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货号： bs-12731R-AF350 基本售价： 2980.0 元 规格： 100ul
产品信息
- 产品编号
- bs-12731R-AF350
- 英文名称
- Anti-PLOD2/AF350
- 中文名称
- AF350标记的赖氨酸羟化酶2抗体
- 别 名
- 2-oxoglutarate 5-dioxygenase 2; LH2; Lysine hydroxylase 2; Lysyl hydroxylase 2; OTTHUMP00000215204; OTTHUMP00000215205; OTTHUMP00000215206; PLOD 2; Plod2; PLOD2_HUMAN; Procollagen lysine 2 oxoglutarate 5 dioxygenase 2; Procollagen lysine, 2 oxoglutarate 5 dioxygenase (lysine hydroxylase) 2; Procollagen-lysine; Telopeptide lysyl hydroxylase; TLH.
- 规格价格
- 100ul/2980元购买 大包装/询价
- 说 明 书
- 100ul
- 研究领域
- 肿瘤 细胞生物 信号转导
- 抗体来源
- Rabbit
- 克隆类型
- Polyclonal
- 交叉反应
- Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit, Sheep,
- 产品应用
- ICC=1:50-200 IF=1:50-200
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
- 分 子 量
- 85kDa
- 性 状
- Lyophilized or Liquid
- 浓 度
- 1mg/ml
- 免 疫 原
- KLH conjugated synthetic peptide derived from human PLOD2
- 亚 型
- IgG
- 纯化方法
- affinity purified by Protein A
- 储 存 液
- 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol
- 保存条件
- Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. 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.
- 产品介绍
- background:
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]
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.
Subcellular Location:
Rough endoplasmic reticulum membrane.
Tissue Specificity:
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.
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.
Similarity:
Contains 1 Fe2OG dioxygenase domain.
Database links:Entrez Gene: 5352Human
Entrez Gene: 26432Mouse
Entrez Gene: 300901Rat
Omim: 601865Human
SwissProt: O00469Human
SwissProt: Q9R0B9Mouse
SwissProt: Q811A3Rat
Unigene: 477866Human
Unigene: 79983Mouse
Unigene: 12945Rat
Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.