Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2014 Feb 27;3(1):37-42.
eCollection 2014.

Kindlin-3 in the immune system

Susanna C Fagerholm  1 , Hwee San Lek  2 , Vicky L Morrison  2
Affiliations
Review

Kindlin-3 in the immune system

Susanna C Fagerholm et al. Am J Clin Exp Immunol. .

Abstract

Kindlin-3 is a member of the kindlin family of focal adhesion proteins which bind to integrin beta-chain cytoplasmic domains to regulate integrin function. In contrast to kindlin-1 and kindlin-2 proteins, kindlin-3 is expressed mainly in the hematopoietic system. Mutations in kindlin-3 result in the rare genetic disorder, leukocyte adhesion deficiency type III (LAD-III), which is characterized by bleeding and recurrent infections due to deficient beta1, beta2 and beta3 integrin activation in platelets and leukocytes. Here, we review the role of kindlin-3 in integrin activation and in different immune cell functions.

Keywords: Kindlin; LAD-III; T cell; adhesion; integrin.

PubMed Disclaimer

Figures

Figure 1
Figure 1
Schematic diagram of kindlin-3 functions in cells. The integrin binding site is in the F3 subdomain of kindlin-3.
See this image and copyright information in PMC

References

    1. Gahmberg CG, Fagerholm SC, Nurmi SM, Chavakis T, Marchesan S, Gronholm M. Regulation of integrin activity and signalling. Biochim Biophys Acta. 2009;1790:431–444. - PMC - PubMed
    1. MacPherson M, Lek HS, Morrison V, Fagerholm S. Leukocyte Beta2-Integrins; Genes and Disease. J Genet Syndr Gene Ther. 2013;4:154.
    1. Nurden AT, Fiore M, Nurden P, Pillois X. Glanzmann thrombasthenia: a review of ITGA2B and ITGB3 defects with emphasis on variants, phenotypic variability, and mouse models. Blood. 2011;118:5996–6005. - PubMed
    1. Takala H, Nurminen E, Nurmi SM, Aatonen M, Strandin T, Takatalo M, Kiema T, Gahmberg CG, Ylanne J, Fagerholm SC. Beta2 integrin phosphorylation on Thr758 acts as a molecular switch to regulate 14-3-3 and filamin binding. Blood. 2008;112:1853–1862. - PubMed
    1. Moser M, Bauer M, Schmid S, Ruppert R, Schmidt S, Sixt M, Wang HV, Sperandio M, Fassler R. Kindlin-3 is required for beta2 integrin-mediated leukocyte adhesion to endothelial cells. Nat Med. 2009;15:300–305. - PubMed

LinkOut - more resources