Serping1 serine (or cysteine) peptidase inhibitor, clade G, member 1 [Mus musculus (house mouse)] - Gene

Summary

Official Symbol: Serping1provided by MGI
Official Full Name: serine (or cysteine) peptidase inhibitor, clade G, member 1provided by MGI
Primary source: MGI:MGI:894696
See related: Ensembl:ENSMUSG00000023224 AllianceGenome:MGI:894696
Gene type: protein coding
RefSeq status: VALIDATED
Organism: Mus musculus
Lineage: Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia; Eutheria; Euarchontoglires; Glires; Rodentia; Myomorpha; Muroidea; Muridae; Murinae; Mus; Mus
Also known as: C1nh; C1Inh; C1 Inh; C1INH.
Summary: Enables complement binding activity and serine-type endopeptidase inhibitor activity. Predicted to be involved in negative regulation of complement activation, lectin pathway and negative regulation of endopeptidase activity. Predicted to act upstream of or within several processes, including complement activation, classical pathway; fibrinolysis; and negative regulation of peptidase activity. Located in extracellular space. Is expressed in several structures, including genitourinary system; neural ectoderm; notochord; pancreas mesenchyme; and visceral pericardium. Human ortholog(s) of this gene implicated in several diseases, including COVID-19; angioedema (multiple); cerebral infarction; pancreatitis; and toxic shock syndrome. Orthologous to human SERPING1 (serpin family G member 1). [provided by Alliance of Genome Resources, Apr 2022]
Expression: Broad expression in liver adult (RPKM 378.2), lung adult (RPKM 269.8) and 18 other tissues See more
Orthologs: human all
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Genomic context

Location:: 2 D; 2 49.45 cM

Exon count:: 8

Annotation release	Status	Assembly	Chr	Location
RS_2024_02	current	GRCm39 (GCF_000001635.27)	2	NC_000068.8 (84595704..84605748, complement)
108.20200622	previous assembly	GRCm38.p6 (GCF_000001635.26)	2	NC_000068.7 (84765360..84775444, complement)

Chromosome 2 - NC_000068.8 Genomic Context describing neighboring genes

Genomic regions, transcripts, and products

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Genomic Sequence:

Go to nucleotide: Graphics FASTA GenBank

Expression

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See details

Project title: Mouse ENCODE transcriptome data
Description: RNA profiling data sets generated by the Mouse ENCODE project.
BioProject: PRJNA66167
Publication: PMID 25409824
Analysis date: n/a

Bibliography

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GeneRIFs: Gene References Into Functions

What's a GeneRIF?

Serping1 associated with alpha-synuclein increase in colonic smooth muscles of MPTP-induced Parkinson's disease mice.
Title: Serping1 associated with α-synuclein increase in colonic smooth muscles of MPTP-induced Parkinson's disease mice.
Tubule-mitophagic secretion of SerpinG1 reprograms macrophages to instruct anti-septic acute kidney injury efficacy of high-dose ascorbate mediated by NRF2 transactivation.
Title: Tubule-mitophagic secretion of SerpinG1 reprograms macrophages to instruct anti-septic acute kidney injury efficacy of high-dose ascorbate mediated by NRF2 transactivation.
A novel murine in vivo model for acute hereditary angioedema attacks.
Title: A novel murine in vivo model for acute hereditary angioedema attacks.
Association of increase in Serping1 level with dopaminergic cell reduction in an MPTP-induced Parkinson's disease mouse model.
Title: Association of increase in Serping1 level with dopaminergic cell reduction in an MPTP-induced Parkinson's disease mouse model.
C1INH has an important role as a gatekeeper to the brain via the neurovascular system.
Title: Knockdown of circulating C1 inhibitor induces neurovascular impairment, glial cell activation, neuroinflammation, and behavioral deficits.
C1INH prevented myocardial ischemia reperfusion injury in mice.
Title: The effect of C1 inhibitor on myocardial ischemia and reperfusion injury.
In addition to the protective activities mediated via inhibition of the complement system, these studies indicate that C1INH also plays a direct role in suppression of leukocyte transmigration into reperfused tissue.
Title: The effect of C1 inhibitor on intestinal ischemia and reperfusion injury.
C1-inhibitor limits neointimal plaque formation and inflammation. This may involve blockade of complement activation, inhibition of leukocyte recruitment, and reduced triglyceride levels
Title: C1-esterase inhibitor protects against neointima formation after arterial injury in atherosclerosis-prone mice.
In vivo, C1 inhibitor (C1INH) reduces the number of viable bacteria in the blood and peritoneal fluid and accelerates killing of bacteria by blood neutrophils and peritoneal macrophages.
Title: C1 inhibitor-mediated protection from sepsis.
Increased vascular permeability in C1 inhibitor-deficient mice mediated by the bradykinin type 2 receptor.
Title: Increased vascular permeability in C1 inhibitor-deficient mice mediated by the bradykinin type 2 receptor.

Submit: New GeneRIF Correction See all GeneRIFs (11)

Variation

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Alleles

Alleles of this type are documented at Mouse Genome Informatics (MGI)

Gene trapped (2) 1 citation
Targeted (1) 1 citation
Endonuclease-mediated (28)

General gene information

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Markers

Serping1 (e-PCR), detects polymorphism
- UniSTS:141199

Gene Ontology Provided by MGI

Function	Evidence Code	Pubs
enables complement binding	IPI Inferred from Physical Interaction more info	PubMed
enables peptidase inhibitor activity	IEA Inferred from Electronic Annotation more info
enables serine-type endopeptidase inhibitor activity	IBA Inferred from Biological aspect of Ancestor more info
enables serine-type endopeptidase inhibitor activity	IDA Inferred from Direct Assay more info	PubMed
enables serine-type endopeptidase inhibitor activity	ISO Inferred from Sequence Orthology more info

Process	Evidence Code	Pubs
acts_upstream_of_or_within blood coagulation	IEA Inferred from Electronic Annotation more info
acts_upstream_of_or_within complement activation, classical pathway	IEA Inferred from Electronic Annotation more info
acts_upstream_of_or_within fibrinolysis	IEA Inferred from Electronic Annotation more info
acts_upstream_of_or_within hemostasis	IEA Inferred from Electronic Annotation more info
acts_upstream_of_or_within immune system process	IEA Inferred from Electronic Annotation more info
acts_upstream_of_or_within innate immune response	IEA Inferred from Electronic Annotation more info
involved_in negative regulation of complement activation, lectin pathway	ISO Inferred from Sequence Orthology more info
involved_in negative regulation of endopeptidase activity	IBA Inferred from Biological aspect of Ancestor more info
acts_upstream_of_or_within negative regulation of peptidase activity	IEA Inferred from Electronic Annotation more info

Component	Evidence Code	Pubs
located_in collagen-containing extracellular matrix	HDA more info	PubMed
located_in extracellular region	IEA Inferred from Electronic Annotation more info
located_in extracellular space	HDA more info	PubMed
is_active_in extracellular space	IBA Inferred from Biological aspect of Ancestor more info
located_in extracellular space	IDA Inferred from Direct Assay more info	PubMed
located_in extracellular space	ISO Inferred from Sequence Orthology more info

General protein information

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Preferred Names: plasma protease C1 inhibitor

Names: C1 esterase inhibitor; C1 inhibitor; C1-inhibiting factor; complement component 1 inhibitor; serine (or cysteine) proteinase inhibitor, clade G (C1 inhibitor), member 1; serine (or cysteine) proteinase inhibitor, clade G, member 1; serpin G1

NCBI Reference Sequences (RefSeq)

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RefSeqs maintained independently of Annotated Genomes

These reference sequences exist independently of genome builds. Explain

These reference sequences are curated independently of the genome annotation cycle, so their versions may not match the RefSeq versions in the current genome build. Identify version mismatches by comparing the version of the RefSeq in this section to the one reported in Genomic regions, transcripts, and products above.

mRNA and Protein(s)

NM_001420027.1 → NP_001406956.1 plasma protease C1 inhibitor precursor

Status: VALIDATED

Source sequence(s)

AL928914

UniProtKB/Swiss-Prot

A2ATR7, O88330, P97290, Q99M43, Q9QX09
NM_001420028.1 → NP_001406957.1 plasma protease C1 inhibitor precursor

Status: VALIDATED

Source sequence(s)

AL928914

UniProtKB/Swiss-Prot

A2ATR7, O88330, P97290, Q99M43, Q9QX09
NM_009776.4 → NP_033906.2 plasma protease C1 inhibitor precursor

See identical proteins and their annotated locations for NP_033906.2

Status: VALIDATED

Source sequence(s)

AL928914

Consensus CDS

CCDS16193.1

UniProtKB/Swiss-Prot

A2ATR7, O88330, P97290, Q99M43, Q9QX09

Related

ENSMUSP00000023994.4, ENSMUST00000023994.10

Conserved Domains (1) summary

cd02050
Location:141 → 500

serpinG1_C1-INH; serpin family G member 1, plasma proteinase C1 inhibitor