FITC标记的胱氨酸抗体
产品名称： FITC标记的胱氨酸抗体
英文名称： Anti-CTNS/FITC
产品编号： HZ-12932R-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-CTNS/FITC Conjugated antibody
FITC标记的胱氨酸抗体
| 英文名称 | Anti-CTNS/FITC |
| 中文名称 | FITC标记的胱氨酸抗体 |
| 别 名 | CTNS LSB; Cystinosin; cystinosis, nephropathic; PQLC4; CTNS_HUMAN. |
| 规格价格 | 100ul/2980元 购买 大包装/询价 |
| 说 明 书 | 100ul |
| 研究领域 | 肿瘤 细胞生物 信号转导 细胞类型标志物 新陈代谢 跨膜蛋白 |
| 抗体来源 | Rabbit |
| 克隆类型 | Polyclonal |
| 交叉反应 | Human, Mouse, Rat, 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 | 42kDa |
| 细胞定位 | 细胞膜 |
| 性 状 | Lyophilized or Liquid |
| 浓 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human CTNS/Cystinosin |
| 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: Cystinosis is an autosomal recessive disorder resulting from defective lysosomal transport of cystine and present at birth as a failure to thrive, rickets and proximal renal tubular acidosis. The human CTNS gene on chromosome 17p13 encodes the protein Cystinosin, and mutations in CTNS are responsible for nephropathic cystinosis. The CTNS promoter contains an Sp1 binding element. Cystinosin is an integral membrane protein containing 7 transmembrane domains that functions as a H+-driven transporter responsible for cystine export from lysosomes. In humans, Cystinosin is expressed abundantly in pancreas, kidney (mature and fetal), and skeletal muscle. The mouse homolog to CTNS encodes a protein which is expressed in all tissues except skeletal muscle. In the cell, Cystinosin co-localizes with LAMP-2 to lysosomes. A C-terminal GYDQL sorting motif within Cystinosin is critical for lysosomal localization. Function: CTNS (Cystinosin) is thought to transport cystine out of lysosomes. Mutations in the CTNS gene are the cause of cystinosis. Subcellular Location: Lysosome membrane; Multi-pass membrane protein. Tissue Specificity: Strongly expressed in pancreas, kidney (adult and fetal) and in skeletal muscle. Expressed at lower levels in placenta and heart. Weakly expressed in lung, liver and brain (adult and fetal). DISEASE: Defects in CTNS are the cause of cystinosis nephropathic type (CTNS) [MIM:219800]. It is a form of cystinosis, a lysosomal storage disease due to defective transport of cystine across the lysosomal membrane. This results in cystine accumulation and crystallization in the cells causing widespread tissue damage. The classical nephropathic form has onset in the first year of life and is characterized by a polyuro-polydipsic syndrome, marked height-weight growth delay, generalized impaired proximal tubular reabsorptive capacity, with severe fluid-electrolyte balance alterations, renal failure, ocular symptoms and other systemic complications. Defects in CTNS are the cause of cystinosis adult non-nephropathic type (CTNSANN) [MIM:219750]. It is a form of cystinosis, a lysosomal storage disease due to defective transport of cystine across the lysosomal membrane. This results in cystine accumulation and crystallization in the cells causing widespread tissue damage. Cystinosis adult non-nephropathic type is characterized by ocular features and a benigne course. Patients manifest mild photophobia due to conjunctival and corneal cystine crystals. Defects in CTNS are the cause of cystinosis late-onset juvenile or adolescent nephropathic type (CTNSJAN) [MIM:219900]. It is a form of cystinosis, a lysosomal storage disease due to defective transport of cystine across the lysosomal membrane. This results in cystine accumulation and crystallization in the cells causing widespread tissue damage. Late-onset juvenile or adolescent nephropathic cystinosis manifests itself first at age 10 to 12 years with proteinuria due to glomerular damage rather than with the manifestations of tubular damage that occur first in infantile cystinosis. There is no excess amino aciduria and stature is normal. Photophobia, late development of pigmentary retinopathy, and chronic headaches are features. Similarity: Belongs to the cystinosin family. Contains 2 PQ-loop domains. Database links: UniProtKB/Swiss-Prot: O60931.2 Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications |
胱氨酸是一种常染色体隐性遗传疾病，导致从胱氨酸和在出生缺陷溶酶体运输未能茁壮成长，佝偻病和近端肾小管酸中毒。对染色体17p13人类该基因编码的蛋白cystinosin，和基因突变在CTNS负责肾病胱氨酸。CTNS启动子包含SP1结合元件。Cystinosin是一个完整的膜蛋白的跨膜结构域含有7作为H +驱动负责转运溶酶体出口胱氨酸。在人类中，Cystinosin在胰腺、肾脏（成熟和胎儿）和骨骼肌中大量表达。小鼠同源CTNS编码一种蛋白，其表达于除骨骼肌以外的所有组织中。在细胞中，胱氨酸与LAMP-2共同定位至溶酶体。Cystinosin内的C-末端GydQL分选基序是溶酶体定位的关键。