Linked Life Data

11753075

Source:http://linkedlifedata.com/resource/pubmed/id/11753075

Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
1
pubmed:dateCreated
2001-12-25
pubmed:abstractText
Leukocyte adhesion deficiency (LAD) syndromes are failures of innate host defenses against bacteria, fungi, and other microorganisms resulting from defective tethering, adhesion, and targeting of myeloid leukocytes to sites of microbial invasion. LAD I and variant LAD I syndromes are caused by mutations that impair expression or function of integrins of the beta 2 class (CD11/CD18 integrins, or "leukocyte" integrins). In contrast, subjects with LAD II have similar clinical features but intact leukocyte integrin expression and function. The molecular basis for LAD II is defective glycosylation of ligands on leukocytes recognized by the selectin family of adhesion molecules as well as defective glycosylation of other glycoconjugates. The defect has recently been attributed to mutations in a novel fucose transporter localized to the Golgi apparatus. Establishing the molecular basis for LAD syndromes has generated insights into mechanisms of leukocyte accumulation relevant to a broad variety of immunodeficiency syndromes as well as to diseases and disorders of unregulated inflammation that result in tissue damage.
pubmed:grant
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
IM
pubmed:chemical
pubmed:status
MEDLINE
pubmed:month
Jan
pubmed:issn
1065-6251
pubmed:author
pubmed:issnType
Print
pubmed:volume
9
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
30-5
pubmed:dateRevised
2007-11-14
pubmed:meshHeading
pubmed:year
2002
pubmed:articleTitle
Leukocyte adhesion deficiency syndromes: adhesion and tethering defects involving beta 2 integrins and selectin ligands.
pubmed:affiliation
Program in Human Molecular Biology and Genetics, Huntsman Cancer Institute, Department of Internal Medicine, The University of Utah, Salt Lake City, Utah, USA.
pubmed:publicationType
Journal Article, Research Support, U.S. Gov't, P.H.S., Review, Research Support, Non-U.S. Gov't