How Do O ‐Fucose Glycans Stabilize Thrombospondin Type 1 Repeats?

2020 ◽  
Vol 34 (S1) ◽  
pp. 1-1
Author(s):  
Steven J. Berardinelli ◽  
Rajashri Manjunath ◽  
Alexander Eletsky ◽  
Atsuko Ito ◽  
Robert S. Haltiwanger
Keyword(s):  
Thrombospondin Type 1 Repeats

Congenital ADAMTS-13 deficiency presenting as life-threatening thrombosis during pregnancy

2021 ◽  
Vol 14 (8) ◽  
pp. e239901
Author(s):  
Faheema Hasan ◽  
Anshul Gupta ◽  
Dinesh Chandra ◽  
Soniya Nityanand
Keyword(s):  
Late Presentation ◽  
Fresh Frozen Plasma ◽  
Artery Thrombosis ◽  
Fresh Frozen ◽  
Life Threatening ◽  
A Disintegrin And Metalloproteinase ◽  
Thrombospondin Type 1 Repeats ◽  
Frozen Plasma ◽  
Plasma Infusions

Thrombotic thrombocytopenic purpura (TTP) is a life-threatening disease characterised by thrombocytopenia, microangiopathic haemolytic anaemia and microvascular thrombosis. Congenital TTP accounting for less than 5% of all TTP cases can have a late presentation in adulthood mostly triggered by predisposing factors such as infection, pregnancy and inflammation. We present a case of a 23-year-old woman who presented to us in the postpartum period with mesenteric artery thrombosis with infarcts and later was diagnosed as a case of TTP based on congenital a disintegrin and metalloproteinase with thrombospondin type 1 repeats 13 (ADAMTS-13) deficiency detected on ADAMTS-13 levels and gene sequencing. She was successfully managed initially with therapeutic plasma exchanges and is now on prophylactic fortnightly fresh frozen plasma infusions at 15 mL/kg body weight and continues to be in remission.

O-Fucosylation of Thrombospondin Type 1 Repeats

2010 ◽  
pp. 401-416 ◽  
Author(s):  
Christina Leonhard-Melief ◽  
Robert S. Haltiwanger
Keyword(s):  
Thrombospondin Type 1 Repeats

scholarly journals O-fucosylation of thrombospondin type 1 repeats restricts epithelial to mesenchymal transition (EMT) and maintains epiblast pluripotency during mouse gastrulation

2010 ◽  
Vol 346 (1) ◽  
pp. 25-38 ◽  
Author(s):  
Jianguang Du ◽  
Hideyuki Takeuchi ◽  
Christina Leonhard-Melief ◽  
Kenneth R. Shroyer ◽  
Malgosia Dlugosz ◽  
...  
Keyword(s):  
Epithelial To Mesenchymal Transition ◽  
Mesenchymal Transition ◽  
Thrombospondin Type 1 Repeats

scholarly journals C-Mannosylation of Toxoplasma gondii proteins promotes attachment to host cells and parasite virulence

2019 ◽  
Vol 295 (4) ◽  
pp. 1066-1076 ◽  
Author(s):  
Andreia Albuquerque-Wendt ◽  
Damien Jacot ◽  
Nicolas Dos Santos Pacheco ◽  
Carla Seegers ◽  
Patricia Zarnovican ◽  
...  
Keyword(s):  
Toxoplasma Gondii ◽  
Protective Immunity ◽  
Host Cells ◽  
Apicomplexan Parasites ◽  
Tryptophan Residues ◽  
Parasite Survival ◽  
Parasite Motility ◽  
Parasite Egress ◽  
Thrombospondin Type 1 Repeats

C-Mannosylation is a common modification of thrombospondin type 1 repeats present in metazoans and recently identified also in apicomplexan parasites. This glycosylation is mediated by enzymes of the DPY19 family that transfer α-mannoses to tryptophan residues in the sequence WX2WX2C, which is part of the structurally essential tryptophan ladder. Here, deletion of the dpy19 gene in the parasite Toxoplasma gondii abolished C-mannosyltransferase activity and reduced levels of the micronemal protein MIC2. The loss of C-mannosyltransferase activity was associated with weakened parasite adhesion to host cells and with reduced parasite motility, host cell invasion, and parasite egress. Interestingly, the C-mannosyltransferase–deficient Δdpy19 parasites were strongly attenuated in virulence and induced protective immunity in mice. This parasite attenuation could not simply be explained by the decreased MIC2 level and strongly suggests that absence of C-mannosyltransferase activity leads to an insufficient level of additional proteins. In summary, our results indicate that T. gondii C-mannosyltransferase DPY19 is not essential for parasite survival, but is important for adhesion, motility, and virulence.

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