Page last updated: 2024-09-27

Angioedema, Hereditary, Type III

A form of hereditary angioedema that occurs in women and is precipitated or worsened by high ESTROGEN levels. It is associated with mutations in the gene for FACTOR XII that result in its increased activity.

Synonyms(8)

Synonym
Angioneurotic Edema, Hereditary, with Normal C1 Inhibitor Concentration and Function
Estrogen-Sensitive HAE
Estrogen-Sensitive Hereditary Angioedema
Hereditary Angioedema with Normal C1 Inhibitor Activity
Angioedema, Hereditary, Type III
Hereditary Angioedema Type III
Estrogen-Related HAE
HAE with Normal C1 Inhibitor Concentration and Function

Research

Studies (13)

TimeframeStudies, This Condition (%)All Conditions %
pre-19900 (0.00)23.3326
1990's0 (0.00)12.5806
2000's1 (7.69)18.1394
2010's11 (84.62)28.8240
2020's1 (7.69)9.53
DrugIndicatedRelationship StrengthStudiesTrials
tranexamic acid0low10
lysine0low30
medroxyprogesterone acetate0low10
threonine0low20
arginine0low10
danazol0low20
bradykinin0medium80
icatibant0low20
ecallantide0low30

Protein Targets (197)

ProteinPotency MeasurementsInhibition MeasurementsActivation MeasurementsDrugs
Chain A, MAJOR APURINIC/APYRIMIDINIC ENDONUCLEASE2002
Chain E, LYSINE, ARGININE, ORNITHINE-BINDING PROTEIN0011
Chain E, LYSINE, ARGININE, ORNITHINE-BINDING PROTEIN0011
Chain E, LYSINE, ARGININE, ORNITHINE-BINDING PROTEIN0011
thioredoxin reductase1001
phosphopantetheinyl transferase1001
NFKB1 protein, partial2002
GLS protein3003
thyroid stimulating hormone receptor2002
alpha-galactosidase1001
euchromatic histone-lysine N-methyltransferase 23003
cytochrome P450 2C19 precursor2002
chromobox protein homolog 12002
flap endonuclease 12002
DNA polymerase iota isoform a (long)3003
muscarinic acetylcholine receptor M12002
lethal factor (plasmid)1001
Cytochrome P450 3A40001
Nitric oxide synthase, endothelial0002
Nitric oxide synthase, brain0001
Nitric oxide synthase, brain 0001
Nitric oxide synthase, inducible0001
Nitric oxide synthase, inducible0013
Cationic amino acid transporter 30202
ATP-dependent phosphofructokinase4004
histone acetyltransferase KAT2A isoform 12002
Angiotensin-converting enzyme 0001
B2 bradykinin receptor0101
B2 bradykinin receptor0215
B1 bradykinin receptor0101
Olfactory receptor 51E20011
arylsulfatase A2002
atrial natriuretic peptide receptor 1 precursor1001
atrial natriuretic peptide receptor 2 precursor2002
ATP-binding cassette sub-family C member 30303
Multidrug resistance-associated protein 40303
Bile salt export pump0404
Histamine H1 receptor0404
MBT domain-containing protein 10101
Potassium voltage-gated channel subfamily H member 20101
Solute carrier family 22 member 10101
Lethal(3)malignant brain tumor-like protein 40101
Canalicular multispecific organic anion transporter 10303
Lethal(3)malignant brain tumor-like protein 30101
Ataxin-22002
Chloroquine resistance transporter0101
Solute carrier family 22 member 20101
Lethal(3)malignant brain tumor-like protein 10101
glp-1 receptor, partial1001
pregnane X receptor2002
hypoxia-inducible factor 1 alpha subunit1001
RAR-related orphan receptor gamma3003
SMAD family member 22002
SMAD family member 32002
TDP1 protein2002
GLI family zinc finger 32002
AR protein3003
estrogen receptor 2 (ER beta)2002
nuclear receptor subfamily 1, group I, member 32002
progesterone receptor2002
cytochrome P450 family 3 subfamily A polypeptide 43003
glucocorticoid receptor [Homo sapiens]2002
retinoic acid nuclear receptor alpha variant 13003
retinoid X nuclear receptor alpha2002
estrogen-related nuclear receptor alpha2002
farnesoid X nuclear receptor2002
pregnane X nuclear receptor2002
estrogen nuclear receptor alpha3003
G2002
cytochrome P450 2D62002
peroxisome proliferator-activated receptor delta2002
peroxisome proliferator activated receptor gamma2002
vitamin D (1,25- dihydroxyvitamin D3) receptor2002
cytochrome P450, family 19, subfamily A, polypeptide 1, isoform CRA_a2002
thyroid stimulating hormone receptor2002
v-jun sarcoma virus 17 oncogene homolog (avian)2002
nuclear receptor subfamily 1, group I, member 22002
Histone H2A.x1001
parathyroid hormone/parathyroid hormone-related peptide receptor precursor1001
thyroid hormone receptor beta isoform 22002
heat shock protein beta-11001
nuclear factor erythroid 2-related factor 2 isoform 12002
geminin2002
peripheral myelin protein 222002
Voltage-dependent calcium channel gamma-2 subunit2002
Rap guanine nucleotide exchange factor 31001
Interferon beta2002
HLA class I histocompatibility antigen, B alpha chain 2002
Estrogen receptor0314
Glucocorticoid receptor0415
Sex hormone-binding globulin0022
Cellular tumor antigen p532002
Cytochrome P450 1A20101
Glycine receptor subunit alpha-10404
Aldo-keto reductase family 1 member B10404
Alpha-2A adrenergic receptor0404
Adenosine receptor A30202
Cytochrome P450 2C9 0101
Beta-3 adrenergic receptor0202
Androgen receptor0404
Alpha-1B adrenergic receptor0202
Alpha-2B adrenergic receptor0404
Alpha-2C adrenergic receptor0202
Glutamate receptor 22002
Muscarinic acetylcholine receptor M30404
Glycine receptor subunit beta0404
Substance-K receptor0202
D(1A) dopamine receptor0202
Glycine receptor subunit alpha-20404
Sodium-dependent noradrenaline transporter 0404
Glycine receptor subunit alpha-30404
5-hydroxytryptamine receptor 2A0404
Sodium-dependent serotonin transporter0404
Mu-type opioid receptor0202
D(3) dopamine receptor0202
Kappa-type opioid receptor0202
Alpha-1A adrenergic receptor0202
Cytochrome P450 2J20202
Spike glycoprotein2002
Sodium-dependent dopamine transporter 0404
Inositol hexakisphosphate kinase 12002
ATPase family AAA domain-containing protein 51001
cytochrome P450 2C9, partial2002
DNA polymerase kappa isoform 11001
5-hydroxytryptamine receptor 40202
Muscarinic acetylcholine receptor M20202
Muscarinic acetylcholine receptor M40202
Muscarinic acetylcholine receptor M50202
Muscarinic acetylcholine receptor M10202
Histamine H2 receptor0404
Alpha-1D adrenergic receptor0202
5-hydroxytryptamine receptor 2C0202
Histamine H1 receptor0001
Histamine H2 receptor0001
Sigma non-opioid intracellular receptor 10202
Histamine H3 receptor0001
Chain A, Ribulose-1,5 bisphosphate carboxylase/oxygenase large subunit N-methyltransferase, chloroplast0101
Chain A, Ribulose-1,5 bisphosphate carboxylase/oxygenase large subunit N-methyltransferase, chloroplast0101
potassium voltage-gated channel subfamily H member 2 isoform d1001
Glutathione reductase, mitochondrial0202
Chain A, Beta-lactamase1001
Chain A, Cruzipain1001
interleukin 81001
ATAD5 protein, partial1001
aldehyde dehydrogenase 1 family, member A11001
PINK11001
nonstructural protein 11001
Parkin1001
IDH11001
nuclear factor of kappa light polypeptide gene enhancer in B-cells 1 (p105), isoform CRA_a1001
nuclear factor erythroid 2-related factor 2 isoform 21001
transcriptional regulator ERG isoform 31001
cytochrome P450 3A4 isoform 11001
lamin isoform A-delta103003
neuropeptide S receptor isoform A1001
Gamma-aminobutyric acid receptor subunit pi1001
Progesterone receptor0213
Mineralocorticoid receptor 0101
Androgen receptor0224
Gamma-aminobutyric acid receptor subunit beta-11001
Gamma-aminobutyric acid receptor subunit delta1001
Gamma-aminobutyric acid receptor subunit gamma-21001
Androgen receptor0011
Gamma-aminobutyric acid receptor subunit alpha-51001
Gamma-aminobutyric acid receptor subunit alpha-31001
Gamma-aminobutyric acid receptor subunit gamma-11001
Gamma-aminobutyric acid receptor subunit alpha-21001
Gamma-aminobutyric acid receptor subunit alpha-41001
Gamma-aminobutyric acid receptor subunit gamma-31001
5-hydroxytryptamine receptor 1B0101
Gamma-aminobutyric acid receptor subunit alpha-61001
Alpha-synuclein1001
Aldo-keto reductase family 1 member C30101
Nuclear receptor ROR-gamma1001
Aldo-keto reductase family 1 member C2 0101
Gamma-aminobutyric acid receptor subunit alpha-11001
Gamma-aminobutyric acid receptor subunit beta-31001
Guanine nucleotide-binding protein G1001
Gamma-aminobutyric acid receptor subunit beta-21001
Aldo-keto reductase family 1 member C10101
GABA theta subunit1001
Estrogen receptor beta0112
Gamma-aminobutyric acid receptor subunit epsilon1001
Chain A, HADH2 protein1001
Chain B, HADH2 protein1001
acetylcholinesterase1001
USP1 protein, partial1001
hypoxia-inducible factor 1, alpha subunit (basic helix-loop-helix transcription factor)1001
Carbonic anhydrase 10101
Carbonic anhydrase 20101
Histamine H4 receptor0101
activating transcription factor 61001
Chain A, PLASMINOGEN0011
Chain A, PLASMINOGEN0011
EWS/FLI fusion protein1001
Plasminogen0213
Urokinase-type plasminogen activator0101
[prepared from compound, protein, and bioassay information from National Library of Medicine (NLM), extracted Dec-2023]