PLOD2, Polyclonal Antibody

Anti -PLOD2 (Procollagen-lysine,2-oxoglutarate 5-dioxygenase 2, Lysyl Hydroxylase 2, LH2)

For Research Use Only. Not for use in diagnostic procedures.

Chromosome: 3; NC_000003.11 (145787225..145879282, complement). Location: 3q24

Polyclonal

IgG

Mouse

Recognizes human PLOD2.

Affinity Purified
Purified by Protein A affinity chromatography.

Supplied as a liquid in PBS, pH 7.2.

May be stored at 4 degree C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20 degree C. Aliquots are stable for at least 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

Small volumes of anti-PLOD2 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

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.

Antibodies; Abs to Enzymes

Western Blot (WB), Immunofluorescence (IF)

Suitable for use in Immunofluorescence and Western Blot.
Dilution: Immunofluorescence: 10ug/ml

84,686 Da[Similar Products]

procollagen-lysine, 2-oxoglutarate 5-dioxygenase 2

procollagen-lysine,2-oxoglutarate 5-dioxygenase 2; lysyl hydroxlase 2; lysyl hydroxylase 2; lysine hydroxylase 2; telopeptide lysyl hydroxylase

Procollagen-lysine,2-oxoglutarate 5-dioxygenase 2

LH2  [Similar Products]

PLOD2_HUMAN

The protein encoded by this gene is a membrane-bound homodimeric enzyme that is localized to the cisternae of the rough endoplasmic reticulum. The enzyme (cofactors iron and ascorbate) catalyzes the hydroxylation of lysyl residues in collagen-like peptides. The resultant hydroxylysyl groups are attachment sites for carbohydrates in collagen and thus are critical for the stability of intermolecular crosslinks. Some patients with Ehlers-Danlos syndrome type VIB have deficiencies in lysyl hydroxylase activity. Mutations in the coding region of this gene are associated with Bruck syndrome. Alternative splicing results in multiple transcript variants encoding different isoforms. [provided by RefSeq, Jul 2008]

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: Oxidoreductase; EC 1.14.11.4; Amino Acid Metabolism - lysine degradation; Endoplasmic reticulum

Chromosomal Location of Human Ortholog: 3q24

Cellular Component: endoplasmic reticulum membrane; endoplasmic reticulum; rough endoplasmic reticulum membrane

Molecular Function: L-ascorbic acid binding; procollagen-lysine 5-dioxygenase activity; iron ion binding

Biological Process: extracellular matrix organization and biogenesis; response to hypoxia; protein modification process

Disease: Bruck Syndrome 2

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