The Role of Bradykinin Receptors in Hereditary Angioedema Due to C1-Inhibitor Deficiency
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...
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MDPI AG
2022-09-01
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| Series: | International Journal of Molecular Sciences |
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| Online Access: | https://www.mdpi.com/1422-0067/23/18/10332 |
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| author | Wojciech Dyga Aleksander Obtulowicz Tomasz Mikolajczyk Anna Bogdali Pawel Dubiela Krystyna Obtulowicz |
| author_facet | Wojciech Dyga Aleksander Obtulowicz Tomasz Mikolajczyk Anna Bogdali Pawel Dubiela Krystyna Obtulowicz |
| author_sort | Wojciech Dyga |
| collection | DOAJ |
| description | 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<sup>+</sup>, CD4<sup>+</sup>, CD8<sup>+</sup>, and CD14<sup>++</sup>CD16<sup>−</sup>, CD14<sup>++</sup>CD16<sup>+</sup> 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. |
| first_indexed | 2024-03-09T23:48:00Z |
| format | Article |
| id | doaj.art-aa62bbb6ddb446c695abe3076c2865bd |
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| issn | 1661-6596 1422-0067 |
| language | English |
| last_indexed | 2024-03-09T23:48:00Z |
| publishDate | 2022-09-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | International Journal of Molecular Sciences |
| spelling | doaj.art-aa62bbb6ddb446c695abe3076c2865bd2023-11-23T16:39:41ZengMDPI AGInternational Journal of Molecular Sciences1661-65961422-00672022-09-0123181033210.3390/ijms231810332The Role of Bradykinin Receptors in Hereditary Angioedema Due to C1-Inhibitor DeficiencyWojciech Dyga0Aleksander Obtulowicz1Tomasz Mikolajczyk2Anna Bogdali3Pawel Dubiela4Krystyna Obtulowicz5Department of Clinical and Environmental Allergology, Jagiellonian University Medical College, Botaniczna 3, 31-503 Krakow, PolandDepartment of Dermatology, Jagiellonian University Medical College, Kopernika 50, 31-501 Krakow, PolandDepartment of Internal and Agricultural Medicine, Jagiellonian University Medical College, Skarbowa 1, 31-121 Krakow, PolandDepartment of Clinical and Environmental Allergology, Jagiellonian University Medical College, Botaniczna 3, 31-503 Krakow, PolandDepartment of Pathophysiology and Allergy Research, Medical University of Vienna, Waehringer Guertel 18-20, A-1090 Vienna, AustriaDepartment of Clinical and Environmental Allergology, Jagiellonian University Medical College, Botaniczna 3, 31-503 Krakow, PolandBackground: 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<sup>+</sup>, CD4<sup>+</sup>, CD8<sup>+</sup>, and CD14<sup>++</sup>CD16<sup>−</sup>, CD14<sup>++</sup>CD16<sup>+</sup> 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.https://www.mdpi.com/1422-0067/23/18/10332angioedemabradykininbradykinin receptorsC1 inhibitorHAE |
| spellingShingle | Wojciech Dyga Aleksander Obtulowicz Tomasz Mikolajczyk Anna Bogdali Pawel Dubiela Krystyna Obtulowicz The Role of Bradykinin Receptors in Hereditary Angioedema Due to C1-Inhibitor Deficiency International Journal of Molecular Sciences angioedema bradykinin bradykinin receptors C1 inhibitor HAE |
| title | The Role of Bradykinin Receptors in Hereditary Angioedema Due to C1-Inhibitor Deficiency |
| title_full | The Role of Bradykinin Receptors in Hereditary Angioedema Due to C1-Inhibitor Deficiency |
| title_fullStr | The Role of Bradykinin Receptors in Hereditary Angioedema Due to C1-Inhibitor Deficiency |
| title_full_unstemmed | The Role of Bradykinin Receptors in Hereditary Angioedema Due to C1-Inhibitor Deficiency |
| title_short | The Role of Bradykinin Receptors in Hereditary Angioedema Due to C1-Inhibitor Deficiency |
| title_sort | role of bradykinin receptors in hereditary angioedema due to c1 inhibitor deficiency |
| topic | angioedema bradykinin bradykinin receptors C1 inhibitor HAE |
| url | https://www.mdpi.com/1422-0067/23/18/10332 |
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