SERPING1 - serpin family G member 1 Gene

Also Known as C1IN; C1NH; HAE1; HAE2; C1INH

Species: Homo sapiens

Gene Type: protein coding

Gene ID: 710

About SERPING1

Cytogenetic location: 11q12.1 Genomic coordinates (GRCh38): 11:57,597,685-57,614,848 (from NCBI)

This gene has 22 transcripts (splice variants), 191 orthologues, 36 paralogues and is associated with 4 phenotypes. Broad expression in liver (RPKM 710.5), gall bladder (RPKM 322.8) and 17 other tissues.

Summary

This gene encodes a highly glycosylated plasma protein involved in the regulation of the complement cascade. Its encoded protein, C1 inhibitor, inhibits activated C1r and C1s of the first complement component and thus regulates complement activation. It is synthesized in the liver, and its deficiency is associated with hereditary angioneurotic oedema (HANE). Alternative splicing results in multiple transcript variants encoding the same isoform. [provided by RefSeq, May 2020]

SERPING1 Products (2)

mRNA	Protein	Name
NM_000062.3	NP_000053.2	plasma protease C1 inhibitor precursor
NM_001032295.2	NP_001027466.1	plasma protease C1 inhibitor precursor

Gene Ontology

Molecular Function
Biological Process

Molecular Function GO Annotation	Evidence	References	Source
enables protein binding	IPI IPI: Inferred from physical interaction	10570951	GOA
enables serine-type endopeptidase inhibitor activity	IDA IDA: Inferred from direct assay	11527969	GOA

Biological Process GO Annotation	Evidence	References	Source
involved in negative regulation of complement activation, lectin pathway	IDA IDA: Inferred from direct assay	10946292	GOA

EXP: Inferred from Experiment IDA: Inferred from direct assay IPI: Inferred from physical interaction IMP: Inferred from mutant phenotype IGI: Inferred from genetic interaction IEP: Inferred from expression pattern

SERPING1 Protein Structure

Serpin

0
100
200
300
400
500 a.a.

Protein Preferred Names	Protein Names
plasma protease C1 inhibitor	C1 esterase inhibitor

SERPING1 Protein-protein interaction Information

Type	Protein Name	Protein ID	Interactor	Interactor Species	Interactor ID	Detection Method	References
Cross	SERPING1	P05155	P0C6X7-PRO_0000037310	Human SARS coronavirus	P0C6X7-PRO_0000037310	Lumier	22046132
Cross	SERPING1	P05155	P0C6X7-PRO_0000037310	Human SARS coronavirus	P0C6X7-PRO_0000037310	Y2H Pooling	22046132
Cross	SERPING1	P05155	P0C6X7-PRO_0000037320	Human SARS coronavirus	P0C6X7-PRO_0000037320	Lumier	22046132
Cross	SERPING1	P05155	P0C6X7-PRO_0000037320	Human SARS coronavirus	P0C6X7-PRO_0000037320	Y2H Pooling	22046132
Cross	SERPING1	P05155	MSP-3	Plasmodium falciparum	A0A7D5SLD3	Anti Bait CoIP	28484054
Intra	SERPING1	P05155	C1S	Homo sapiens	P09871	Comigration in Gel	28742139
Intra	SERPING1	P05155	C1R	Homo sapiens	P00736	Comigration in Gel	28742139
Intra	SERPING1	P05155	PLAT	Homo sapiens	P00750	Anti Tag CoIP	33961781
Intra	SERPING1	P05155	CREB3	Homo sapiens	O43889-2	Validated Y2H	25910212
Intra	SERPING1	P05155	CREB3	Homo sapiens	O43889-2	Y2H Bait-Prey Pool	25910212
Intra	SERPING1	P05155	CREB3	Homo sapiens	O43889-2	Y2H Array	25910212
Intra	SERPING1	P05155	MASP2	Homo sapiens	O00187	Comigration in Gel	28742139
Cross	SERPING1	P05155	vag8	Bordetella pertussis	Q79GN7	Comigration in Gel	28742139

Cross: Cross-species interaction Intra: Intraspecies interaction

Recombinant SERPING1 Proteins

Cat. No.	Product Name	Accession	Purity
HY-P73526	Serpin G1 Protein, Human (HEK293, His)	P05155/NP_000053.2 (N23-A500)	≥ 95%, as determined by reducing SDS-PAGE.

SERPING1 Antibodies

Cat. No.	Product Name	Application	Reactivity
HY-P82235	SERPING1 Antibody (YA1980)	WB, IP	Human, Mouse, Rat

Related Diseases

Diseases	Alias
Angioedema, Hereditary, 1	Hereditary Angioedema Type I Hereditary Angioneurotic Edema Hane C1 Esterase Inhibitor Deficiency Angioedema, Hereditary, 1 And 2 HAE1 Angioneurotic Edema, Hereditary Angioedema, Hereditary, Type I Hereditary Angioedema Type 2 Hae 2 Hae-Ii Hereditary Angioneurotic Edema Type 2 Angioedema, Hereditary HAE Angioedema, Hereditary, Types I And Ii Hereditary Angioedema, Type Ii Angioedema, Hereditary, Type 1 Angioedemas, Hereditary Hereditary C1 Esterase Inhibitor Deficiency - Dysfunctional Factor Hereditary C1 Esterase Inhibitor Deficiency - Deficient Factor Hereditary Angioedema Types I And Ii
Hereditary Angioedema	Hereditary Angioneurotic Edema Hereditary Angioedema Type 1 Hane Angioedema, Hereditary Hae Angioedemas, Hereditary Deficiency Of C1 Esterase Inhibitor C1 Esterase Inhibitor Deficiency C1 Inhibitor Deficiency Familial Angioneurotic Edema Hereditary Bradykinine-Induced Angioedema Hereditary Non Histamine-Induced Angioedema Hae 1 Hae-I Hereditary Angioneurotic Edema Type 1 Hereditary C1 Esterase Inhibitor Deficiency - Deficient Factor Hereditary Angioedema Types I And Ii Hereditary Angioneurotic Oedema Familial Angioedema Hae - [Hereditary Angioneurotic Oedema] Bannister Disease, Hereditary Quincke Disease Or Oedema Hereditary Quincke Oedema
Complement Component 4, Partial Deficiency Of	Angioedemas, Hereditary
C1 Inhibitor Deficiency	Quincke Edema Angioedemas, Hereditary Angioedema
Hereditary Angioedema With C1inh Deficiency	Hae With C1 Inhibitor Deficiency Hae With C1inh Deficiency Hereditary Angioneurotic Edema With C1 Inhibitor Deficiency Hereditary Angioneurotic Edema With C1inh Deficiency
Angioedema	Angioneurotic Oedema Quincke'S Edema Angioneurotic Edema Giant Urticaria
Acquired Angioedema	Acquired C1 Inhibitor Deficiency Angioedema, Acquired Aae Acquired Angioneurotic Edema Acquired Bradykinine-Induced Angioedema Acquired Non Histamine-Induced Angioedema Acquired Angioneurotic Oedema Aae - [Acquired Angioneurotic Oedema]
Laryngeal Small Cell Carcinoma	Small Cell Carcinoma Of Larynx
Angioedema, Hereditary, 3	Angioedema, Hereditary, Type Iii Hereditary Angioedema Type Iii Hereditary Angioedema Type 3 HAE3 Estrogen-Related Hae Estrogen-Sensitive Hae Angioneurotic Edema, Hereditary, With Normal C1 Inhibitor Concentration And Function Hae With Normal C1 Inhibitor Concentration And Function Hereditary Angioedema With Normal C1 Inhibitor Activity F12-Related Hereditary Angioedema With Normal C1inh F12-Related Hae With Normal C1 Inhibitor Hae 3 Hae-Iii Hereditary Angioneurotic Edema Type 3 Inherited Estrogen-Associated Angioedema Inherited Estrogen-Associated Angioneurotic Edema Inherited Estrogen-Dependent Angioedema Inherited Estrogen-Dependent Angioneurotic Edema Angioneurotic Edema Hereditary With Normal C1 Inhibitor Concentration And Function Hereditary Angioedema With Normal C1 Esterase Inhibitor Activity
Physical Urticaria	Symptomatic Dermographism
Cholinergic Urticaria
Covid-19	2019 Novel Coronavirus 2019-Ncov Infection Covid19 Sars-Cov-2 Infection Wuhan Coronavirus Infection Wuhan Seafood Market Pneumonia Virus Infection
Louse-Borne Relapsing Fever	Relapsing Fever, Louse-Borne Relapsing Fever Due To Borrelia Recurrentis
Melkersson-Rosenthal Syndrome	Melkersson Syndrome Mros Mrs Cheilitis Granulomatosa Of Mescher-Melkersson-Rosenthal Melkersson'S Syndrome Cheilitis Granulomatosa Granulomatous Cheilitis
Dermatographia	Dermatographic Urticaria Dermographism Symptomatic Dermographism Urticaria Factitia Factitious Urticaria Dermatographism
Urticaria	Nettle Rash Hives Wheal Urticaria Nos
Complement Deficiency	Complement Deficiency Disease Hereditary Complement Deficiency Diseases
Fissured Tongue	Furrowed Tongue Plicated Tongue Tongue, Fissured Congenital Fissure Of Tongue Congenital Plicated Tongue Fissure Of Tongue Fissure Of Tongue, Congenital Geographic Tongue And Fissured Tongue Lingua Plicata Scrotal Tongue
Epiglottitis	Acute Epiglottitis Acute Epiglottitis And Supraglottitis Acute Epiglottiditis Epiglottiditis Epiglottitis Nos
Allergic Urticaria
Blepharochalasis	Dermatolysis Palpebrarum Adiposa Ptosis
Tick-Borne Relapsing Fever	Relapsing Fever, Tick-Borne Relapsing Fever Due To Any Borrelia Species Other Than Borrelia Recurrentis African Tick-Borne Fever
Chronic Inducible Urticaria
Capillary Leak Syndrome	Systemic Capillary Leak Syndrome Clarkson Disease Capillary Leak Syndrome With Monoclonal Gammopathy Scls Periodic Systemic Capillary Leak Syndrome Capillary Hyperpermeability Syndrome Idiopathic Capillary Leak Syndrome
Chronic Spontaneous Urticaria	Chronic Idiopathic Urticaria
Integumentary System Disease
Capillary Disease	Disease Of Capillaries
Cecal Disease	Cecal Diseases Disorder Of Cecum
Hemolytic Uremic Syndrome, Atypical 1	Atypical Hemolytic-Uremic Syndrome Hemolytic Uremic Syndrome, Atypical, Susceptibility To, 1 Atypical Hemolytic Uremic Syndrome Hemolytic Uremic Syndrome, Atypical, Susceptibility To Ahus AHUS1 Hemolytic-Uremic Syndrome Ahus 1 Ahus, Susceptibility To, 1 Hemolytic Uremic Syndrome, Atypical Non-Shiga-Like Toxin-Associated Hus Non-Stx-Hus Nonenteropathic Hus Atypical Hus Shiga Toxin-Associated Hemolytic Uremic Syndrome D+ Hus Ehec-Hus Hemolytic Uremic Syndrome Associated With Shiga Toxin-Producing Escherichia Coli Hemolytic Uremic Syndrome With Diarrhea Stec-Hus Shiga-Like Toxin-Associated Hus Stx-Hus Typical Hus Typical Hemolytic Uremic Syndrome Atypical Hemolytic Uremic Syndrome With Anti-Factor H Antibodies Atypical Hus With Anti-Factor H Antibodies Ahus With Anti-Factor H Antibodies Ahus With Neutralizing Autoantibodies Against Factor H Hemolytic Uremic Syndrome Atypical 1 Atypical Hemolytic Uremic Syndrome With H Factor Anomaly D Hus Hemolytic-Uremic Syndrome Without Diarrhea Hemolytic-Uremic Syndrome, Atypical, Type 1 Hemolytic Uremic Syndrome, Typical
Alpha-2-Plasmin Inhibitor Deficiency	Plasmin Inhibitor Deficiency Antiplasmin Deficiency Antiplasmin Defiency Anti-Plasmin Deficiency, Congenital Antiplasmin Deficiency, Congenital Congenital Alpha2-Antiplasmin Deficiency APLID Congenital Alpha2 Antiplasmin Deficiency
Macular Degeneration, Age-Related, 1	Macular Degeneration Age-Related Macular Degeneration Macular Degeneration, Age-Related Age Related Macular Degeneration Age Related Macular Degeneration 1 ARMD1 Senile Macular Degeneration Maculopathy, Age-Related, 1 Macular Degeneration, Age-Related, Reduced Risk Of Age Related Maculopathy 1 Age Related Maculopathies Age Related Maculopathy Senile Macular Retinal Degeneration Macular Degeneration Of Retina Age-Related Maculopathy Amd Armd Age-Related Maculopathy, Susceptibility To Maculopathy Age-Related Macular Degeneration, Age-Related, 1, Susceptibility To Maculopathy, Age-Related Macular Degeneration, Age-Related, Type 1 Macular Degeneration, Age-Related, 2
Myocardial Infarction	Heart Attack Myocardial Infarction, Susceptibility To Myocardial Infarction 1 Myocardial Infarction, Protection Against Myocardial Infarction, Decreased Susceptibility To Myocardial Infarction, Decreased Myocardial Infarct MCI1 Premature Myocardial Infarction Myocardial Infarction, Susceptibility To, Type 1
Skin Disease	Skin Diseases Genodermatosis Abnormality Of The Skin Skin Diseases, Genetic Skin And Subcutaneous Tissue Disease Dermatologic Disorders

Diseases	Alias
Waldenstroem'S Macroglobulinemia	Waldenstroem'S Macroglobulinemia Waldenstroem'S Macroglobulinemia Macroglobulinemia Of Waldenstrom Lymphoplasmacytic Lymphoma With Igm Gammopathy Lymphoplasmacytic Lymphoma Waldenstroem'S Macroglobulinemia Waldenstroem'S Macroglobulinemia Macroglobulinemia Of Waldenstrom Lymphoplasmacytic Lymphoma With Igm Gammopathy Lymphoplasmacytic Lymphoma

Related Agents

Pre-clinical Phase

Pre-clinical Phase

Bioactive Molecules (4)

Cat. No.	Product Name	Effect	Purity	Rare Diseases
HY-RS12694	SERP1 Human Pre-designed siRNA Set A	Pre-designed Set	/	Unkown
HY-RS12727	SERPING1 Human Pre-designed siRNA Set A	Pre-designed Set	/	Unkown
HY-RS18444	Serping1 Mouse Pre-designed siRNA Set A	Pre-designed Set	/	Unkown
HY-RS24926	Serping1 Rat Pre-designed siRNA Set A	Pre-designed Set	/	Unkown

Orthologs Information

Species	Symbol	Source	ID
Mus musculus	SERPING1	MGD	MGI:894696
Felis catus	SERPING1	VGNC	VGNC:65029
Bos taurus	SERPING1	VGNC	VGNC:54891
Rattus norvegicus	SERPING1	RGD	RGD:735225
Macaca mulatta	SERPING1	VGNC	VGNC:77223
Canis familiaris	SERPING1	VGNC	VGNC:52042
Others	SERPING1	NCBI