FITC标记的13号染色体开放阅读框12抗体
产品名称： FITC标记的13号染色体开放阅读框12抗体
英文名称： Anti-HSPC014/C13orf12/FITC
产品编号： HZ-18086R-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-HSPC014/C13orf12/FITC Conjugated antibody
FITC标记的13号染色体开放阅读框12抗体
| 英文名称 | Anti-HSPC014/C13orf12/FITC |
| 中文名称 | FITC标记的13号染色体开放阅读框12抗体 |
| 别 名 | 2510048O06Rik; C13orf12; Chromosome 13 open reading frame 12; HSPC 014; HSPC036 protein; hUMP 1; hUMP1; PNAS 110; PNAS110; Pomp; POMP_HUMAN; Proteasome maturation protein; Proteassemblin; Protein UMP1 homolog; UMP 1; UMP1; UMP1, yeast, homolog of; Voltage gated K channel beta subunit 4.1; Voltage-gated K channel beta subunit 4.1; voltage-gated potassium channel beta subunit 4.1. |
| 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 |
| 抗体来源 | Rabbit |
| 克隆类型 | Polyclonal |
| 交叉反应 | Human, Mouse, |
| 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 | 16kDa |
| 性 状 | Lyophilized or Liquid |
| 浓 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human HSPC014/C13orf12 |
| 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 a molecular chaperone that binds 20S preproteasome components and is essential for 20S proteasome formation. The 20S proteasome is the proteolytically active component of the 26S proteasome complex. The encoded protein is degraded before the maturation of the 20S proteasome is complete. A variant in the 5' UTR of this gene has been associated with KLICK syndrome, a rare skin disorder.[provided by RefSeq, Aug 2010] Function: Molecular chaperone essential for the assembly of standard proteasomes and immunoproteasomes. Degraded after completion of proteasome maturation. Mediates the association of 20S preproteasome with the endoplasmic reticulum. Subcellular Location: Cytoplasm > cytosol. Nucleus. Microsome membrane. Tissue Specificity: Strongly expressed from the basal layer to the granular layer of healthy epidermis, whereas in KLICK patients there is a gradual decrease of expression toward the granular layer. DISEASE: Defects in POMP are the cause of keratosis linearis with ichthyosis congenita and sclerosing keratoderma (KLICK) [MIM:601952]. KLICK is a keratinizing disorder characterized by ichthyosis, palmoplantar keratoderma with constricting bands around fingers, flexural deformities of fingers and keratotic papules in a linear distribution on the flexural side of large joints. Histological examination of the skin of affected individuals shows hypertrophy and hyperplasia of the spinous, granular and horny epidermal layer. Similarity: Belongs to the POMP/UMP1 family. Database links: Entrez Gene: 51371 Human Entrez Gene: 66537 Mouse Entrez Gene: 288455 Rat Omim: 613386 Human SwissProt: Q9Y244 Human SwissProt: Q9CQT5 Mouse Unigene: 268742 Human Unigene: 332855 Mouse Unigene: 28242 Rat Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
该基因编码的蛋白质是结合20S蛋白酶体前组分的分子伴侣，对20S蛋白酶体的形成是必不可少的。20S蛋白酶体是26S蛋白酶体复合体的蛋白水解活性成分。编码的蛋白质在20S蛋白酶体成熟完成之前被降解。这个基因的5’UTR中的一个变体与KLICK综合征相关，这是一种罕见的皮肤疾病。[由RefSeq，Aug 2010提供]