FITC标记的过氧化物酶体生物合成因子16抗体
产品名称： FITC标记的过氧化物酶体生物合成因子16抗体
英文名称： Anti-PEX16/FITC
产品编号： HZ-12622R-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-PEX16/FITC Conjugated antibody
FITC标记的过氧化物酶体生物合成因子16抗体
| 英文名称 | Anti-PEX16/FITC |
| 中文名称 | FITC标记的过氧化物酶体生物合成因子16抗体 |
| 别 名 | Peroxin 16; PBD8A; PBD8B; Peroxin16; Peroxisomal biogenesis factor 16; Peroxisomal membrane protein PEX16; PEX 16. |
| 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, Dog, Pig, Cow, Horse, Rabbit, 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 | 39kDa |
| 性 状 | Lyophilized or Liquid |
| 浓 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human PEX16 |
| 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 protein encoded by this gene is an integral peroxisomal membrane protein. An inactivating nonsense mutation localized to this gene was observed in a patient with Zellweger syndrome of the complementation group CGD/CG9. Expression of this gene product morphologically and biochemically restores the formation of new peroxisomes, suggesting a role in peroxisome organization and biogenesis. Alternative splicing has been observed for this gene and two variants have been described. [provided by RefSeq, Jul 2008] Function: Required for peroxisome membrane biogenesis. May play a role in early stages of peroxisome assembly. Can recruit other peroxisomal proteins, such as PEX3 and PMP34, to de novo peroxisomes derived from the endoplasmic reticulum (ER). May function as receptor for PEX3. Subcellular Location: Peroxisome membrane; Multi-pass membrane protein. Endoplasmic reticulum membrane. 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). Similarity: Belongs to the peroxin-16 family. Database links: Entrez Gene: 9409 Human Omim: 603360 Human SwissProt: Q9Y5Y5 Human Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications |
由该基因编码的蛋白质是一个完整的过氧化物酶体膜蛋白。在互补组CGD/CG9的Zellweger综合征患者中观察到一个定位于该基因的失活性无义突变。该基因产物在形态学和生化上的表达恢复了新过氧化物酶体的形成，表明其在过氧化物酶体的组织和生物发生中起作用。已经观察到该基因的选择性剪接，并且已经描述了两种变体。[ RefSeq，JUL 2008 ]