Leukocyte Adhesion Deficiency Type II is a generalized defect of de novo GDP-fucose biosynthesis. Endothelial cell fucosylation is not required for neutrophil rolling on human nonlymphoid endothelium.

@article{Karsan1998LeukocyteAD,
  title={Leukocyte Adhesion Deficiency Type II is a generalized defect of de novo GDP-fucose biosynthesis. Endothelial cell fucosylation is not required for neutrophil rolling on human nonlymphoid endothelium.},
  author={Aly Karsan and Carol J. Cornejo and Robert K. Winn and Barbara R. Schwartz and William Way and N Lannir and Ruth Gershoni-baruch and Amos Etzioni and Hans D. Ochs and John Marshall Harlan},
  journal={The Journal of clinical investigation},
  year={1998},
  volume={101 11},
  pages={
          2438-45
        }
}
Leukocyte Adhesion Deficiency Type II (LAD II) is a recently described syndrome and the two patients with this defect lack fucosylated glycoconjugates. These glycoconjugates include the selectin ligand, sialyl LewisX, and various fucosylated blood group antigens. To date, the molecular anomaly in these patients has not been identified. We localized the defect in LAD II to the de novo pathway of GDP-fucose biosynthesis, by inducing cell-surface expression of fucosylated glycoconjugates after… 
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Leukocyte adhesion deficiency type II.
The gene defective in leukocyte adhesion deficiency II encodes a putative GDP-fucose transporter
TLDR
The first putative GDP-fucose transporter is identified, which has been highly conserved throughout evolution, and a point mutation in its gene is responsible for the disease in this patient with LAD II.
Leukocyte Adhesion Deficiency II: Therapy and Genetic Defect
TLDR
LAD II represents the first developmental and immune defect that is based on a malfunctioning nucleotide sugar transporter and shows severe mental and growth retardations suggesting a role of fucose in development.
Novel Developmental, Cellular and Biochemical Functions of Fucosylated Glycans in Mammals.
TLDR
A severe thymic atrophy phenotype is observed in FX(-/-) mice, in temporal association with the loss of fucosylated glycan structures on soluble and cell surface proteins, but restoration of fukosylation with a fucose-supplemented diet restores thymi development within 2 weeks.
Insights into leukocyte adhesion deficiency type 2 from a novel mutation in the GDP-fucose transporter gene.
TLDR
The robust response to fucose despite a severely truncated transporter suggests alternative means to transport GDP-fucose into the Golgi complex.
Reduced phagocytic activity of polymorphonuclear leukocytes in α(1,3) fucosyltransferase VII‐deficient mice
TLDR
A defect in the sialyl‐Lewis X antigen (selectin ligand) caused by disruption of the gene encodingα(1,3)fucosyltransferase VII leads to several morphological and functional abnormalities without an impact on survival rate.
The role of fucosylation in leukocyte adhesion deficiency II.
TLDR
Two leukocyte adhesion deficiencies (LAD) have been described in humans and any interference with these interactions should reduce the emigration of leukocytes to sites of infection, thus compromising immune responses.
Correction of leukocyte adhesion deficiency type II with oral fucose.
TLDR
A simple, noninvasive, and effective therapy for leukocyte adhesion deficiency type II (LAD II), a rare inherited disorder of fucose metabolism, which leads to an immunodeficiency caused by the absence of carbohydrate-based selectin ligands on the surface of neutrophils and severe psychomotor and mental retardation.
Conditional control of selectin ligand expression and global fucosylation events in mice with a targeted mutation at the FX locus
TLDR
Conditional control of fucosylation in FX(−/−) mice identifies cellular fucOSylation events as essential concomitants to fertility, early growth and development, and leukocyte adhesion.
A novel syndrome of variant leukocyte adhesion deficiency involving defects in adhesion mediated by beta1 and beta2 integrins.
TLDR
Clinical features and analysis of functions of cells from a patient with a myelodysplastic syndrome and infectious complications similar to those in the severe form of LAD-1, but whose circulating neutrophils displayed normal levels of beta2 integrins are reported.
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References

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TLDR
Neutrophil rolling, the first step in their adhesion, is markedly decreased, and therefore neutrophil emigration through the endothelium and arrival at site of inflammation is significantly diminished, providing evidence for a general fucose deficiency as the primary defect.
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TLDR
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TLDR
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TLDR
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TLDR
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TLDR
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TLDR
The identification of several human B cell lines that exhibit binding to E- selectin without expression of any of the previously identified E-selectin binding carbohydrate epitopes suggests that they may express a novel, sialylated carbohydrate structure(s) that binds to E -selectin.
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TLDR
The results support the concept of a bidirectional interaction between L-selectin bearing sLe(X) on neutrophils and activated EC in vivo, and suggest that L- Selectin may mediate rolling of lymphocytes that lack carbohydrate ligands for E- or P- selectin, although probably less efficiently than through bid Directional recognition.
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