富含亮氨酸重复蛋白50抗体
规格：1mg/1ml
英文名： LRRC50
别名： CILD13; DNAAF1; Dynein assembly factor 1, axonemal; Leucine rich repeat containing 50; ODA7; DAAF1_HUMAN.
分子量： 80kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：Polyclonal
亚型：IgG
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human LRRC50
交叉反应：Human, Mouse, Rat, Cow, Horse, Sheep, Guinea Pig, Cat, hu, mo, rat, shp, hrs, cow
细胞定位：细胞浆
富含亮氨酸重复蛋白50抗体产品介绍：background: The protein encoded by this gene is cilium-specific and is required for the stability of the ciliary architecture. It is involved in the regulation of microtubule-based cilia and actin-based brush border microvilli. Mutations in this gene are associated with primary ciliary dyskinesia-13.[provided by RefSeq, May 2010] Function: Cilium-specific protein required for the stability of the ciliary architecture. Plays a role in cytoplasmic preassembly of dynein arms. Involved in regulation of microtubule-based cilia and actin-based brush border microvilli. Subcellular Location: Cell projection; cilium. Cytoplasm. Cytoplasm; cytoskeleton, spindle pole. Note: In HEK293T cells, it is diffusely cytoplasmic and concentrates at the mitotic spindle poles, while in MDCK cells, it localizes in the cilium. In vivo, this protein is probably restricted to the cilium. Tissue Specificity: Mainly expressed in trachea and testis. DISEASE: Ciliary dyskinesia, primary, 13 (CILD13) [MIM:613193]: 富含亮氨酸重复蛋白50抗体A disorder characterized by abnormalities of motile cilia. Respiratory infections leading to chronic inflammation and bronchiectasis are recurrent, due to defects in the respiratory cilia; reduced fertility is often observed in male patients due to abnormalities of sperm tails. Half of the patients exhibit randomization of left-right body asymmetry and situs inversus, due to dysfunction of monocilia at the embryonic node. Primary 富含亮氨酸重复蛋白50抗体ciliary dyskinesia associated with situs inversus is referred to as Kartagener syndrome. At ultrastructural level, CILD13 is characterized by a marked reduction or absence of both dynein arms from the patients cilia. Similarity: Belongs to the DNAAF1 family. Contains 6 LRR (leucine-rich) repeats. Contains 1 LRRCT domain. Gene ID: 123872 Database links: Entrez Gene: 523187 Cow Entrez Gene: 123872 Human Entrez Gene: 68270 Mouse Entrez Gene: 361419 Rat Omim: 613190 Human SwissProt: Q3SYS4 Cow SwissProt: Q8NEP3 Human SwissProt: Q9D2H9 Mouse SwissProt: Q6AYH9 Rat Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
富含亮氨酸重复蛋白50抗体产品应用：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 信号转导 细胞骨架
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid