KLKB1 - kallikrein B1 Gene

Also Known as PKK; PPK; KLK3; PKKD

Species: Homo sapiens

Gene Type: protein coding

Gene ID: 3818

About KLKB1

Cytogenetic location: 4q35.2 Genomic coordinates (GRCh38): 4:186,210,853-186,258,471 (from NCBI)

This gene has 6 transcripts (splice variants), 209 orthologues, 16 paralogues and is associated with 2 phenotypes. Biased expression in liver (RPKM 44.5) and kidney (RPKM 1.2).

Summary

This gene encodes a glycoprotein that participates in the surface-dependent activation of blood coagulation, fibrinolysis, kinin generation and inflammation. The encoded preproprotein present in plasma as a non-covalent complex with high molecular weight kininogen undergoes proteolytic processing mediated by activated coagulation factor XII to generate a disulfide-linked, heterodimeric serine protease comprised of heavy and light chains. Certain mutations in this gene cause prekallikrein deficiency. Alternative splicing results in multiple transcript variants encoding different isoforms. [provided by RefSeq, Jan 2016]

KLKB1 Products (3)

mRNA	Protein	Name
NM_000892.5	NP_000883.2	plasma kallikrein isoform 1 preproprotein
NM_001318394.2	NP_001305323.1	plasma kallikrein isoform 2
NM_001318396.2	NP_001305325.1	plasma kallikrein isoform 3 precursor

Gene Ontology

Molecular Function
Biological Process
Cellular Component

Molecular Function GO Annotation	Evidence	References	Source
enables protein binding	IPI IPI: Inferred from physical interaction	2844223	GOA

Biological Process GO Annotation	Evidence	References	Source
involved in plasminogen activation	IDA IDA: Inferred from direct assay	89876	GOA
involved in positive regulation of fibrinolysis	IDA IDA: Inferred from direct assay	89876	GOA

Cellular Component GO Annotation	Evidence	References	Source
located in extracellular space	IDA IDA: Inferred from direct assay	89876	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

KLKB1 Protein Structure

PAN_1

Trypsin

0
100
200
300
400
500
600
638 a.a.

Protein Preferred Names	Protein Names
plasma kallikrein	kallikrein B, plasma (Fletcher factor) 1

KLKB1 Protein-protein interaction Information

Type	Protein Name	Protein ID	Interactor	Interactor Species	Interactor ID	Detection Method	References
Intra	KLKB1	P03952	TMEM185A	Homo sapiens	Q8NFB2	Y2H Pooling	32814053
Intra	KLKB1	P03952	TMEM185A	Homo sapiens	Q8NFB2	Validated Y2H	32814053
Intra	KLKB1	P03952	TMEM185A	Homo sapiens	Q8NFB2	Y2H Array	32814053
Intra	KLKB1	P03952	NPHP1	Homo sapiens	C9J082	Validated Y2H	32814053
Intra	KLKB1	P03952	NPHP1	Homo sapiens	C9J082	Y2H Array	32814053
Intra	KLKB1	P03952	NPHP1	Homo sapiens	C9J082	Y2H Pooling	32814053
Intra	KLKB1	P03952	TGOLN2	Homo sapiens	O43493-5	Validated Y2H	32814053
Intra	KLKB1	P03952	TGOLN2	Homo sapiens	O43493-5	Y2H Pooling	32814053
Intra	KLKB1	P03952	TGOLN2	Homo sapiens	O43493-5	Y2H Array	32814053
Intra	KLKB1	P03952	SLFN12	Homo sapiens	Q8IYM2	Validated Y2H	32814053
Intra	KLKB1	P03952	SLFN12	Homo sapiens	Q8IYM2	Y2H Pooling	32814053
Intra	KLKB1	P03952	SLFN12	Homo sapiens	Q8IYM2	Y2H Array	32814053
Intra	KLKB1	P03952	PRMT5	Homo sapiens	O14744	Validated Y2H	32814053
Intra	KLKB1	P03952	PRMT5	Homo sapiens	O14744	Y2H Array	32814053
Intra	KLKB1	P03952	PRMT5	Homo sapiens	O14744	Y2H Pooling	32814053
Intra	KLKB1	P03952	NME4	Homo sapiens	O00746	Y2H Array	32814053
Intra	KLKB1	P03952	NME4	Homo sapiens	O00746	Validated Y2H	32814053
Intra	KLKB1	P03952	NME4	Homo sapiens	O00746	Y2H Pooling	32814053

Cross: Cross-species interaction Intra: Intraspecies interaction

Recombinant KLKB1 Proteins

Cat. No.	Product Name	Accession	Purity
HY-P70249	Plasma kallikrein/KLKB1 Protein, Human (HEK293, His, solution)	P03952 (G20-A638)	≥ 95%, as determined by reducing SDS-PAGE.

KLKB1 Antibodies

Cat. No.	Product Name	Application	Reactivity
HY-P83618	Plasma Kallikrein Antibody (YA3363)	WB, IHC-P, IP	Human, Rat

Related Diseases

Diseases	Alias
Prekallikrein Deficiency	Fletcher Factor Deficiency PKK DEFICIENCY Fletcher Factor Deficiency Congenital Prekallikrein Deficiency PKKD Fletcher Trait
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
Angioedema	Angioneurotic Oedema Quincke'S Edema Angioneurotic Edema Giant Urticaria
High Molecular Weight Kininogen Deficiency	HMWK DEFICIENCY Fitzgerald Trait Congenital High-Molecular-Weight Kininogen Deficiency Flaujeac Factor Deficiency Kininogen Deficiency, High Molecular Weight Kininogen Deficiency Hmwk High-Molecular-Weight Kininogen Deficiency, Congenital
Renal Hypertension	Hypertension Renal Hypertension, Renal
C1 Inhibitor Deficiency	Quincke Edema Angioedemas, Hereditary Angioedema
Pyruvate Kinase Deficiency Of Red Cells	Pyruvate Kinase Deficiency Pk Deficiency Pyruvate Kinase Deficiency Of Erythrocyte Hemolytic Anemia Due To Red Cell Pyruvate Kinase Deficiency Pyruvate Kinase Deficiency Of Erythrocytes Pkd PKRD Hereditary Non-Spherocytic Hemolytic Anemia Due To Pyruvate Kinase Deficiency Hnsha Pyruvate Kinase-Deficient Hemolytic Anemia Red Cell Pyruvate Kinase Deficiency Deficiency Of Pyruvate Kinase Anemia, Hemolytic, Congenital Nonspherocytic
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]
Congenital Nonspherocytic Hemolytic Anemia	Hereditary Non-Spherocytic Hemolytic Anemia Hereditary Nonspherocytic Hemolytic Anemia Anemia, Hemolytic, Congenital Nonspherocytic Congenital Nonspherocytic Hemolytic Anaemia Hereditary Nonspherocytic Hemolytic Anaemia Hnsha
Liver Cirrhosis	Cirrhosis Cirrhosis Of Liver CIRRH Cryptogenic Cirrhosis Cirrhosis, Cryptogenic Cirrhosis Nos
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
Factor Xii Deficiency	Hageman Factor Deficiency Haf Deficiency Factor Xii Deficiency Disease F12 Deficiency Deficiency, Hageman Coagulation Factor 12 Deficiency Factor 12 Deficiency Congenital Factor Xii Deficiency Congenital Hageman Factor Deficiency FA12D Factor Xii Deficiency, Factor Xii
Hypertension, Essential	Essential Hypertension Hypertension High Blood Pressure Hypertension, Essential, Susceptibility To Hypertensive Disease Primary Hypertension EHT Hypertension, Salt-Sensitive Essential, Susceptibility To Hyperpiesia Idiopathic Hypertension Hypertensive Disorder Hypertension, Essential, Susceptibility To, 3 Hypertension, Essential 3 Hypertension, Essential, Salt-Sensitive Hypertension, Essential, Susceptibility To, 6 Hypertension, Essential 6 Hypertension, Salt-Sensitive Essential Hypertension, Susceptibility To Hypertension, Essential, Susceptibility To, 4 Hypertension, Essential 4 Hypertension, Essential, Susceptibility To, 2 Hypertension, Essential 2 Hypertension, Essential, Susceptibility To, 1 Hypertension, Essential 1 Hypertension, Essential, Susceptibility To, 5 Hypertension, Essential 5 Htn Vascular Hypertensive Disorder Systemic Primary Arterial Hypertension Hbp - [High Blood Pressure] Systemic Arterial Hypertensive Disorder Elevated Blood Pressure Arterial Hypertension Nos Hypertension Nos Benign Hypertension Systemic Arterial Hypertension Systemic Hypertension Artery Htn Benign Htn Vascular Htn Vascular Hypertension Cholesterol Hypertension Cholesterol Htn Idiopathic Htn Malignant Hypertension Malignant Htn Raised Blood Pressure Cardiovascular Hypertension Primary Htn - [Hypertension] High Arterial Tension High Blood Pressure Disorder Ht - [Hypertension] Htn - [Hypertension] Hypertensive Vascular Disease Hypertensive Vascular Degeneration
Microvascular Complications Of Diabetes 5	Diabetic Retinopathy Microvascular Complications Of Diabetes, Susceptibility To, 5 MVCD5 Retinopathy, Diabetic Diabetic Nephropathy Retinopathy, Diabetic, Susceptibility To Retinal Abnormality - Diabetes-Related
Diabetic Macular Edema
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 (1)

Cat. No.	Product Name	Effect	Purity	Rare Diseases
HY-148949A	Kallikrein 5-IN-2 TFA	Inhibitor	99.53%	Unkown

Orthologs Information

Species	Symbol	Source	ID
Macaca mulatta	KLKB1	VGNC	VGNC:74195
Rattus norvegicus	KLKB1	RGD	RGD:67382
Bos taurus	KLKB1	VGNC	VGNC:30683
Felis catus	KLKB1	VGNC	VGNC:67969
Canis familiaris	KLKB1	VGNC	VGNC:42484
Mus musculus	KLKB1	MGD	MGI:102849
Others	KLKB1	NCBI