TY  - JOUR
A1  - Fekadu, Julia Hanna
A1  - Modlich, Ute
A1  - Bader, Peter
A1  - Bakhtiar, Shahrzad
T1  - Understanding the role of LFA-1 in leukocyte adhesion deficiency Type I (LAD I): moving towards inflammation?
T2  - International journal of molecular sciences
N2  - LFA-1 (Lymphocyte function-associated antigen-1) is a heterodimeric integrin (CD11a/CD18) present on the surface of all leukocytes; it is essential for leukocyte recruitment to the site of tissue inflammation, but also for other immunological processes such as T cell activation and formation of the immunological synapse. Absent or dysfunctional expression of LFA-1, caused by mutations in the ITGB2 (integrin subunit beta 2) gene, results in a rare immunodeficiency syndrome known as Leukocyte adhesion deficiency type I (LAD I). Patients suffering from severe LAD I present with recurrent infections of the skin and mucosa, as well as inflammatory symptoms complicating the clinical course of the disease before and after allogeneic hematopoietic stem cell transplantation (alloHSCT); alloHSCT is currently the only established curative treatment option. With this review, we aim to provide an overview of the intrinsic role of inflammation in LAD I.
KW  - inborn errors of immunity
KW  - inflammation
KW  - hematopoietic stem cell transplantation
Y1  - 2022
UR  - http://publikationen.ub.uni-frankfurt.de/frontdoor/index/index/docId/83152
UR  - https://nbn-resolving.org/urn:nbn:de:hebis:30:3-831528
SN  - 1422-0067
VL  - 23
IS  - 7, art.  3578
SP  - 1
EP  - 12
PB  - Molecular Diversity Preservation International
CY  - Basel
ER  -