FITC标记的过氧化物酶体生物合成因子6抗体
产品名称： FITC标记的过氧化物酶体生物合成因子6抗体
英文名称： Anti-PEX6/FITC
产品编号： HZ-12625R-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
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Rabbit Anti-PEX6/FITC Conjugated antibody
FITC标记的过氧化物酶体生物合成因子6抗体
| 英文名称 | Anti-PEX6/FITC |
| 中文名称 | FITC标记的过氧化物酶体生物合成因子6抗体 |
| 别 名 | PAF 2; PAF2; PBD4A; PDB4B; Peroxin 6; Peroxisomal AAA type ATPase 1; Peroxisomal biogenesis factor 6; Peroxisomal type ATPase 1; Peroxisome assembly factor 2; PXAAA1. |
| 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 信号转导 |
| 抗体来源 | Rabbit |
| 克隆类型 | Polyclonal |
| 交叉反应 | Human, Mouse, Rat, Pig, Horse, Rabbit, |
| 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 | 104kDa |
| 性 状 | Lyophilized or Liquid |
| 浓 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human PEX6 |
| 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: This gene encodes a member of the AAA (ATPases associated with diverse cellular activities) family of ATPases. This member is a predominantly cytoplasmic protein, which plays a direct role in peroxisomal protein import and is required for PTS1 (peroxisomal targeting signal 1, a C-terminal tripeptide of the sequence ser-lys-leu) receptor activity. Mutations in this gene cause peroxisome biogenesis disorders of complementation group 4 and complementation group 6. [provided by RefSeq, Oct 2008] Function: Involved in peroxisome biosynthesis. Required for stability of the PTS1 receptor. Anchored by PEX26 to peroxisome membranes, possibly to form heteromeric AAA ATPase complexes required for the import of proteins into peroxisomes. Subcellular Location: Cytoplasm. Peroxisome membrane. Note: Associated with peroxisomal membranes. DISEASE: The disease is caused by mutations affecting the gene represented in this entry. Disease description:A peroxisomal disorder arising from a failure of protein import into the peroxisomal membrane or matrix. The peroxisome biogenesis disorders (PBD group) are genetically heterogeneous with at least 14 distinct genetic groups as concluded from complementation studies. Include disorders are: Zellweger syndrome (ZWS), neonatal adrenoleukodystrophy (NALD), infantile Refsum disease (IRD), and classical rhizomelic chondrodysplasia punctata (RCDP). ZWS, NALD and IRD are distinct from RCDP and constitute a clinical continuum of overlapping phenotypes known as the Zellweger spectrum (PBD-ZSS). Database links: Entrez Gene: 5190 Human Entrez Gene: 224824 Mouse Entrez Gene: 117265 Rat Omim: 601498 Human SwissProt: Q13608 Human SwissProt: Q99LC9 Mouse SwissProt: P54777 Rat Unigene: 656425 Human Unigene: 299399 Mouse Unigene: 10675 Rat Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications |
该基因编码AAA家族成员（ATPase与多种细胞活性相关）家族的ATPase。该成员主要是细胞质蛋白，它在过氧化物酶体蛋白导入中起直接作用，并且是PTS1(过氧化物酶体靶向信号1，序列ser-lys-leu的C端三肽)受体活性所必需的。该基因的突变导致互补组4和互补组6的过氧化物酶体生物发生障碍。[ RefSeq，OCT 2008提供]