10.1073/pnas.89.12.5635. PMC 49347. PMID 1376927.

[entrez-6] "Entrez Gene: SERPINB1 serpin peptidase inhibitor, clade B (ovalbumin), member 1".

[Benarafa_2005-7] Benarafa C, Remold-O'Donnell E (August 2005). "The ovalbumin serpins revisited: perspective from the chicken genome of clade B serpin evolution in vertebrates". Proc. Natl. Acad. Sci. U.S.A. 102 (32): 11367–72. Bibcode:2005PNAS..10211367B. doi:10.1073/pnas.0502934102. PMC 1183561. PMID 16055559.

[pmid21791661-8] Gong D, Farley K, White M, Hartshorn KL, Benarafa C, Remold-O'Donnell E (August 2011). "Critical role of serpinB1 in regulating inflammatory responses in pulmonary influenza infection". J. Infect. Dis. 204 (4): 592–600. doi:10.1093/infdis/jir352. PMC 3144176. PMID 21791661.

[pmid21248149-9] Benarafa C, LeCuyer TE, Baumann M, Stolley JM, Cremona TP, Remold-O'Donnell E (July 2011). "SerpinB1 protects the mature neutrophil reserve in the bone marrow". J. Leukoc. Biol. 90 (1): 21–9. doi:10.1189/jlb.0810461. PMC 3114599. PMID 21248149.

[pmid17664292-10] Benarafa C, Priebe GP, Remold-O'Donnell E (August 2007). "The neutrophil serine protease inhibitor serpinb1 preserves lung defense functions in Pseudomonas aeruginosa infection". J. Exp. Med. 204 (8): 1901–9. doi:10.1084/jem.20070494. PMC 2118684. PMID 17664292.

[1]

v t e Serpins
inhibitory	Alpha 1-antichymotrypsin Alpha 1-antitrypsin Alpha 2-antiplasmin Antithrombin C1-inhibitor Heparin cofactor II Protein C inhibitor Plasminogen activator inhibitor-1 Plasminogen activator inhibitor-2 Protein Z-related protease inhibitor SERPINA1 SERPINA2 SERPINA3 SERPINA4 SERPINA5 SERPINA9 SERPINA14 SERPINB1 SERPINB2 SERPINB3 SERPINB4 SERPINB5 SERPINB6 SERPINB7 SERPINB8 SERPINB9 SERPINB10 SERPINB13 SERPINC1 SERPIND1 SERPINE1 SERPINE2 SERPINE2 SERPINF1 SERPING1 SERPINH1 SERPINI1 SERPINI2
Cross class inhibitory	MENT SCCA-1 CrmA
noninhibitory	Heat shock protein 47 Maspin Ovalbumin Transcortin Thyroxine-binding globulin Angiotensinogen PEDF
see also disorders of globin and globulin proteins

SERPINB1

Function

See also

References

Further reading

External links