赖氨酸羟化酶3抗体
规格：1尘驳/1尘濒
英文名： PLOD3
别名： 2-oxoglutarate 5-dioxygenase 3; bone protein I; BP-I; LH3; Lysine hydroxylase 3; Lysyl hydroxylase 3; Plod3; PLOD3_HUMAN; Procollagen lysine,2 oxoglutarate 5 dioxygenase 3 precursor; Procollagen-lysin
分子量： 82kDa
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 PLOD3
交叉反应：Human, Mouse, Rat, Dog, Pig, Cow, Horse, Rabbit, Sheep,
细胞定位：细胞膜
赖氨酸羟化酶3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. [provided by RefSeq, Jul 2008] Function: Forms hydroxylysine residues in -Xaa-Lys-Gly- sequences in collagens. These hydroxylysines serve as sites 赖氨酸羟化酶3抗体of attachment for carbohydrate units and are essential for the stability of the intermolecular collagen cross-links. Subcellular Location: Rough endoplasmic reticulum membrane. DISEASE: Defects in PLOD3 are the cause of lysyl hydroxylase 3 deficiency (LH3 deficiency) [MIM:612394]; also known as bone fragility with contractures arterial rupture and deafness. LH3 deficiency is a connective tissue disorder. The syndrome is characterized by congenital malformations severely affecting many tissues and organs and revealing features of several collagen disorders, most of them involving COL2A1 (type II collagen). The findings suggest that the failure of lysyl hydroxylation and hydroxylysyl carbohydrate addition,赖氨酸羟化酶3抗体 which affects many collagens, is the molecular basis of this syndrome. Similarity: Contains 1 Fe2OG dioxygenase domain. Gene ID: 8985 Database links: Entrez Gene: 8985 Human Entrez Gene: 26433 Mouse Entrez Gene: 288583 Rat Omim: 603066 Human SwissProt: O60568 Human SwissProt: Q9R0E1 Mouse SwissProt: Q5U367 Rat Unigene: 153357 Human Unigene: 251003 Mouse Unigene: 90152 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3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：细胞生物 **学 细胞膜蛋白
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid