학술논문
Biological Impact of Monoallelic and Biallelic BIRC3Loss in Del(11q) Chronic Lymphocytic Leukemia Progression
Document Type
Article
Author
Quijada Álamo, Miguel; Hernandez-Sanchez, Maria; Rodriguez, Ana E.; Pérez Carretero, Claudia; Martín Izquierdo, Marta; Alonso Pérez, Verónica; García-Tuñón, Ignacio; Bastida, Jose Maria; Vidal, Maria Jesus; Galende DEL Canto, Josefina; Aguilar, Carlos; Queizán, José Antonio; González-Gascón Y Marín, Isabel; Hernandez, Jose-Angel; Benito, Rocio; Ordóñez, José Luis; Hernández-Rivas, Jesus Maria
Source
Blood; November 2020, Vol. 136 Issue: 1, Number 1 Supplement 1 p4-4, 1p
Subject
Language
ISSN
00064971; 15280020
Abstract
Chronic lymphocytic leukemia (CLL) patients harboring 11q22.3 deletion, del(11q), are characterized by a rapid disease progression. One of the suggested genes to be involved in the pathogenesis of this deletion is BIRC3, a negative regulator of NF-κB, which is monoallelically deleted in ~80% of del(11q) CLL cases. In addition, truncating mutations in the remaining allele of this gene can lead to BIRC3biallelic inactivation, which accounts for marked reduced survival in CLL. Nevertheless, the biological mechanisms by which monoallelic or biallelic BIRC3lesions could contribute to del(11q) CLL pathogenesis, progression and therapy response are partially unexplored.