Angioedema, Hereditary, Type I

Forms of hereditary angioedema that occur due to mutations in the gene for COMPLEMENT C1 INHIBITOR PROTEIN. Type I hereditary angioedema is associated with reduced serum levels of complement C1 inhibitor protein. Type II hereditary angioedema is associated with the production of a non-functional complement C1 inhibitor protein.

Synonym
Hereditary Angioedema Types I and II
Hereditary Angioedema Type 1
Hereditary Angioedema Type II
Hereditary Angioedema Type I
C1 Esterase Inhibitor, Deficiency Of
Angioedema, Hereditary, Types I and II
Deficiency of C1 Esterase Inhibitor
Angioedema, Hereditary, Type II
Angioedema, Hereditary, Type I

Excerpt	Reference
"Among patients with hereditary angioedema type I or II, treatment with subcutaneous lanadelumab for 26 weeks significantly reduced the attack rate compared with placebo."	( Anderson, J; Banerji, A; Baptista, J; Bernstein, JA; Busse, PJ; Cicardi, M; Craig, T; Davis-Lorton, M; Gierer, S; Hebert, J; Jacobs, J; Johnston, DT; Li, HH; Longhurst, HJ; Lumry, WR; Magerl, M; Manning, ME; Martinez-Saguer, I; Maurer, M; Nothaft, W; Rehman, SM; Riedl, MA; Schranz, J; Schwartz, LB; Shapiro, R; Shennak, M; Smith, AM; Soteres, D; Staubach, P; Tachdjian, R; Wedner, HJ; Yang, WH; Zanichelli, A; Zaragoza-Urdaz, RH; Zuraw, BL, 2018)

Timeframe	Studies, This Condition (%)	All Conditions %
pre-1990	0 (0.00)	23.3326
1990's	0 (0.00)	12.5806
2000's	1 (1.54)	18.1394
2010's	56 (86.15)	28.8240
2020's	8 (12.31)	9.53

Drug	Relationship Strength	Studies	Trials
histamine	low	1	0
cetirizine	low	1	0
tranexamic acid	low	9	0
stanozolol	low	1	0
danazol	low	12	0
fibrinogen	low	1	0
bradykinin	medium	44	2
inositol 3-phosphate	low	1	0
arginine vasopressin	low	1	0
levocetirizine	low	1	0
brefeldin a	low	1	0
kallidin	low	1	0
r 715	low	1	0
nitrofurantoin	low	1	0
icatibant	medium	26	2
endothelin-1	low	1	0
hyaluronoglucosaminidase	medium	1	1
adrenomedullin	low	1	0
ecallantide	medium	11	2
thromboplastin	low	1	0

Protein Targets (269)

Protein	Potency Measurements	Inhibition Measurements	Activation Measurements	Drugs
vitamin D3 receptor isoform VDRA	1	0	0	1
peripheral myelin protein 22	4	0	0	4
ATP-binding cassette sub-family C member 3	0	4	0	4
Multidrug resistance-associated protein 4	0	4	0	4
Bile salt export pump	0	5	0	5
Translocator protein	0	0	1	1
Vasopressin V2 receptor	0	2	1	4
Oxytocin receptor	0	1	1	2
Vasopressin V1a receptor	0	0	2	2
Vasopressin V1a receptor	0	1	1	2
Vasopressin V1b receptor	0	1	1	2
Vasopressin V2 receptor	0	0	1	1
Vasopressin V1b receptor	0	1	1	3
Translocator protein	0	1	0	2
Oxytocin receptor	0	1	0	1
Vasopressin V2 receptor	0	1	1	2
Canalicular multispecific organic anion transporter 1	0	4	0	4
histone acetyltransferase KAT2A isoform 1	1	0	0	1
Angiotensin-converting enzyme	0	0	0	1
B2 bradykinin receptor	0	1	0	1
B2 bradykinin receptor	0	2	1	5
B1 bradykinin receptor	0	1	0	1
Olfactory receptor 51E2	0	0	1	1
PAX8	0	0	0	1
thioredoxin reductase	3	0	0	3
ATAD5 protein, partial	2	0	0	2
USP1 protein, partial	5	0	0	5
TDP1 protein	4	0	0	4
thioredoxin glutathione reductase	2	0	0	2
Smad3	1	0	0	1
67.9K protein	2	0	0	2
IDH1	3	0	0	3
euchromatic histone-lysine N-methyltransferase 2	4	0	0	4
NPC intracellular cholesterol transporter 1 precursor	1	0	0	1
major prion protein preproprotein Prp precursor	0	1	0	1
nuclear receptor subfamily 0 group B member 1	0	1	0	1
chromobox protein homolog 1	1	0	0	1
thyroid hormone receptor beta isoform a	2	0	0	2
nuclear factor erythroid 2-related factor 2 isoform 2	1	0	0	1
ras-related protein Rab-9A	1	0	0	1
steroidogenic factor 1	0	1	0	1
urokinase-type plasminogen activator precursor	2	0	0	2
plasminogen precursor	2	0	0	2
urokinase plasminogen activator surface receptor precursor	2	0	0	2
geminin	4	0	0	4
atrial natriuretic peptide receptor 2 precursor	1	0	0	1
Histamine H1 receptor	0	3	2	5
Angiopoietin-1 receptor	0	0	1	1
Potassium voltage-gated channel subfamily H member 2	0	1	0	1
Multidrug and toxin extrusion protein 1	0	2	0	2
Histamine H4 receptor	0	2	2	4
ATP-dependent phosphofructokinase	2	0	0	2
glp-1 receptor, partial	1	0	0	1
pregnane X receptor	2	0	0	2
hypoxia-inducible factor 1 alpha subunit	3	0	0	3
RAR-related orphan receptor gamma	4	0	0	4
SMAD family member 2	2	0	0	2
SMAD family member 3	2	0	0	2
GLI family zinc finger 3	4	0	0	4
AR protein	5	0	0	5
estrogen receptor 2 (ER beta)	4	0	0	4
nuclear receptor subfamily 1, group I, member 3	3	0	0	3
progesterone receptor	4	0	0	4
cytochrome P450 family 3 subfamily A polypeptide 4	4	0	0	4
glucocorticoid receptor [Homo sapiens]	3	0	0	3
retinoic acid nuclear receptor alpha variant 1	3	0	0	3
retinoid X nuclear receptor alpha	4	0	0	4
estrogen-related nuclear receptor alpha	4	0	0	4
farnesoid X nuclear receptor	3	0	0	3
pregnane X nuclear receptor	3	0	0	3
estrogen nuclear receptor alpha	4	0	0	4
G	3	0	0	3
cytochrome P450 2D6	4	0	0	4
peroxisome proliferator-activated receptor delta	3	0	0	3
peroxisome proliferator activated receptor gamma	3	0	0	3
vitamin D (1,25- dihydroxyvitamin D3) receptor	2	0	0	2
cytochrome P450, family 19, subfamily A, polypeptide 1, isoform CRA_a	3	0	0	3
thyroid stimulating hormone receptor	2	0	0	2
v-jun sarcoma virus 17 oncogene homolog (avian)	3	0	0	3
nuclear receptor subfamily 1, group I, member 2	1	0	0	1
Histone H2A.x	1	0	0	1
parathyroid hormone/parathyroid hormone-related peptide receptor precursor	1	0	0	1
thyroid hormone receptor beta isoform 2	3	0	0	3
heat shock protein beta-1	3	0	0	3
nuclear factor erythroid 2-related factor 2 isoform 1	4	0	0	4
Voltage-dependent calcium channel gamma-2 subunit	3	0	0	3
Rap guanine nucleotide exchange factor 3	1	0	0	1
Interferon beta	3	0	0	3
HLA class I histocompatibility antigen, B alpha chain	3	0	0	3
Estrogen receptor	0	6	1	7
Glucocorticoid receptor	0	4	0	4
Sex hormone-binding globulin	0	0	2	2
Cellular tumor antigen p53	3	0	0	3
Cytochrome P450 1A2	0	1	0	1
Glycine receptor subunit alpha-1	0	4	0	4
Aldo-keto reductase family 1 member B1	0	4	0	4
Alpha-2A adrenergic receptor	0	2	0	2
Adenosine receptor A3	0	6	0	6
Cytochrome P450 2C9	0	2	0	2
Beta-3 adrenergic receptor	0	2	0	2
Androgen receptor	0	6	0	6
Alpha-1B adrenergic receptor	0	6	0	6
Alpha-2B adrenergic receptor	0	2	0	2
Alpha-2C adrenergic receptor	0	2	0	2
Glutamate receptor 2	3	0	0	3
Muscarinic acetylcholine receptor M3	0	2	0	2
Glycine receptor subunit beta	0	4	0	4
Substance-K receptor	0	2	0	2
D(1A) dopamine receptor	0	2	0	2
Glycine receptor subunit alpha-2	0	4	0	4
Sodium-dependent noradrenaline transporter	0	4	0	4
Glycine receptor subunit alpha-3	0	4	0	4
5-hydroxytryptamine receptor 2A	0	2	0	2
Sodium-dependent serotonin transporter	0	4	0	4
Mu-type opioid receptor	0	4	0	4
D(3) dopamine receptor	0	2	0	2
Kappa-type opioid receptor	0	4	0	4
Alpha-1A adrenergic receptor	0	6	0	6
Cytochrome P450 2J2	0	2	0	2
Spike glycoprotein	1	0	0	1
Sodium-dependent dopamine transporter	0	4	0	4
Inositol hexakisphosphate kinase 1	3	0	0	3
ATPase family AAA domain-containing protein 5	2	0	0	2
Ataxin-2	2	0	0	2
cytochrome P450 2C9, partial	3	0	0	3
Endothelin-1 receptor	0	1	0	1
Luciferase	1	0	0	1
interleukin 8	1	0	0	1
Fumarate hydratase	1	0	0	1
PPM1D protein	1	0	0	1
Microtubule-associated protein tau	2	0	0	2
caspase 7, apoptosis-related cysteine protease	1	0	0	1
thyroid stimulating hormone receptor	3	0	0	3
polyprotein	1	0	0	1
caspase-3	1	0	0	1
activating transcription factor 6	1	0	0	1
nuclear factor of kappa light polypeptide gene enhancer in B-cells 1 (p105), isoform CRA_a	2	0	0	2
lysosomal alpha-glucosidase preproprotein	1	0	0	1
Vpr	1	0	0	1
cytochrome P450 3A4 isoform 1	2	0	0	2
lamin isoform A-delta10	4	0	0	4
Gamma-aminobutyric acid receptor subunit pi	2	0	0	2
Solute carrier family 22 member 2	0	1	0	2
Cytochrome P450 2B1	0	1	0	1
Cytochrome P450 3A4	0	1	0	1
Neuronal acetylcholine receptor subunit alpha-4	1	0	0	1
Neuronal acetylcholine receptor subunit beta-2	1	0	0	1
Polyunsaturated fatty acid lipoxygenase ALOX15	0	1	0	1
Gamma-aminobutyric acid receptor subunit beta-1	2	0	0	2
Gamma-aminobutyric acid receptor subunit delta	2	0	0	2
Gamma-aminobutyric acid receptor subunit gamma-2	2	0	0	2
Gamma-aminobutyric acid receptor subunit alpha-5	2	0	0	2
Gamma-aminobutyric acid receptor subunit alpha-3	2	0	0	2
Cytochrome P450 2B6	0	1	0	1
UDP-glucuronosyltransferase 1A1	0	1	0	2
Gamma-aminobutyric acid receptor subunit gamma-1	2	0	0	2
Gamma-aminobutyric acid receptor subunit alpha-2	2	0	0	2
3-oxo-5-alpha-steroid 4-dehydrogenase 1	0	1	0	1
Gamma-aminobutyric acid receptor subunit alpha-4	2	0	0	2
Gamma-aminobutyric acid receptor subunit gamma-3	2	0	0	2
Adenosine receptor A2a	0	2	0	2
Gamma-aminobutyric acid receptor subunit alpha-6	2	0	0	2
Sulfotransferase 1A1	0	2	1	4
Nuclear receptor ROR-gamma	2	0	0	2
Gamma-aminobutyric acid receptor subunit alpha-1	2	0	0	2
Gamma-aminobutyric acid receptor subunit beta-3	2	0	0	2
Gamma-aminobutyric acid receptor subunit beta-2	2	0	0	2
Aldehyde oxidase	0	1	0	1
TAR DNA-binding protein 43	1	0	0	1
Nuclear receptor subfamily 1 group I member 3	0	1	0	1
Multidrug and toxin extrusion protein 2	0	1	0	1
GABA theta subunit	2	0	0	2
Estrogen receptor beta	0	1	1	2
Gamma-aminobutyric acid receptor subunit epsilon	2	0	0	2
Chain A, CARBONIC ANHYDRASE II	0	0	1	1
Chain A, MAJOR APURINIC/APYRIMIDINIC ENDONUCLEASE	1	0	0	1
Chain A, Protein (female-specific Histamine Binding Protein 2)	0	0	1	1
Chain A, Beta-lactamase	1	0	0	1
aryl hydrocarbon receptor nuclear translocator	0	0	0	1
phosphopantetheinyl transferase	2	0	0	2
GALC protein	1	0	0	1
GLS protein	1	0	0	1
apical membrane antigen 1, AMA1	1	0	0	1
regulator of G-protein signaling 4	1	0	0	1
EWS/FLI fusion protein	2	0	0	2
bromodomain adjacent to zinc finger domain 2B	2	0	0	2
potassium voltage-gated channel subfamily H member 2 isoform d	2	0	0	2
transforming acidic coiled-coil-containing protein 3	0	0	0	1
DNA polymerase iota isoform a (long)	2	0	0	2
Solute carrier family 22 member 1	0	0	0	1
Carbonic anhydrase 12	0	0	0	1
Solute carrier family 22 member 3	0	1	0	2
Carbonic anhydrase 1	0	0	0	1
Carbonic anhydrase 2	0	0	0	2
Plasma kallikrein	0	0	0	1
Carbonic anhydrase 3	0	0	0	1
Macrophage migration inhibitory factor	0	2	0	2
Carbonic anhydrase 4	0	0	0	1
Carbonic anhydrase 6	0	0	0	1
Histamine H2 receptor	0	1	2	3
Adenosine receptor A1	0	0	0	1
5-hydroxytryptamine receptor 1D	0	0	0	1
Adenosine receptor A2a	0	0	0	1
Histamine H1 receptor	0	1	2	3
Histamine H1 receptor	0	2	1	3
Metabotropic glutamate receptor 5	0	1	1	2
Carbonic anhydrase 5A, mitochondrial	0	0	0	1
Glutamate receptor ionotropic, NMDA 1	0	0	0	1
Carbonic anhydrase	0	0	0	1
Carbonic anhydrase 7	0	0	0	1
Histamine H2 receptor	0	0	1	1
Carbonic anhydrase	0	0	0	2
Histamine H3 receptor	0	1	0	1
Glutamate receptor ionotropic, NMDA 2A	0	0	0	1
Glutamate receptor ionotropic, NMDA 2B	0	0	0	1
Glutamate receptor ionotropic, NMDA 2C	0	0	0	1
Histamine N-methyltransferase	0	1	0	1
Glutaminyl-peptide cyclotransferase	0	1	0	1
Carbonic anhydrase 9	0	0	0	1
Carbonic anhydrase	0	0	0	2
Carbonic anhydrase-like protein, putative	0	0	0	1
Carbonic anhydrase	0	0	0	2
Carbonic anhydrase	0	0	0	2
Glutamate receptor ionotropic, NMDA 2D	0	0	0	1
Solute carrier family 22 member 1	0	1	0	2
Carbonic anhydrase	0	0	0	2
Equilibrative nucleoside transporter 4	0	0	0	1
Carbonic anhydrase 13	0	0	0	1
Glutamate receptor ionotropic, NMDA 3B	0	0	0	1
Histamine H4 receptor	0	1	2	3
Histamine H4 receptor	0	1	2	3
Histamine H4 receptor	0	1	0	1
Carbonic anhydrase 4	0	0	0	1
Carbonic anhydrase 15	0	0	0	1
Carbonic anhydrase 13	0	0	0	1
Histamine H3 receptor	0	1	0	1
Carboxylic ester hydrolase	0	0	0	1
Solute carrier family 22 member 2	0	1	0	2
Glutamate receptor ionotropic, NMDA 3A	0	0	0	1
Carbonic anhydrase 14	0	0	0	1
Carbonic anhydrase 5B, mitochondrial	0	0	0	1
Histamine H3 receptor	0	1	2	3
Chain A, JmjC domain-containing histone demethylation protein 3A	1	0	0	1
beta-2 adrenergic receptor	1	0	0	1
transcriptional regulator ERG isoform 3	1	0	0	1
Chain A, TYROSYL-DNA PHOSPHODIESTERASE	1	0	0	1
Chain A, Putative fructose-1,6-bisphosphate aldolase	1	0	0	1
Chain A, 2-oxoglutarate Oxygenase	1	0	0	1
acetylcholinesterase	1	0	0	1
glucocerebrosidase	1	0	0	1
aryl hydrocarbon receptor	1	0	0	1
DNA polymerase kappa isoform 1	1	0	0	1
survival motor neuron protein isoform d	1	0	0	1
muscleblind-like protein 1 isoform 1	1	0	0	1
streptokinase A precursor	0	0	1	1
lethal factor (plasmid)	1	0	0	1
Bile salt export pump	0	1	0	1
Estrogen receptor	0	0	1	1
Caspase-7	1	0	0	1
60 kDa chaperonin	0	1	0	1
Estrogen receptor beta	0	0	1	1
10 kDa chaperonin	0	1	0	1
CDGSH iron-sulfur domain-containing protein 1	0	2	0	2
Cytochrome P450 2D6	0	1	0	1
Cytochrome P450 2C19	0	1	0	1
Chain A, PLASMINOGEN	0	0	1	1
Chain A, PLASMINOGEN	0	0	1	1
Plasminogen	0	2	1	3
Urokinase-type plasminogen activator	0	1	0	1
[prepared from compound, protein, and bioassay information from National Library of Medicine (NLM), extracted Dec-2023]

Angioedema, Hereditary, Type I

Synonyms(9)

Research Excerpts

Treatment

Research

Studies (65)

Protein Targets (269)

Angioedema, Hereditary, Type I

Synonyms(9)

Research Excerpts

Treatment

Research

Studies (65)

Drugs Studied (21)

Protein Targets (269)