KNG1

Chr 3q27.3

kininogen 1

Aliases:: BK, HMWK
MANE:: ENST00000644859.2

OMIM: 612358 ↗NCBI: 3827 ↗Ensembl ↗

Annotations refreshed 1 month ago.

Predicted protein structure

AlphaFold predicted structure

KNG1 · P01042

v62025-08-01

Predicted Aligned Error matrix for KNG1 — diagonal shows residue index, colour shows pairwise prediction confidence (lower is better) — Predicted Aligned Error

Mean pLDDT

65.9/ 100

Low

644 residues

Confidence breakdown

Very high(≥ 90)30%
Confident(70–90)20%
Low(50–70)10%
Very low(< 50)40%

Open interactive 3D viewer

AlphaFold (Jumper et al., 2021) · CC BY 4.0

Clinical relevance (Genomics England PanelApp)

Diagnostic Grade (Green)

Bleeding and platelet disorders
BIALLELIC, autosomal or pseudoautosomal
Inherited bleeding disorders
BIALLELIC, autosomal or pseudoautosomal

Disease associations (Open Targets)

congenital high-molecular-weight kininogen deficiency
0.73
venous thromboembolism
0.50
hereditary angioedema
0.46
serum lipopolysaccharide activity
0.34
ischemic stroke
0.33
coronary artery disorder
0.33
Thrombophlebitis
0.31
phlebitis
0.31
alcohol drinking
0.25
premature birth
0.23

Score is the Open Targets composite evidence score (0-1). Higher = stronger gene-disease association.

Protein function (UniProt)

Kininogen-1

Kininogens are inhibitors of thiol proteases. HMW-kininogen plays an important role in blood coagulation by helping to position optimally prekallikrein and factor XI next to factor XII; HMW-kininogen inhibits the thrombin- and plasmin-induced aggregation of thrombocytes. LMW-kininogen inhibits the aggregation of thrombocytes. LMW-kininogen is in contrast to HMW-kininogen not involved in blood clotting