C-Mannosylation is a post-translational modification of proteins in the endoplasmic
reticulum. Monomeric α-mannose is attached to specific Trp residues at the first Trp in the …

Glycosylation is perhaps the most common post-translational modification. Recently there
has been growing interest in cataloging the glycan structures, glycoproteins, and specific …

Platelet recruitment to sites of blood vessel damage is highly dependent upon von
Willebrand factor (VWF). VWF platelet-tethering function is proteolytically regulated by the …

Previous studies demonstrated importance of C-mannosylation for efficient protein secretion.
To study its impact on protein folding and stability, we analyzed both C-mannosylated and …

Recombinant ADAMTS13 is currently undergoing clinical trials as a treatment for hereditary
thrombotic thrombocytopenic purpura, a lethal microvascular condition resulting from …

Immune thrombotic thrombocytopenic purpura (iTTP) is an autoimmune disorder of which
the etiology is not fully understood. Autoantibodies targeting ADAMTS13 in iTTP patients …

Mass spectrometry (MS) easily detects C-mannosylated peptides from purified proteins but
not from complex biological samples. Enrichment of specific glycopeptides by lectin affinity …

Introduction Severe ADAMTS13 deficiency defines thrombotic thrombocytopenic purpura
(TTP). ADAMTS13 is responsible for VWF cleavage. In the absence of this enzyme …

Synonymous variants within coding regions may influence protein expression and function.
We have previously reported increased protein expression levels ex vivo (~ 120% in …

A disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13
(ADAMTS13) is a multidomain metalloprotease for which until now only a single substrate …