A Novel Leukocyte Adhesion Deficiency III Variant: Kindlin-3 Deficiency Results in Integrin- and Nonintegrin-Related Defects in Different Steps of Leukocyte Adhesion
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چکیده
منابع مشابه
Periodontal Involvement in Leukocyte Adhesion Deficiency: Review of the Literature and a Case Report
Objective: Leukocyte adhesion deficiency(LAD) is a scarce, autosomal recessive inherited disorder . LAD-I which is the most common type occurs due to mutations on the CD18 gene. This mutation leads to absence or severe reduction of leukocyte cell surface expression of ß2 integrin molecules which are necessary for the adhesion of the leukocytes to the endothelial cells, transendothelial mi...
متن کاملLeukocyte Adhesion Deficiency Type 1 Presenting as Leukemoid Reaction
The hallmarks of leukocyte adhesion deficiency (LAD) are defects in the leukocyte adhesion process, marked leukocytosis and recurrent infections. These molecular and clinical manifestations result from an impaired step in the inflammatory process, namely, the emigration of leukocytes from the blood vessels to sites of infection, which requires adhesion of leukocytes to the endothelium. Over las...
متن کاملleukocyte adhesion deficiency: report of two family related newborn infants
leukocyte adhesion deficiency type 1 (lad 1) is an autosomal recessive hereditary disorder resulting from deficiency of cd18, characterized by recurrent bacterial infections. we report two consanguineous patients with leukocyte adhesion deficiency type 1( lad1). these two infant boy patients were referred to us, within a short period of time, with the complaints of recurrent infections at the a...
متن کاملA novel syndrome of variant leukocyte adhesion deficiency involving defects in adhesion mediated by b1 and b2 integrins
Leukocyte adhesion deficiency type I (LAD-1) is a disorder associated with severe and recurrent bacterial infections, impaired extravascular targeting and accumulation of myeloid leukocytes, altered wound healing, and significant morbidity that is caused by absent or greatly diminished surface expression of integrins of the b2 class. We report clinical features and analysis of functions of cell...
متن کاملA novel syndrome of variant leukocyte adhesion deficiency involving defects in adhesion mediated by beta1 and beta2 integrins.
Leukocyte adhesion deficiency type I (LAD-1) is a disorder associated with severe and recurrent bacterial infections, impaired extravascular targeting and accumulation of myeloid leukocytes, altered wound healing, and significant morbidity that is caused by absent or greatly diminished surface expression of integrins of the beta2 class. We report clinical features and analysis of functions of c...
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ژورنال
عنوان ژورنال: The Journal of Immunology
سال: 2011
ISSN: 0022-1767,1550-6606
DOI: 10.4049/jimmunol.1003141