KLKB1 - kallikrein B1 Gene

Homo sapiens

Also known as PKK; PPK; KLK3; PKKD

Gene ID: 3818 | Gene type: protein coding

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	Reference	Source
enables protein binding	IPI IPI: Inferred from physical interaction	2844223	GOA

Biological Process GO Annotation	Evidence	Reference	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	Reference	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 Cross Intra	Protein Name	Protein ID	Interactor	Interactor Species	Interactor ID	Detection Method	Reference
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%

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

Bioactive Molecules (1)

Cat. No.	Product Name	Effect	Purity	Rare Diseases
HY-148949A	Kallikrein 5-IN-2 TFA	Inhibitor	/	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
Macaca fascicularis	KLKB1	NCBI	NCBI:102142369
Others	KLKB1	NCBI

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