FITC标记的膜蛋白MLC1抗体
产品名称： FITC标记的膜蛋白MLC1抗体
英文名称： Anti-MLC1/FITC
产品编号： HZ-7115R-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-MLC1/FITC Conjugated antibody
FITC标记的膜蛋白MLC1抗体
| 英文名称 | Anti-MLC1/FITC |
| 中文名称 | FITC标记的膜蛋白MLC1抗体 |
| 别 名 | KIAA0027; LVM; Megalencephalic leukoencephalopathy with subcortical cysts 1; Membrane protein MLC1; MLC; MLC-1; MLC1_HUMAN; VL; WKL1. |
| 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 神经生物学 信号转导 通道蛋白 细胞膜蛋白 |
| 抗体来源 | Rabbit |
| 克隆类型 | Polyclonal |
| 交叉反应 | Human, Mouse, Rat, Dog, Pig, Horse, 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 | 41kDa |
| 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 MLC1 |
| 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: MLC1 is a 377 amino acid multi-pass membrane protein that may serve as a non-selective neuronal cation channel in brain. Mutant MLC1 proteins that show impaired folding have been corrected in vitro with the addition of a Ca(2+)-ATPase inhibitor, curcumin. Mutations in the gene encoding MLC1 is the cause of megalencephalic leukoencephalopathy with subcortical cysts, also known as van der Knaap disease, a rare syndrome characterized early in life by progressive brain destruction causing mental retardation and incoordination. Single nucleotide polymorphisms within the MLC1 gene may be associated with periodic catatonia, but there seems to be conflicting evidence on whether or not the gene is implicated in general schizophrenia. Function: May be a transporter. May act as a non-selective neuronal cation channel. Subunit: Interacts with ATP1B1. Part of a complex containing ATP1B1, TRPV4, AQP4 and HEPACAM. Subcellular Location: Membrane; Multi-pass membrane protein (Potential). Cell membrane. Cytoplasm, perinuclear region. Endoplasmic reticulum. Tissue Specificity: Expressed in the brain, with highest levels found in the amygdala, nucleus caudatus, thalamus and hippocampus. DISEASE: Defects in MLC1 are a cause of leukoencephalopathy megalencephalic with subcortical cysts (MLC) [MIM:604004]. MLC is a syndrome of cerebral leukoencephalopathy and megalencephaly characterized by ataxia, spasticity, seizures, delay in motor development and mild mental retardation. The brain appears swollen on magnetic resonance imaging, with diffuse white-matter abnormalities and the invariable presence of subcortical cysts in frontal and temporal lobes. Database links: UniProtKB/Swiss-Prot: Q15049.5 Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications |
MLC1是一种377氨基酸多通道膜蛋白，可作为脑内非选择性神经元阳离子通道。通过添加Ca(2+)-ATPase抑制剂姜黄素，体外纠正了显示受损折叠的突变MLC1蛋白。编码MLC1的基因突变是伴有皮层下囊肿的大脑白质脑病的原因，也称为范德克纳普病，一种罕见的症状，特征为早期生活进行性脑破坏，导致智力迟钝和不协调。MLC1基因内的单核苷酸多态性可能与周期性紧张症有关，但在该基因是否与一般精神分裂症有关，似乎存在相互矛盾的证据。