Understanding the Role of LFA-1 in Leukocyte Adhesion Deficiency Type I (LAD I): Moving towards Inflammation?
Julia FekaduUte ModlichPeter BaderShahrzad BakhtiarPublished in: International journal of molecular sciences (2022)
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.
Keyphrases
- oxidative stress
- peripheral blood
- allogeneic hematopoietic stem cell transplantation
- end stage renal disease
- prognostic factors
- cell migration
- acute myeloid leukemia
- chronic kidney disease
- newly diagnosed
- cell adhesion
- ejection fraction
- acute lymphoblastic leukemia
- poor prognosis
- replacement therapy
- peritoneal dialysis
- biofilm formation
- genome wide
- copy number
- case report
- patient reported outcomes
- gene expression
- nk cells
- staphylococcus aureus
- cystic fibrosis
- depressive symptoms
- soft tissue
- binding protein
- patient reported
- drug induced
- sleep quality