PLOD2 Rabbit pAb
- 产品详情
- 实验流程
Application
| IHC-P, IHC-F, IF, E |
|---|---|
| Primary Accession | O00469 |
| Other Accession | O00469 |
| Host | Rabbit |
| Clonality | Polyclonal |
| Calculated MW | 84686 Da |
| Physical State | Liquid |
| Immunogen | KLH conjugated synthetic peptide derived from human PLOD2 |
| Epitope Specificity | 501-600/737 |
| Isotype | IgG |
| Purity | affinity purified by Protein A |
| Buffer | 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| SIMILARITY | Contains 1 Fe2OG dioxygenase domain. |
| DISEASE | Defects in PLOD2 are the cause of Bruck syndrome type 2 (BRKS2) [MIM:609220]. 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. |
| Important Note | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| Background Descriptions | 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] |
| Gene ID | 5352 |
|---|---|
| Other Names | BRKS2; LH2; TLH; D530025C14Rik; Plod-2; PLOD2_HUMAN; PLOD2; Lysyl hydroxylase 2 (LH2); 1.14.11.4; PLOD2_MOUSE; PLOD2_RAT; procollagen-lysine, 2-oxoglutarate 5-dioxygenase 2; procollagen-lysine, 2-oxoglutarate 5-dioxygenase (lysine hydroxylase) 2; procollagen-lysine, 2-oxoglutarate 5-dioxygenase 2; lysyl hydroxlase 2; telopeptidyl lysyl hydroxylase; procollagen lysyl hydroxylase 2 |
| Dilution | IHC-P=1:100-500,IHC-F=1:100-500,ICC/IF=1:100-500,IF=1:100-500,ELISA=1:5000-10000 |
| Storage | Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C. |
For Research Use Only. Not For Use In Diagnostic Procedures.
| Name | PLOD2 (HGNC:9082) |
|---|---|
| Function | 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. |
| Cellular Location | Rough endoplasmic reticulum membrane; Peripheral membrane protein; Lumenal side. Cytoplasm |
| Tissue Location | Highly expressed in pancreas and muscle. Isoform 1 and isoform 2 are expressed in the majority of the examined cell types Isoform 2 is specifically expressed in skin, lung, dura and aorta |
Research Areas
Application Protocols
Provided below are standard protocols that you may find useful for product applications.
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