[HTML][HTML] Hereditary angioedema: diagnosis, clinical implications, and pathophysiology

ES Sinnathamby, PP Issa, L Roberts, H Norwood… - Advances in …, 2023 - Springer
Hereditary angioedema (HAE) is an autosomal dominant disorder caused by a mutation in
the C1 esterase inhibitor gene. HAE affects 1/50,000 people worldwide. Three main types of …

Recent advances in factor XII structure and function

A Shamanaev, M Litvak, D Gailani - Current opinion in hematology, 2022 - journals.lww.com
We propose a model in which FXII is normally maintained in a closed conformation resistant
to activation by intramolecular interactions involving the fibronectin type-2 and kringle …

[HTML][HTML] Mechanisms involved in hereditary angioedema with normal C1-inhibitor activity

A Shamanaev, SK Dickeson, I Ivanov, M Litvak… - Frontiers in …, 2023 - frontiersin.org
Patients with the inherited disorder hereditary angioedema (HAE) suffer from episodes of
soft tissue swelling due to excessive bradykinin production. In most cases, dysregulation of …

Factor XII Structure–Function Relationships

A Shamanaev, M Litvak, I Ivanov… - … in Thrombosis and …, 2023 - thieme-connect.com
Factor XII (FXII), the zymogen of the protease FXIIa, contributes to pathologic processes
such as bradykinin-dependent angioedema and thrombosis through its capacity to convert …

Angiotensin-converting enzyme inhibitor–induced angioedema

RG Wilkerson, ME Winters - Emergency Medicine Clinics, 2022 - emed.theclinics.com
Background ACEi medications were developed after it was noted that people bitten by the
Brazilian pit viper Bothrops jararaca developed profound hypotension and frequently died …

Pharmacological suppression of the kallikrein kinin system with KVD900: an orally available plasma kallikrein inhibitor for the on‐demand treatment of hereditary …

EJ Duckworth, N Murugesan, L Li… - Clinical & …, 2022 - Wiley Online Library
Background Hereditary angioedema (HAE) is a rare genetic disease that leads to recurrent
episodes of swelling and pain caused by uncontrolled plasma kallikrein (PKa) activity …

[HTML][HTML] Neurologic and psychiatric manifestations of bradykinin-mediated angioedema: old and new challenges

I Mormile, F Palestra, A Petraroli, S Loffredo… - International Journal of …, 2023 - mdpi.com
Neurologic manifestations have been occasionally described in patients with bradykinin-
mediated angioedema. The existing literature is currently limited to case series and case …

The MOE modification of RNA: Origins and widescale impact on the oligonucleotide therapeutics field

AC Hill, J Hall - Helvetica Chimica Acta, 2023 - Wiley Online Library
In an article published by Helvetica Chimica Acta in 1995, chemist P. Martin describes the
synthesis of 2′‐O‐alkylated ribonucleosides for use in therapeutic antisense …

[HTML][HTML] Insights into the pathogenesis of hereditary angioedema using genetic sequencing and recombinant protein expression analyses

Z Ren, S Zhao, T Li, HJ Wedner, JP Atkinson - Journal of Allergy and …, 2023 - Elsevier
Background The pathogenesis of hereditary angioedema (HAE) type I and type II is linked to
defective C1 esterase inhibitor (C1-INH) encoded by the SERPING1 gene. There are …

Genetic variants leading to urticaria and angioedema and associated biomarkers

JJ Lyons, H Farkas, AE Germenis, M Rijavec… - The Journal of Allergy …, 2023 - Elsevier
Advances in next generation sequencing technologies, as well as their expanded
accessibility and clinical use over the past 2 decades, have led to an exponential increase in …