Uniprot Description
PLOD2: Forms hydroxylysine residues in -Xaa-Lys-Gly- sequences in collagens. These hydroxylysines serve as sites of attachment for carbohydrate units and are essential for the stability of the intermolecular collagen cross-links. Defects in PLOD2 are the cause of Bruck syndrome type 2 (BRKS2). Bruck syndrome, also known as osteogenesis imperfecta with congenital joint contractures, is an autosomal recessive disease characterized by generalized osteopenia, joint contractures at birth, fragile bones and short stature. It can be distinguished from osteogenesis imperfecta by the absence of hearing loss and dentinogenesis imperfecta, and by the presence of clubfoot and congenital joint limitations. The molecular defect is an aberrant cross-linking of bone collagen, due to underhydroxylation of lysine residues within the telopeptides of type I collagen, whereas the lysine residues in the triple helix are normal. 2 isoforms of the human protein are produced by alternative splicing.
Protein type: EC 1.14.11.4; Endoplasmic reticulum; Amino Acid Metabolism - lysine degradation; Oxidoreductase
Cellular Component: membrane; endoplasmic reticulum
Molecular Function: oxidoreductase activity, acting on paired donors, with incorporation or reduction of molecular oxygen; dioxygenase activity; L-ascorbic acid binding; metal ion binding; procollagen-lysine 5-dioxygenase activity; iron ion binding; oxidoreductase activity, acting on paired donors, with incorporation or reduction of molecular oxygen, 2-oxoglutarate as one donor, and incorporation of one atom each of oxygen into both donors; oxidoreductase activity
Product Notes
The PLOD2 plod2 (Catalog #
AAA8207573) is a siRNA produced from Synthetic and is intended for research purposes only. The product is available for immediate purchase. The PLOD2 siRNA (Mouse) reacts with Mouse and may cross-react with other species as described in the data sheet. AAA Biotech's PLOD2 can be used in a range of immunoassay formats including, but not limited to, RNA Interference (RNAi). Researchers should empirically determine the suitability of the PLOD2 plod2 for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process.
It is sometimes possible for the material contained within the vial of
"PLOD2, siRNA" to become dispersed throughout the inside of
the vial, particularly around the seal of said vial, during shipment and storage. We always
suggest centrifuging these vials
to consolidate all of the liquid away from the lid and to the bottom of the vial prior to
opening. Please be advised that
certain products may require dry ice for shipping and that, if this is the case, an
additional dry ice fee may also be
required.
Precautions
All products in the AAA Biotech catalog are strictly for research-use only, and are
absolutely not suitable for use in any
sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a
product from AAA Biotech, you
are explicitly certifying that said products will be properly tested and used in line with
industry standard. AAA Biotech
and its authorized distribution partners reserve the right to refuse to fulfill any order if
we have any indication that a
purchaser may be intending to use a product outside of our accepted criteria.
Disclaimer
Though we do strive to guarantee the information represented in this datasheet, AAA Biotech
cannot be held
responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any
aspect of this datasheet at
any time and without notice. It is the responsibility of the customer to inform AAA Biotech
of any product performance
issues observed or experienced within 30 days of receipt of said product. To see additional
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