FITC标记的过氧化物酶体生物合成因子5抗体
产品名称： FITC标记的过氧化物酶体生物合成因子5抗体
英文名称： Anti-PEX5/FITC
产品编号： HZ-12624R-FITC
产品价格： null
产品产地： 中国/上海
品牌商标： HZbscience
更新时间： 2023-08-17T10:24:20
使用范围： ICC=1:50-200 IF=1:50-200
上海沪震实业有限公司
- 联系人 : 鲍丽雯
- 地址 : 上海市闵行区闵北路88弄1-30号第22幢AQ136室
- 邮编 : 200612
- 所在区域 : 上海
- 电话 : 139****0749 点击查看
- 传真 : 点击查看
- 邮箱 : www.shzbio.net
- 二维码 : 点击查看
Rabbit Anti-PEX5/FITC Conjugated antibody
FITC标记的过氧化物酶体生物合成因子5抗体
| 英文名称 | Anti-PEX5/FITC |
| 中文名称 | FITC标记的过氧化物酶体生物合成因子5抗体 |
| 别 名 | FLJ50634; FLJ50721; FLJ51948; Peroxin 5; Peroxin-5; Peroxisomal biogenesis factor 5; Peroxisomal C terminal targeting signal import receptor; Peroxisomal C-terminal targeting signal import receptor; Peroxisomal targeting signal 1 receptor; Peroxisome receptor 1; pex5; PEX5_HUMAN; PTS1 BP; PTS1 receptor; PTS1-BP; PTS1R; PXR1. |
| 规格价格 | 100ul/2980元 购买 大包装/询价 |
| 说 明 书 | 100ul |
| 研究领域 | 细胞生物 神经生物学 信号转导 转录调节因子 |
| 抗体来源 | Rabbit |
| 克隆类型 | Polyclonal |
| 交叉反应 | Human, Mouse, Rat, Dog, Pig, Cow, Horse, 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 | 71kDa |
| 性 状 | Lyophilized or Liquid |
| 浓 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human PEX5 |
| 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 product of this gene binds to the C-terminal PTS1-type tripeptide peroxisomal targeting signal (SKL-type) and plays an essential role in peroxisomal protein import. Peroxins (PEXs) are proteins that are essential for the assembly of functional peroxisomes. The peroxisome biogenesis disorders (PBDs) are a group of genetically heterogeneous autosomal recessive, lethal diseases characterized by multiple defects in peroxisome function. The peroxisomal biogenesis disorders are a heterogeneous group with at least 14 complementation groups and with more than 1 phenotype being observed in cases falling into particular complementation groups. Although the clinical features of PBD patients vary, cells from all PBD patients exhibit a defect in the import of one or more classes of peroxisomal matrix proteins into the organelle. Defects in this gene are a cause of neonatal adrenoleukodystrophy (NALD), a cause of Zellweger syndrome (ZWS) as well as may be a cause of infantile Refsum disease (IRD). Alternatively spliced transcript variants encoding different isoforms have been identified. [provided by RefSeq, Oct 2008] Function: Binds to the C-terminal PTS1-type tripeptide peroxisomal targeting signal (SKL-type) and plays an essential role in peroxisomal protein import. Subcellular Location: Cytoplasm. Peroxisome membrane. Its distribution appears to be dynamic. It is probably a cycling receptor found mainly in the cytoplasm and as well associated to the peroxisomal membrane through a docking factor. Tissue Specificity: Detected in heart, brain, placenta, lung, liver, skeletal muscle, kidney and pancreas. DISEASE: Defects in PEX5 are a cause of adrenoleukodystrophy neonatal (NALD) [MIM:202370]. NALD is a peroxisome biogenesis disorder (PBD) characterized by the accumulation of very long-chain fatty acids, adrenal insufficiency and mental retardation. Inheritance is autosomal recessive. Defects in PEX5 are a cause of Zellweger syndrome (ZWS) [MIM:214100]. ZWS is a fatal peroxisome biogenesis disorder characterized by dysmorphic facial features, hepatomegaly, ocular abnormalities, renal cysts, hearing impairment, profound psychomotor retardation, severe hypotonia and neonatal seizures. Death occurs within the first year of life. Defects in PEX5 may be a cause of infantile Refsum disease (IRD) [MIM:266510]. IRD is a mild peroxisome biogenesis disorder (PBD). Clinical features include early onset, mental retardation, minor facial dysmorphism, retinopathy, sensorineural hearing deficit, hepatomegaly, osteoporosis, failure to thrive, and hypocholesterolemia. The biochemical abnormalities include accumulation of phytanic acid, very long chain fatty acids (VLCFA), di- and trihydroxycholestanoic acid and pipecolic acid. Similarity: Belongs to the peroxisomal targeting signal receptor family. Contains 7 TPR repeats. Database links: Entrez Gene: 418299 Chicken Entrez Gene: 514832 Cow Entrez Gene: 486710 Dog Entrez Gene: 100135597 Guinea pig Entrez Gene: 5830 Human Entrez Gene: 19305 Mouse Entrez Gene: 312703 Rat Omim: 600414 Human SwissProt: Q5ZMQ9 Chicken SwissProt: Q1RMV0 Cow SwissProt: O70525 Guinea pig SwissProt: P50542 Human SwissProt: O09012 Mouse SwissProt: Q2M2R8 Rat Unigene: 19020 Chicken Unigene: 10901 Cow Unigene: 567327 Human Unigene: 22418 Mouse Unigene: 14316 Rat Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications |
该基因产物与C端PTS1型三肽过氧化物酶体靶向信号（SKL型）结合，在过氧化物酶体蛋白导入中起重要作用。Peroxins（PXEs）是功能性过氧化物酶体组装必不可少的蛋白质。过氧化物酶体生物发生障碍（PBD）是一组遗传异质的常染色体隐性致死性疾病，以过氧化物酶体功能多重缺陷为特征。过氧化物酶体生物发生障碍是具有至少14个互补组的异质组，并且在属于特定互补组的病例中观察到超过1个表型。尽管PBD患者的临床特征各不相同，但是所有PBD患者的细胞在将一类或多类过氧化物酶体基质蛋白导入细胞器方面都表现出缺陷。该基因的缺陷是新生儿肾上腺脑白质营养不良（NALD）的原因，是Zellweger综合征（ZWS）的原因，也可能是婴儿反刍病（IRD）的原因。另一种编码不同的异构体的剪接转录变体已被鉴定。[ RefSeq，OCT 2008提供]