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integrin, beta 2 subunit (complement component 3 receptor 3 and 4 subunit)

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Immunopharmacology Ligand target has curated data in GtoImmuPdb

Target id: 2456

Nomenclature: integrin, beta 2 subunit (complement component 3 receptor 3 and 4 subunit)

Family: Integrins

Quaternary Structure: Complexes
integrin αLβ2
Gene and Protein Information Click here for help
Species TM AA Chromosomal Location Gene Symbol Gene Name Reference
Human 1 769 21q22.3 ITGB2 integrin subunit beta 2
Mouse 1 771 10 39.72 cM Itgb2 integrin beta 2
Rat - 768 20p12 Itgb2 integrin subunit beta 2
Previous and Unofficial Names Click here for help
CD antigen CD18 | Integrin beta-2 | integrin, beta 2 | integrin, beta 2 (antigen CD18 (p95) | LFA-1 | lymphocyte function-associated antigen 1
Database Links Click here for help
Alphafold
CATH/Gene3D
ChEMBL Target
Ensembl Gene
Entrez Gene
Human Protein Atlas
KEGG Gene
OMIM
Pharos
RefSeq Nucleotide
RefSeq Protein
UniProtKB
Wikipedia

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Antibodies
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Antibody Sp. Action Value Parameter Reference
rovelizumab Peptide Primary target of this compound Click here for species-specific activity table Immunopharmacology Ligand Hs Binding - - 4,6
[4,6]
Immunopharmacology Comments
ITGB2 is the beta component of the β2 integrins. It forms αβ heterodimers with the CD11 alpha subunits ITGAL (αL aka CD11a forming integrin LFA-1), ITGAM (αM aka CD11b, forming integrin Mac-1) and ITGAX (αX aka CD11c, forming integrin p150/95). Beta2-integrins are essential for leukocyte extravasation to sites of infection (i.e. leukocyte trafficking), and other immunological processes including neutrophil phagocytosis and ROS production, and T cell activation. Their absence causes a leukocyte adhesion deficiency, which manifests clinically as recurrent severe infections, defective wound healing and neutrophilia [3].
Immuno Process Associations
Immuno Process:  Inflammation
Immuno Process:  Antigen presentation
Immuno Process:  Immune regulation
Immuno Process:  Cytokine production & signalling
Immuno Process:  Chemotaxis & migration
Immuno Process:  Cellular signalling
Immuno Process:  T cell (activation)
Immuno Disease Associations
Disease Name:  leukocyte adhesion deficiency type 1
Disease Synonyms:  no synonynms
Comment:  Multiple inherited mutations in the integrin β2 protein (CD18) that reduce expression or function have been identified as the causative factor in patients with LAD1.
Disease X-refs:  OMIM: 116920
References:  1-2,5
Clinically-Relevant Mutations and Pathophysiology Click here for help
Disease:  leukocyte adhesion deficiency type 1
Description: LAD1 is an inherited immunodeficiency disease. Mutations in the β2 intergrin (ITGB2) gene lead to absent, reduced, or aberrant CD18 protein expression, resulting in subnormal levels of β2 intergrins on leukocyte membranes. This disrupts neutrophil migration (extravasation) to sites of infection resulting in recurrent infections.
OMIM: 116920
References:  3

References

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1. Dinauer MC. (2019) Inflammatory consequences of inherited disorders affecting neutrophil function. Blood, 133 (20): 2130-2139. [PMID:30898864]

2. Fagerholm SC, Guenther C, Llort Asens M, Savinko T, Uotila LM. (2019) Beta2-Integrins and Interacting Proteins in Leukocyte Trafficking, Immune Suppression, and Immunodeficiency Disease. Front Immunol, 10: 254. [PMID:30837997]

3. Kishimoto TK, Hollander N, Roberts TM, Anderson DC, Springer TA. (1987) Heterogeneous mutations in the beta subunit common to the LFA-1, Mac-1, and p150,95 glycoproteins cause leukocyte adhesion deficiency. Cell, 50 (2): 193-202. [PMID:3594570]

4. Liles WC, Dale DC, Price TH, Gaviria JM, Turner T, Saoud J, Frumkin LR. (2000) Inhibition of in vivo neutrophil transmigration by a novel humanized anti-CD11/CD18 monoclonal antibody. Cytokines Cell Mol Ther, 6 (3): 121-6. [PMID:11140880]

5. Sun B, Chen Q, Dong X, Liu D, Hou J, Wang W, Ying W, Hui X, Zhou Q, Yao H et al.. (2019) Report of a Chinese Cohort with Leukocyte Adhesion Deficiency-I and Four Novel Mutations. J Clin Immunol, 39 (3): 309-315. [PMID:30919141]

6. Yenari MA, Kunis D, Sun GH, Onley D, Watson L, Turner S, Whitaker S, Steinberg GK. (1998) Hu23F2G, an antibody recognizing the leukocyte CD11/CD18 integrin, reduces injury in a rabbit model of transient focal cerebral ischemia. Exp Neurol, 153 (2): 223-33. [PMID:9784282]

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