Neuroserpin

SERPINI1
Available structures
PDB	Ortholog search: PDBe RCSB
List of PDB id codes
	3F02, 3F5N, 3FGQ
Identifiers
Aliases	SERPINI1, PI12, neuroserpin, serpin family I member 1, HNS-S2, HNS-S1
External IDs	OMIM: 602445; MGI: 1194506; HomoloGene: 21045; GeneCards: SERPINI1; OMA:SERPINI1 - orthologs
Gene location (Human)
Chr.	Chromosome 3 (human)
End	167,825,569 bp
Gene location (Mouse)
Chr.	Chromosome 3 (mouse)
End	75,550,802 bp
RNA expression pattern
	Top expressed in
	frontal pole; ; orbitofrontal cortex; ; Brodmann area 10; ; middle temporal gyrus; ; superior frontal gyrus; ; Brodmann area 23; ; postcentral gyrus; ; dorsolateral prefrontal cortex; ; endothelial cell; ; Brodmann area 46;
	Top expressed in
	Region I of hippocampus proper; ; mammillary body; ; prefrontal cortex; ; primary motor cortex; ; subiculum; ; perirhinal cortex; ; piriform cortex; ; CA3 field; ; ventral tegmental area; ; dorsal tegmental nucleus;
	More reference expression data
	More reference expression data
Gene ontology
Molecular function	peptidase inhibitor activity; serine-type endopeptidase inhibitor activity;
Cellular component	extracellular region; extracellular exosome; secretory granule lumen; soma; cytoplasmic vesicle lumen; extracellular space; cytoplasmic vesicle; perikaryon;
Biological process	central nervous system development; negative regulation of peptidase activity; regulation of cell adhesion; peripheral nervous system development; negative regulation of endopeptidase activity; positive regulation of neuron projection development;
	Sources:Amigo / QuickGO
Orthologs
	5274
	20713
	ENSG00000163536
	ENSMUSG00000027834
	Q99574
	O35684
	NM_005025; NM_001122752
	NM_009250; NM_001357514
	NP_001116224; NP_005016
	NP_033276; NP_001344443
	Wikidata
View/Edit Human	View/Edit Mouse

Neuroserpin is a protein that in humans is encoded by the SERPINI1 gene.^[5]

It is associated with Familial encephalopathy with neuroserpin inclusion bodies.

Serine protease inhibitors of the serpin superfamily are involved in many cellular processes. Neuroserpin was first identified as a protein secreted from the axons of dorsal root ganglion neurons (Stoeckli et al., 1989). It is expressed in the late stages of neurogenesis during the process of synapse formation.[supplied by OMIM]^[5]

Interactions

SERPINI1 has been shown to interact with Tissue plasminogen activator.^[6]

References

^ ^a ^b ^c GRCh38: Ensembl release 89: ENSG00000163536 – Ensembl, May 2017
^ ^a ^b ^c GRCm38: Ensembl release 89: ENSMUSG00000027834 – 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.
^ ^a ^b "Entrez Gene: SERPINI1 serpin peptidase inhibitor, clade I (neuroserpin), member 1".
^ Parmar PK, Coates Leigh C, Pearson John F, Hill Rena M, Birch Nigel P (September 2002). "Neuroserpin regulates neurite outgrowth in nerve growth factor-treated PC12 cells". J. Neurochem. 82 (6): 1406–15. doi:10.1046/j.1471-4159.2002.01100.x. ISSN 0022-3042. PMID 12354288. S2CID 23571680.

Yepes M, Lawrence DA (2004). "Neuroserpin: a selective inhibitor of tissue-type plasminogen activator in the central nervous system". Thromb. Haemost. 91 (3): 457–64. doi:10.1160/TH03-12-0766. PMID 14983220. S2CID 39118265.
Schrimpf SP, Bleiker AJ, Brecevic L, et al. (1997). "Human neuroserpin (PI12): cDNA cloning and chromosomal localization to 3q26". Genomics. 40 (1): 55–62. doi:10.1006/geno.1996.4514. PMID 9070919.
Hastings GA, Coleman TA, Haudenschild CC, et al. (1998). "Neuroserpin, a brain-associated inhibitor of tissue plasminogen activator is localized primarily in neurons. Implications for the regulation of motor learning and neuronal survival". J. Biol. Chem. 272 (52): 33062–7. doi:10.1074/jbc.272.52.33062. PMID 9407089.
Davis RL, Shrimpton AE, Holohan PD, et al. (1999). "Familial dementia caused by polymerization of mutant neuroserpin". Nature. 401 (6751): 376–9. doi:10.1038/43894. PMID 10517635. S2CID 4413322.
Chang WS, Chang NT, Lin SC, et al. (2000). "Tissue-specific cancer-related serpin gene cluster at human chromosome band 3q26". Genes Chromosomes Cancer. 29 (3): 240–55. doi:10.1002/1098-2264(2000)9999:9999<::AID-GCC1029>3.0.CO;2-A. PMID 10992299. S2CID 23441111.
Belorgey D, Crowther DC, Mahadeva R, Lomas DA (2002). "Mutant Neuroserpin (S49P) that causes familial encephalopathy with neuroserpin inclusion bodies is a poor proteinase inhibitor and readily forms polymers in vitro". J. Biol. Chem. 277 (19): 17367–73. doi:10.1074/jbc.M200680200. PMID 11880376.
Davis RL, Shrimpton AE, Carrell RW, et al. (2002). "Association between conformational mutations in neuroserpin and onset and severity of dementia". Lancet. 359 (9325): 2242–7. doi:10.1016/S0140-6736(02)09293-0. PMID 12103288. S2CID 10722760.
Barker-Carlson K, Lawrence DA, Schwartz BS (2003). "Acyl-enzyme complexes between tissue-type plasminogen activator and neuroserpin are short-lived in vitro". J. Biol. Chem. 277 (49): 46852–7. doi:10.1074/jbc.M207740200. PMID 12228252.
Parmar PK, Coates LC, Pearson JF, et al. (2002). "Neuroserpin regulates neurite outgrowth in nerve growth factor-treated PC12 cells". J. Neurochem. 82 (6): 1406–15. doi:10.1046/j.1471-4159.2002.01100.x. PMID 12354288. S2CID 23571680.
Strausberg RL, Feingold EA, Grouse LH, et al. (2003). "Generation and initial analysis of more than 15,000 full-length human and mouse cDNA sequences". Proc. Natl. Acad. Sci. U.S.A. 99 (26): 16899–903. Bibcode:2002PNAS...9916899M. doi:10.1073/pnas.242603899. PMC 139241. PMID 12477932.
Miranda E, Römisch K, Lomas DA (2004). "Mutants of neuroserpin that cause dementia accumulate as polymers within the endoplasmic reticulum". J. Biol. Chem. 279 (27): 28283–91. doi:10.1074/jbc.M313166200. PMID 15090543.
Teesalu T, Kulla A, Simisker A, et al. (2005). "Tissue plasminogen activator and neuroserpin are widely expressed in the human central nervous system". Thromb. Haemost. 92 (2): 358–68. doi:10.1160/TH02-12-0310. PMID 15269833.
Belorgey D, Sharp LK, Crowther DC, et al. (2004). "Neuroserpin Portland (Ser52Arg) is trapped as an inactive intermediate that rapidly forms polymers: implications for the epilepsy seen in the dementia FENIB". Eur. J. Biochem. 271 (16): 3360–7. doi:10.1111/j.1432-1033.2004.04270.x. PMID 15291813.
Gerhard DS, Wagner L, Feingold EA, et al. (2004). "The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)". Genome Res. 14 (10B): 2121–7. doi:10.1101/gr.2596504. PMC 528928. PMID 15489334.
Onda M, Belorgey D, Sharp LK, Lomas DA (2005). "Latent S49P neuroserpin forms polymers in the dementia familial encephalopathy with neuroserpin inclusion bodies". J. Biol. Chem. 280 (14): 13735–41. doi:10.1074/jbc.M413282200. PMID 15664988.
Rual JF, Venkatesan K, Hao T, et al. (2005). "Towards a proteome-scale map of the human protein-protein interaction network". Nature. 437 (7062): 1173–8. Bibcode:2005Natur.437.1173R. doi:10.1038/nature04209. PMID 16189514. S2CID 4427026.
Kinghorn KJ, Crowther DC, Sharp LK, et al. (2006). "Neuroserpin binds Abeta and is a neuroprotective component of amyloid plaques in Alzheimer disease". J. Biol. Chem. 281 (39): 29268–77. doi:10.1074/jbc.M600690200. PMID 16849336.
Chen PY, Chang WS, Chou RH, et al. (2007). "Two non-homologous brain diseases-related genes, SERPINI1 and PDCD10, are tightly linked by an asymmetric bidirectional promoter in an evolutionarily conserved manner". BMC Mol. Biol. 8: 2. doi:10.1186/1471-2199-8-2. PMC 1796892. PMID 17212813.
Gourfinkel-An I, Duyckaerts C, Camuzat A, et al. (2007). "Clinical and neuropathologic study of a French family with a mutation in the neuroserpin gene". Neurology. 69 (1): 79–83. doi:10.1212/01.wnl.0000265052.99144.b5. PMID 17606885. S2CID 39704442.

External links

The MEROPS online database for peptidases and their inhibitors: I04.025

This article on a gene on human chromosome 3 is a stub. You can help Wikipedia by expanding it.

[refGRCh38Ensembl-1] GRCh38: Ensembl release 89: ENSG00000163536 – Ensembl, May 2017

[refGRCm38Ensembl-2] GRCm38: Ensembl release 89: ENSMUSG00000027834 – 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.

[entrez-5] "Entrez Gene: SERPINI1 serpin peptidase inhibitor, clade I (neuroserpin), member 1".

[pmid12354288-6] Parmar PK, Coates Leigh C, Pearson John F, Hill Rena M, Birch Nigel P (September 2002). "Neuroserpin regulates neurite outgrowth in nerve growth factor-treated PC12 cells". J. Neurochem. 82 (6): 1406–15. doi:10.1046/j.1471-4159.2002.01100.x. ISSN 0022-3042. PMID 12354288. S2CID 23571680.

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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

Neuroserpin

Interactions

References

Further reading

External links

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