Anti-PEX19/FITC Conjugated抗体
产品名称： Anti-PEX19/FITC Conjugated抗体
英文名称： Anti-PEX19/FITC
产品编号： YB--12623R-FITC
产品价格： null
产品产地： 中国/美国
品牌商标： Ybscience
更新时间： 2023-08-17T10:29:50
使用范围： 科研使用
上海钰博生物科技有限公司
- 联系人 : 陈环环
- 地址 : 上海市沪闵路6088号龙之梦大厦8楼806室
- 邮编 : 200612
- 所在区域 : 上海
- 电话 : 183****2235
- 传真 : 021-60514606
- 邮箱 : shybio@126.com
Anti-PEX19/FITC Conjugated抗体
| 产品编号 | YB-12623R-FITC |
| 英文名称 | Anti-PEX19/FITC |
| 中文名称 | FITC标记的过氧化物酶体生物合成因子19抗体 |
| 别 名 | 33 kDa housekeeping protein; D1S2223E; HK33; Housekeeping gene 33kD; OK/SW-cl.22; PBD12A; Peroxin 19; Peroxin-19; Peroxisomal biogenesis factor 19; Peroxisomal farnesylated protein; PEX19; PEX19_HUMAN; PMP1; PMPI; PXF; PXMP1. |
| 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, |
| 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 | 33kDa |
| 性 状 | Lyophilized or Liquid |
| 浓 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human PEX19 |
| 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 is necessary for early peroxisomal biogenesis. It acts both as a cytosolic chaperone and as an import receptor for peroxisomal membrane proteins (PMPs). 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. These disorders have at least 14 complementation groups, with more than one phenotype being observed for some 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 Zellweger syndrome (ZWS), as well as peroxisome biogenesis disorder complementation group 14 (PBD-CG14), which is also known as PBD-CGJ. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Aug 2010] Function: Necessary for early peroxisomal biogenesis. Acts both as a cytosolic chaperone and as an import receptor for peroxisomal membrane proteins (PMPs). Binds and stabilizes newly synthesized PMPs in the cytoplasm by interacting with their hydrophobic membrane-spanning domains, and targets them to the peroxisome membrane by binding to the integral membrane protein PEX3. Excludes CDKN2A from the nucleus and prevents its interaction with MDM2, which results in active degradation of TP53. Subcellular Location: Cytoplasm. Peroxisome membrane. Mainly cytoplasmic. Some fraction membrane-associated to the outer surface of peroxisomes. Tissue Specificity: Ubiquitously expressed. Isoform 1 is strongly predominant in all tissues except in utero where isoform 2 is the main form. DISEASE: Defects in PEX19 are the cause of peroxisome biogenesis disorder complementation group 14 (PBD-CG14) [MIM:600279]; also known as PBD-CGJ. PBD refers to a group of peroxisomal disorders arising from a failure of protein import into the peroxisomal membrane or matrix. The PBD group is comprised of four disorders: 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. The PBD group is genetically heterogeneous with at least 14 distinct genetic groups as concluded from complementation studies. Defects in PEX19 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. Similarity: Belongs to the peroxin-19 family. Database links: Entrez Gene: 5824 Human Entrez Gene: 19298 Mouse Entrez Gene: 289233 Rat Omim: 600279 Human SwissProt: P40855 Human SwissProt: Q8VCI5 Mouse SwissProt: Q9QYU1 Rat Unigene: 517232 Human Unigene: 247764 Mouse Unigene: 203104 Rat Unigene: 225447 Rat Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications |