The Role of Bradykinin Receptors in Hereditary Angioedema Due to C1-Inhibitor Deficiency

被引：1

作者：

Dyga, Wojciech ^{[1
]}

Obtulowicz, Aleksander ^{[2
]}

Mikolajczyk, Tomasz ^{[3
]}

Bogdali, Anna ^{[1
]}

Dubiela, Pawel ^{[4
,5
]}

Obtulowicz, Krystyna ^{[1
]}

机构：

[1] Jagiellonian Univ Med Coll, Dept Clin & Environm Allergol, Botaniczna 3, PL-31503 Krakow, Poland

[2] Jagiellonian Univ Med Coll, Dept Dermatol, Kopernika 50, PL-31501 Krakow, Poland

[3] Jagiellonian Univ Med Coll, Dept Internal & Agr Med, Skarbowa 1, PL-31121 Krakow, Poland

[4] Med Univ Vienna, Dept Pathophysiol & Allergy Res, Waehringer Guertel 18-20, A-1090 Vienna, Austria

[5] Med Univ Bialystok, Dept Regenerat Med & Immune Regulat, Waszyngtona 13, PL-15269 Bialystok, Poland

来源：

INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES | 2022年 / 23卷 / 18期

关键词：

angioedema; bradykinin; bradykinin receptors; C1; inhibitor; HAE; MOLECULAR-WEIGHT KININOGEN; B-1; RECEPTOR; ENDOTHELIAL-CELLS; UP-REGULATION; EXPRESSION; BINDING; PATHOPHYSIOLOGY; PHARMACOLOGY; ANTAGONIST; ICATIBANT;

D O I：

10.3390/ijms231810332

中图分类号：

Q5 [生物化学]; Q7 [分子生物学];

学科分类号：

071010 ; 081704 ;

摘要：

Background: Hereditary angioedema (HAE) is a rare, genetic disease caused by the decreased level or function of the C1 inhibitor. The primary mediator of symptoms in HAE is bradykinin acting through its two receptors, namely receptors 1 (BR1) and 2 (BR2). Although BR2 is well characterized, the role of BR1 remains unclear. Objective: To study the role of bradykinin receptors 1 (BR1) in the etiopathogenesis of HAE. Methods: A total of 70 individuals, 40 patients with HAE, and 30 healthy subjects were recruited to the study. HAE was diagnosed in accordance with the international guideline. The level of bradykinin receptors was determined in populations of CD3(+), CD4(+), CD8(+), and CD14(++)CD16(-), CD14(++)CD16(+) monocytes. In addition, the level of disease activity-specific markers was measured. Results: There were statistically significant differences in the subpopulation of lymphocytes and monocytes between patients with HAE compared to healthy subjects. The level of BR1 and BR2 on PBMCs was comparable in healthy subjects and HAE patients during remission with significant overexpression of both receptors, triggered by HAE attack. Moreover, a significant increase in TNF-alpha and IL-1 plasma levels was observed among HAE patients. Conclusions: BR1 expression may play an important role in the pathomechanism of HAE.

引用

页数：12