FITC标记的硫辛酰连接酶2抗体
产品名称： FITC标记的硫辛酰连接酶2抗体
英文名称： Anti-LIPT2/FITC
产品编号： HZ-18297R-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-LIPT2/FITC Conjugated antibody
FITC标记的硫辛酰连接酶2抗体
| 英文名称 | Anti-LIPT2/FITC |
| 中文名称 | FITC标记的硫辛酰连接酶2抗体 |
| 别 名 | EC 2.3.1.181; FCT2; Lipoate-protein ligase B; Lipoyl(octanoyl) transferase 2 (putative); Lipoyl/octanoyl transferase; Lipt2; LIPT2_HUMAN; mitochondrial; Octanoyl-[acyl-carrier-protein]-protein N-octanoyltransferase; OTTHUMP00000230589; OTTHUMP00000230590; OTTHUMP00000230591; Putative lipoyltransferase 2; Putative lipoyltransferase 2, mitochondrial; Putative octanoyltransferase, mitochondrial; SLC22A16. |
| 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 免疫学 信号转导 新陈代谢 线粒体 |
| 抗体来源 | Rabbit |
| 克隆类型 | Polyclonal |
| 交叉反应 | Human, Mouse, Rat, Dog, 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 | 23kDa |
| 性 状 | Lyophilized or Liquid |
| 浓 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human LIPT2 |
| 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: LIPT2 is a 231 amino acid mitochondrial protein that belongs to the LipB family. LIPT2 catalyzes the exchange of octanoic acid from octanoyl-acyl-carrier-protein to lipoate-dependent enzymes. The gene encoding LIPT2 maps to human chromosome 11, which comprises approximately 4% of human genomic DNA and is considered a gene and disease association dense chromosome. The chromosome 11 encoded Atm gene is important for regulation of cell cycle arrest and apoptosis following double strand DNA breaks. Atm mutation leads to the disorder known as ataxia-telangiectasia. The blood disorders Sickle cell anemia and thalassemia are caused by HBB gene mutations, while Wilms' tumors, WAGR syndrome and Denys-Drash syndrome are associated with mutations of the WT1 gene. Jervell and Lange-Nielsen syndrome, Jacobsen syndrome, Niemann-Pick disease, hereditary angioedema and Smith-Lemli-Opitz syndrome are also associated with defects in chromosome 11-encoded genes. Function: Catalyzes the transfer of endogenously produced octanoic acid from octanoyl-acyl-carrier-protein onto the lipoyl domains of lipoate-dependent enzymes. Lipoyl-ACP can also act as a substrate although octanoyl-ACP is likely to be the physiological substrate. Subcellular Location: Mitochondrion. Similarity: Belongs to the lipB family. Database links: Entrez Gene: 387787 Human Entrez Gene: 67164 Mouse Entrez Gene: 365314 Rat SwissProt: A6NK58 Human SwissProt: Q9D009 Mouse Unigene: 591971 Human Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications |
LIPT2是属于LipB家族的231氨基酸线粒体蛋白。LIPT2催化辛酸从辛酰基酰基载体蛋白到脂依赖性酶的交换。编码LIPT2的基因定位于人类染色体11，该染色体包含大约4%的人类基因组DNA，被认为是基因和疾病关联的密集染色体。编码Atm基因的11号染色体对双链DNA断裂后细胞周期阻滞和凋亡的调控具有重要意义。ATM突变导致称为共济失调毛细血管扩张症。镰状细胞贫血和地中海贫血是由HBB基因突变引起的，而Wilms肿瘤、WAGR综合征和Denys-Drash综合征与WT1基因突变有关。Jervell和Lange-Nielsen综合征、Jac.n综合征、Niemann-Pick病、遗传性血管水肿和Smith-Lemli-Opitz综合征也与染色体11编码基因的缺陷有关。