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FLASH GENE

Symbol

KNG1

contributors: mct/npt/shn - updated : 28-11-2017

HGNC name	kininogen 1
HGNC id	6383

FLASH GENE DNA RNA EXPRESSION PROTEIN DISORDER ANIMAL & CELL MODELS

Corresponding disease	KNG kininogen deficiency
Location	3q27.3      Physical location : 186.435.097 - 186.462.198
Synonym name	kininostatin (HKD5)
	alpha-2-thiol proteinase inhibitor
	bradykinin
	T-kininogen 1
	kininogen-1
	fitzgerald factor
	high molecular weight kininogen
	williams-Fitzgerald-Flaujeac factor
Synonym symbol(s)	KNG, BDK, kininogen, HMWK, BK

DNA

TYPE	functioning gene
STRUCTURE	27.10 kb     11 Exon(s)

MAPPING

cloned

Y

linked

N

status

confirmed

Map	cen - D3S1262 - D3S357 - KNG1 - D3S243 - D3S3600 - qter

RNA

TRANSCRIPTS	type	messenger

identification nb exons type bp product Protein kDa AA specific expression Year Pubmed NM_000893 (transcript variant 2) 11 splicing 2143 NP_000884 - 427 - - NM_001102416 (transcript variant 1) 10 splicing 2732 NP_001095886 - 644 - - NM_001166451 (transcript variant 3) 10 splicing 2035 NP_001159923 - 391 - -

EXPRESSION

Type	restricted

expressed in	(based on citations)
organ(s)	System Organ level 1 Organ level 2 Organ level 3 Organ level 4 Level Pubmed Species Stage Rna symbol Digestive liver       highly Urinary kidney

cell lineage
cell lines
fluid/secretion	plasma

at STAGE

PROTEIN

PHYSICAL PROPERTIES

STRUCTURE

motifs/domains	four homologous repeats,D1,D2,D3 cysteine proteinase inhibitor-like and D4 releasing bradykinin or lys-bradykinin
	a D5 surface binding D5(H), that may modulate likely cell adhesion and invasion together with ACTN4, and inhibits collagen-mediated cancer cell adhesion and invasion in association with ACTN4
	a D6 binding site for kallikrein or factor XI (F11)

conjugated

GlycoP

isoforms

Precursor

coding for high molecular (HD,110kDa) and low molecular weight (LW,65kDa) kininogene

HOMOLOGY

interspecies	ortholog to kng1, Mus musculus
	ortholog to kng1, Rattus norvegicus

Homologene

FAMILY

cystatin superfamily

CATEGORY

enzyme

SUBCELLULAR LOCALIZATION	extracellular
text	secreted protein

basic FUNCTION	contact activation pathway
	cysteine proteinase inhibitor
	inhibiting endothelial cell proliferation and angiogenesis through domain D5
	may be involved in smooth muscle contraction, natriuresis, diuresis, inflammatory response, positive regulation of apoptosis
	antimicrobial activity of the heparin-binding and cell-binding domain 5 of high molecular weight kininogen
	playing an essential role in prevention of metastasis of cancer cells
	inhibits ERK-dependent T cell proliferation, and these observations may have a possible role in immunosenescence (Acuna-Castillo 2005)
	acts as an important signal directing the invasion of glioma cells toward blood vessels (
	multiple functions, including the regulation of coagulation

CELLULAR PROCESS	cell life, cell death/apoptosis

PHYSIOLOGICAL PROCESS

coagulation/hemostasis , inflammation

text	hemostasis

PATHWAY

metabolism

signaling

a component

INTERACTION

DNA

RNA

small molecule	metal binding,
	ion Zn2+ binding

protein	interacting with urokinase plasminogen activator receptor
	neutrophil-derived PRTN3 can proteolyze KNG1 and liberate PRTN3-kinin, thereby initiating kallikrein-independent activation of the kinin pathway

cell & other

REGULATION

Other

up-regulated by agonists of the farnesoid X receptor

ASSOCIATED DISORDERS

corresponding disease(s)

KNG

Susceptibility

to shortening of the activated partial thromboplastin time (aPTT)

Variant & Polymorphism SNP	rs710446 increasing the risk of aPTT (Houlihan 2010)

Candidate gene

Marker

Therapy target

ANIMAL & CELL MODELS

Kng1(-/-) mice are viable, but lacked plasma High-molecular-weight kininogen and low-molecular-weight kininogen , as well as DeltamHK-D5, displayed a significantly prolonged time to carotid artery occlusion following Rose Bengal administration and laser-induced arterial injury (