| 产品编号 | bs-8719R-Gold |
| 英文名称 | Anti-LRP130/LRPPRC/Gold |
| 中文名称 | 胶体金标记的富含亮氨酸蛋白LRP130抗体 |
| 别 名 | LRPPRC; 130 kDa leucine-rich protein; GP130; Leucine-rich PPR-motif containing protein; LRP 130; LPPRC_HUMAN. |
| 规格价格 | 100ul/2980元 购买 大包装/询价 |
| 说 明 书 | 100ul（10nm 15nm 35nm） |
| 研究领域 | 细胞生物 免疫学 神经生物学 信号转导 |
| 抗体来源 | Rabbit |
| 克隆类型 | Polyclonal |
| 交叉反应 | Rat, (predicted: Human, Mouse, Chicken, Horse, Rabbit, ) |
| 产品应用 | IEM=1:20-200 ICA=1:20-200 ChIP=1:20-200
not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 分 子 量 | 152kDa |
| 性 状 | Lyophilized or Liquid |
| 浓 度 | 0.4mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human LRP130 |
| 亚 型 | IgG |
| 纯化方法 | affinity purified by Protein A |
| 储 存 液 | 0.02M TBS(pH8.2) with 1% BSA, 0.03% Proclin300. |
| 保存条件 | Store at 2-8 ºC for 3-6 months. Avoid repeated freeze/thaw cycles. |
| 产品介绍 |
background: Leucine-rich protein (LRP130) is a cytoplasmic mRNA-binding protein likely to be involved in the processing of mitochondrial DNA transcripts. Defects in the LRPPRC gene that encodes LRP130 result in the French-Canadian type of Leigh syndrome, a severe neurological disorder characterized by lesions in the subcortical region of the brain. LRP130 also interacts with the low-affinity receptor for leukemia inhibitory factor to produce an intracelluar signal cascade. Function: LPPRC is thought to play a role in RNA metabolism in both nuclei and mitochondria. In the nucleus, LPPRC binds to HNRPA1-associated poly(A) mRNAs and is part of nmRNP complexes at late stages of mRNA maturation, which are possibly associated with nuclear mRNA export. In mitochondria, LPPRC binds to poly(A) mRNA. LPPRC may play a role in translation or stability of mitochondrially encoded cytochrome c oxidase (COX) subunits. Defects in LRPPRC are the cause of Leigh syndrome French-Canadian type (LSFC). Leigh syndrome is a severe neurological disorder characterized by bilaterally symmetrical necrotic lesions in subcortical brain regions that is commonly associated with systemic cytochrome c oxidase (COX) deficiency. In the Saguenay-Lac Saint Jean region of Quebec province in Canada, a biochemically distinct form of Leigh syndrome with COX deficiency has been described. Patients have been observed to have a developmental delay, hypotonia, mild facial dysmorphism, chronic well-compensated metabolic acidosis, and high mortality due to episodes of severe acidosis and coma. Enzyme activity was close to normal in kidney and heart, 50% of normal in fibroblasts and skeletal muscle, and nearly absent in brain and liver. Subunit: Interacts with INPP5D/SHIP1 (By similarity). Forms heterodimers composed of LIPR and IL6ST (type I OSM receptor). Also forms heterodimers composed of OSMR and IL6ST (type II OSM receptor). Homodimer. The homodimer binds two molecules of herpes virus IL6. Component of a hexamer of two molecules each of IL6, IL6R and IL6ST. Interacts with HCK. Subcellular Location: Mitochondrion. Nucleus; nucleoplasm. Nucleus inner membrane. Nucleus outer membrane. Note: Seems to be predominantly mitochondrial. Tissue Specificity: Found in all the tissues and cell lines examined. Expression not restricted to IL6 responsive cells. DISEASE: Defects in LRPPRC are the cause of Leigh syndrome French-Canadian type (LSFC) [MIM:220111]. Leigh syndrome is a severe neurological disorder characterized by bilaterally symmetrical necrotic lesions in subcortical brain regions that is commonly associated with systemic cytochrome c oxidase (COX) deficiency. In the Saguenay-Lac Saint Jean region of Quebec province in Canada, a biochemically distinct form of Leigh syndrome with COX deficiency has been described. Patients have been observed to have a developmental delay, hypotonia, mild facial dysmorphism, chronic well-compensated metabolic acidosis, and high mortality due to episodes of severe acidosis and coma. Enzyme activity was close to normal in kidney and heart, 50% of normal in fibroblasts and skeletal muscle, and nearly absent in brain and liver. LSFC patients show reduced (<30%) levels of LRPPRC in both fibroblast and liver mitochondria and a specifically reduced translation of COX subunits MT-CO1/COXI and MT-CO3 (COXIII). Similarity: Contains 20 PPR (pentatricopeptide) repeats. Database links: Entrez Gene: 10128 Human Omim: 607544 Human SwissProt: P42704 Human Unigene: 368084 Human Unigene: 92051 Rat Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| 1、抗体溶解方法 | |
| 2、抗体修复方式 | |
| 3、常用试剂的配制 | |
| 4、免疫组化操作步骤 | |
| 5、免疫组化问题解答 | |
| 6、Western Blotting 操作步骤 | |
| 7、Western Blotting 问题解答 | |
| 8、关于肽链的设计 | |
| 9、多肽的溶解与保存 | |
| 10、酶标抗体效价测定程序 | |
