MyBioSource.com's ADAMTS18 Antibody is a Rabbit Polyclonal antibody. This antibody has been shown to work in applications such as: ELISA, Immunocytochemistry, Immunofluorescence, and Western Blot.
Description
Function: Variant Leu-179 has been originally reported as disease-causing mutation in a patient with Knobloch syndrome (PubMed:21862674). It has been subsequently shown that Knobloch syndrome in the patient was due to an intronic mutation in COL18A1. Variant Leu-179 is, therefore, not responsible for the disease phenotype (PubMed:23667181).
Post-translational Modifications: The precursor is cleaved by a furin endopeptidase. Glycosylated. Can be O-fucosylated by POFUT2 on a serine or a threonine residue found within the consensus sequence C1-X2-(S/T)-C2-G of the TSP type-1 repeat domains where C1 and C2 are the first and second cysteine residue of the repeat, respectively. Fucosylated repeats can then be further glycosylated by the addition of a beta-1, 3-glucose residue by the glucosyltransferase, B3GALTL. Fucosylation mediates the efficient secretion of ADAMTS family members. Also can be C-glycosylated with one or two mannose molecules on tryptophan residues within the consensus sequence W-X-X-W of the TPRs, and N-glycosylated. These other glycosylations can also facilitate secretion (By similarity).
Similarity: The conserved cysteine present in the cysteine-switch motif binds the catalytic zinc ion, thus inhibiting the enzyme. The dissociation of the cysteine from the zinc ion upon the activation-peptide release activates the enzyme