扫码关注公众号           扫码咨询技术支持           扫码咨询技术服务
  
客服热线:400-901-9800  客服QQ:4009019800  技术答疑  技术支持  质量反馈  人才招聘  关于我们  联系我们
产品中心-北京博奥森生物技术有限公司
首页 > 产品中心 > 标记一抗 > 产品信息
Rabbit Anti-MLC1/Biotin Conjugated antibody (bs-7115R-Bio)
订购热线:400-901-9800
订购邮箱:sales@bioss.com.cn
订购QQ:  400-901-9800
技术支持:techsupport@bioss.com.cn
说 明 书: 100ul  
100ul/2980.00元
大包装/询价
产品编号 bs-7115R-Bio
英文名称1 Rabbit Anti-MLC1/Biotin Conjugated antibody
中文名称 生物素标记的膜蛋白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 (predicted: Human, Mouse, Rat, Dog, Pig, Horse, Rabbit, )
产品应用 WB=1:50-200 ELISA=1:100-1000 IHC-P=1:50-200 IHC-F=1:50-200 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
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.
版权所有 2004-2026 www.bioss.com.cn 北京博奥森生物技术有限公司
通过国际质量管理体系ISO 9001:2015 GB/T 19001-2016    证书编号: 00124Q34771R2M/1100
通过国际医疗器械-质量管理体系ISO 13485:2016 GB/T 42061-2022    证书编号: CQC24QY10047R0M/1100
京ICP备05066980号-1         京公网安备110107000727号