Conversion of the severe to the moderate disease phenotype with donor leukocyte microchimerism in canine leukocyte adhesion deficiency
نویسندگان
چکیده
منابع مشابه
Mixed chimeric hematopoietic stem cell transplant reverses the disease phenotype in canine leukocyte adhesion deficiency.
The genetic disease canine leukocyte adhesion deficiency (CLAD) is characterized by recurrent, severe bacterial infections, typically culminating in death by 6 months of age. CLAD is due to a mutation in the leukocyte integrin CD18 subunit, which prevents surface expression of the CD11/CD18 leukocyte integrin complex. We demonstrate that stable mixed donor:host hematopoietic chimerism, achieved...
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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...
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Leukocyte adhesion deficiency (LAD) is a heritable deficiency of the LFA-1, Mac-1, p150,95 family of leukocyte alpha beta heterodimers (the leukocyte integrins). We have studied the defect in patients who synthesize an aberrantly small form of the beta subunit common to all three proteins. S1 nuclease protection showed the presence of a 90-nucleotide mismatch in RNA from patients and relatives,...
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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...
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ژورنال
عنوان ژورنال: Bone Marrow Transplantation
سال: 2006
ISSN: 0268-3369,1476-5365
DOI: 10.1038/sj.bmt.1705291