赖氨酸羟化酶2抗体
规格：1尘驳/1尘濒
英文名： PLOD2
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 oxoglutarat
分子量： 85kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：笔辞濒测肠濒辞苍补濒
亚型：滨驳骋
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human PLOD2
交叉反应：Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit, Sheep,
细胞定位：
赖氨酸羟化酶2抗体产物介绍：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 赖氨酸羟化酶2抗体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 赖氨酸羟化酶2抗体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. Gene ID: 5352 Database links: Entrez Gene: 5352 Human Entrez Gene: 26432 Mouse Entrez Gene: 300901 Rat Omim: 601865 Human SwissProt: O00469 Human SwissProt: Q9R0B9 Mouse SwissProt: Q811A3 Rat Unigene: 477866 Human Unigene: 79983 Mouse Unigene: 12945 Rat Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
赖氨酸羟化酶2抗体产物应用：WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
研究领域：肿瘤 细胞生物 信号转导
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid