Serping1 MGI Mouse Gene Detail - MGI:894696 - serine (or cysteine) peptidase inhibitor, clade G, member 1

Symbol

Serping1
Name

serine (or cysteine) peptidase inhibitor, clade G, member 1
Synonyms

C1INH, C1 inhibitor, C1nh

Feature Type

protein coding gene
IDs

MGI:894696
NCBI Gene: 12258
Alliance

genome feature page
Transcription Start Sites

12 TSS

Sequence Map

Chr2:84595704-84605748 bp, - strand
From NCBI annotation of GRCm39
View this region in JBrowse
Genome Browsers

Ensembl | UCSC | NCBI

Genetic Map

Chromosome 2, 49.45 cM
Mapping Data

4 experiments

SNPs within 2kb

286 from dbSNP Build 142
Strain Annotations

19
PCR

1

For selected strains:

Strain	Gene Model ID	Feature Type	Coordinates
C57BL/6J	MGI_C57BL6J_894696	protein coding gene	Chr2:84595704-84605788 (-)
129S1/SvImJ	ENSMUSG00200051098	protein coding gene	Chr2:81612158-81622211 (-)
A/J	ENSMUSG00195048750	protein coding gene	Chr2:81725081-81735188 (-)
AKR/J	ENSMUSG00220049911	protein coding gene	Chr2:81603758-81613858 (-)
BALB/cJ	ENSMUSG00180054584	protein coding gene	Chr2:81652918-81663018 (-)
C3H/HeJ	ENSMUSG00175051466	protein coding gene	Chr2:81792505-81802606 (-)
C57BL/6NJ	ENSMUSG00215052224	protein coding gene	Chr2:81697253-81707311 (-)
CAROLI/EiJ	MGP_CAROLIEiJ_G0023729	protein coding gene	Chr2:78976910-78986912 (-)
CAST/EiJ	ENSTCUG00005050371	protein coding gene	Chr2:81143155-81153246 (-)
CBA/J	ENSMUSG00210031998	protein coding gene	Chr2:81820411-81830517 (-)
DBA/2J	ENSMUSG00185056887	protein coding gene	Chr2:81649160-81659258 (-)
FVB/NJ	ENSMUSG00205048572	protein coding gene	Chr2:81030740-81040803 (-)
JF1/MsJ	ENSUMUG00000004271	protein coding gene	Chr2:81574277-81584501 (-)
LP/J	ENSMUSG00230038896	protein coding gene	Chr2:83400258-83410354 (-)
NOD/ShiLtJ	ENSMUSG00190040846	protein coding gene	Chr2:81689683-81699725 (-)
NZO/HlLtJ	ENSMUSG00225001696	protein coding gene	Chr2:91284829-91294869 (-)
PWK/PhJ	ENSLUMG00010042678	protein coding gene	Chr2:81590159-81600244 (-)
SPRET/EiJ	ENSMSPG00010052014	protein coding gene	Chr2:83492052-83502152 (-)
WSB/EiJ	ENSIUOG00005052931	protein coding gene	Chr2:81629443-81639599 (-)

Human Ortholog

SERPING1, serpin family G member 1

Vertebrate Orthologs

3

Vertebrate Orthology Source

Alliance of Genome Resources

Human Ortholog

SERPING1, serpin family G member 1
Synonyms

C1IN, C1INH, C1NH, HAE1, HAE2
Links

HGNC:1228

NCBI Gene ID: 710

UniProt: P05155
Chr Location

11q12.1; chr11:57597387-57619246 (+) GRCh38

MGI Vertebrate Homology

Serping1 stringent orthology
1 human;1 mouse;1 rat;1 zebrafish
HCOP

vertebrate homology predictions: SERPING1
Gene Tree

Serping1

Diseases

8 with human SERPING1 associations

				Human Disease	Mouse Models
				angioedema IDs angioedema DOID:1558 ICD10CM:T78.3 MESH:D000799 NCI:C112175 UMLS_CUI:C0002994
				cerebral infarction IDs cerebral infarction DOID:3526 ICD10CM:I63 MESH:D002544 NCI:C50486 OMIM:601367 UMLS_CUI:C0007785
				COVID-19 IDs COVID-19 DOID:0080600 ICD10CM:U07.1 MESH:D000086382 NCI:C171133 UMLS_CUI:C5203670
				hereditary angioedema IDs hereditary angioedema DOID:14735 MESH:D054179 NCI:C84758 OMIM:PS106100 ORDO:91378 UMLS_CUI:C0019243
				hereditary angioedema type I IDs hereditary angioedema type I DOID:0080939 OMIM:106100
				macular degeneration IDs macular degeneration DOID:4448 MESH:D008268 NCI:C123330 ORDO:279 UMLS_CUI:C0024437
				pancreatitis IDs pancreatitis DOID:4989 EFO:0000278 ICD10CM:K85.9 MESH:D010195 NCI:C3306 OMIM:167800 UMLS_CUI:C0030305
				toxic shock syndrome IDs toxic shock syndrome DOID:14115 ICD10CM:A48.3 ICD9CM:040.82 MESH:D012772 NCI:C35498 UMLS_CUI:C0600327

Click on a disease name to see all genes associated with that disease.

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Phenotype Summary

8 phenotypes from 2 alleles in 2 genetic backgrounds
1 phenotype from multigenic genotypes
23 phenotype references

Phenotype Overview

Blue squares indicate phenotypes directly attributed to mutations/alleles of this gene.

adipose tissue	behavior/neurological	cardiovascular system	cellular	craniofacial	digestive/alimentary system	embryo	endocrine/exocrine glands	growth/size/body	hearing/vestibular/ear	hematopoietic system	homeostasis/metabolism	integument	immune system	limbs/digits/tail	liver/biliary system	mortality/aging	muscle	nervous system	pigmentation	renal/urinary system	reproductive system	respiratory system	skeleton	taste/olfaction	neoplasm	vision/eye

Click cells to view annotations.

All Mutations and Alleles

44
Chemically induced (other)

1
Endonuclease-mediated

31
Gene trapped

6
Targeted

6
Genomic Mutations

3 involving Serping1
Incidental Mutations

Mutagenetix , APF
Find Mice (IMSR)

30 strains or lines available
Comparison Matrix

Gene Expression + Phenotype

Mutant mice exhibit an increased vascular permeability compared to controls.

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All GO Annotations

20
GO References

12

Molecular Function

carbohydrate derivative binding	cytoskeletal protein binding	DNA binding	enzyme regulator	hydrolase	ligase	lipid binding	oxidoreductase	RNA binding	signaling receptor activity	signaling receptor binding	transcription	transferase	transporter

Biological Process

carbohydrate derivative metabolism	cell differentiation	cell population proliferation	cellular component organization	DNA-templated transcription	establishment of localization	homeostatic process	immune system process	lipid metabolic process	programmed cell death	protein metabolic process	response to stimulus	signaling	system development

Cellular Component

cell projection	cytoplasmic vesicle	cytoskeleton	cytosol	endoplasmic reticulum	endosome	extracellular region	Golgi apparatus	mitochondrion	membraneless organelle	nucleus	organelle envelope	organelle lumen	plasma membrane	protein-containing complex	synapse	vacuole

Click cells to view annotations.

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Expression Overview

GXD's primary emphasis is on endogenous gene expression during development. Click on grid cells to view annotations.

Blue cells = expressed in wild-type.
Gray triangles = other expression annotations only
(e.g. absence of expression or data from mutants).

early conceptus	embryo ectoderm	embryo endoderm	embryo mesoderm	embryo mesenchyme	extraembryonic component	alimentary system	auditory system	branchial arches	cardiovascular system	connective tissue	endocrine system	exocrine system	hemolymphoid system	integumental system	limbs	liver and biliary system	musculoskeletal system	nervous system	olfactory system	reproductive system	respiratory system	urinary system	visual system

Click cells to view annotations.

Assay Results

620
Tissues

21
cDNA Data

254
Literature Summary

9

Images

Tissue x Stage Matrix

Comparison Matrix

Gene Expression + Phenotype

Other Mouse Links

Allen Institute

GEO

Expression Atlas
Other Vertebrate Links

ZFIN serping1

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All Sequences

45
RefSeq

6
UniProt

2
CCDS

CCDS16193.1

Ensembl

ENSMUSG00000023224 (Evidence)
NCBI Gene

12258

			Length	Strain/Species	Flank
genomic	12258	NCBI Gene Model \| MGI Sequence Detail	10045	C57BL/6J	± kb
transcript	NM_001420027	RefSeq \| MGI Sequence Detail	1800	C57BL/6
polypeptide	P97290	UniProt \| EBI \| MGI Sequence Detail	504	Not Applicable

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UniProt

2 Sequences
Protein Ontology

PR:000014707 plasma protease C1 inhibitor

(term hierarchy)
InterPro Domains

IPR023795 Serpin, conserved site

IPR023796 Serpin domain

IPR000215 Serpin family

IPR036186 Serpin superfamily

IPR042178 Serpin superfamily, domain 1

IPR042185 Serpin superfamily, domain 2
GlyGen

P97290 5 sites, 2 N-linked glycans (2 sites)

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All nucleic 258

Genomic 2

cDNA 254

Primer pair 2

Microarray probesets 3

Summaries

All 70

Developmental Gene Expression 9

Gene Ontology 12

Phenotypes 23
Earliest

J:40775 Russell JA, et al., The sequence of a cDNA encoding functional murine C1-inhibitor protein. Biochim Biophys Acta. 1997 May 30;1352(2):156-60
Latest

J:345621 Adams DJ, et al., Genetic determinants of micronucleus formation in vivo. Nature. 2024 Mar;627(8002):130-136

TSS for Serping1:

(View these features in JBrowse)

Transcription Start Site	Location	Distance from Gene 5'-end
Tssr25053	Chr2:84605764-84605782 (-)	-25 bp
Tssr25052	Chr2:84605732-84605755 (-)	4 bp
Tssr25051	Chr2:84605207-84605220 (-)	534 bp
Tssr25050	Chr2:84605153-84605204 (-)	569 bp
Tssr25049	Chr2:84605140-84605151 (-)	602 bp
Tssr25048	Chr2:84603981-84603993 (-)	1,761 bp
Tssr25047	Chr2:84603858-84603897 (-)	1,870 bp
Tssr25046	Chr2:84603772-84603808 (-)	1,958 bp
Tssr25045	Chr2:84603716-84603749 (-)	2,015 bp
Tssr25044	Chr2:84603682-84603715 (-)	2,049 bp
Tssr25043	Chr2:84603601-84603611 (-)	2,142 bp
Tssr25042	Chr2:84603585-84603596 (-)	2,157 bp

Contributing Projects: Mouse Genome Database (MGD), Gene Expression Database (GXD), Mouse Models of Human Cancer database (MMHCdb) (formerly Mouse Tumor Biology (MTB)), Gene Ontology (GO)
Citing These Resources Funding Information Warranty Disclaimer, Privacy Notice, Licensing, & Copyright Send questions and comments to User Support.	last database update 09/08/2026 MGI 6.24