PLOD2, Polyclonal Antibody

Procollagen-lysine,2-oxoglutarate 5-dioxygenase 2; Lysyl hydroxylase 2; LH2; PLOD2

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

Polyclonal

Rabbit Ig

Rabbit

This PLOD2 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 483-512 amino acids from the C-terminal region of human PLOD2.

Peptide Affinity Purified Rabbit Polyclonal Antibody (Pab)

Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is purified through a protein A column, followed by peptide affinity purification.

Vial Concentration: 0.5 (lot specific)

Maintain refrigerated at 2-8 degree C for up to 6 months. For long term storage store at -20 degree C in small aliquots to prevent freeze-thaw cycles.

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.

Cancer; Metabolism; Signal Transduction

Western Blot (WB), ELISA (EIA)

WB~~1:1000

PLOD2 Antibody (C-term) western blot analysis in Hela cell line lysates (35ug/lane).This demonstrates the PLOD2 antibody detected the PLOD2 protein (arrow).

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procollagen-lysine, 2-oxoglutarate 5-dioxygenase 2

procollagen-lysine,2-oxoglutarate 5-dioxygenase 2

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

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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