核输出信号相互作用蛋白抗体
规格：1mg/1ml
英文名： LMBRD1
别名： HDAg-L-interacting protein NESI; LMBD1_HUMAN; LMBR1 domain-containing protein 1; lmbrd1; Nuclear export signal-interacting protein; Probable lysosomal cobalamin transporter.
分子量： 61kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：Polyclonal
亚型：IgG
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human LMBRD1
交叉反应：Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Horse, Rabbit, Sheep,
细胞定位：
核输出信号相互作用蛋白抗体产品介绍：background: This gene encodes a lysosomal membrane protein that may be involved in the transport and metabolism of cobalamin. This protein also interacts with the large form of the hepatitis delta antigen and may be required for the nucleocytoplasmic shuttling of the hepatitis delta virus. Mutations in this gene are associated with the vitamin B12 metabolism disorder termed, homocystinuria-megaloblastic anemia complementation type F.[provided by RefSeq, Oct 2009] Function: Probable lysosomal cobalamin transporter. Required to export cobalamin from lysosomes allowing its conversion to cofactors. Isoform 3 may play a role in the assembly of hepatitis delta virus (HDV). Subcellular Location: Lysosome membrane. Tissue Specificity: Isoform 3 is expressed in liver. Post-translational modifications: N-glycosylated. DISEASE: Defects in LMBRD1 are the cause of methylmalonic aciduria and homocystinuria type cblF (MMAFHC) [MIM:277380]; also known as核输出信号相互作用蛋白抗体 homocystinuria-megaloblastic anemia complementation type F. MMAFHC is a disorder of cobalamin metabolism characterized by decreased levels of the coenzymes adenosylcobalamin (AdoCbl) and methylcobalamin (MeCbl). It is due to accumulation of free cobalamin in lysosomes, thus hindering its conversion to cofactors. Clinical features include developmental delay, stomatitis, glossitis, seizures and methylmalonic aciduria responsive to vitamin B12. Similarity: Belongs to the LIMR family. LMBRD1 subfamily. Gene ID: 55788 Database links: Entrez Gene: 核输出信号相互作用蛋白抗体55788 Human Entrez Gene: 68421 Mouse Entrez Gene: 246046 Rat Omim: 612625 Human SwissProt: Q9NUN5 Human SwissProt: Q8K0B2 Mouse SwissProt: Q6AZ61 Rat Unigene: 271643 Human Unigene: 677072 Human Unigene: 477783 Mouse Unigene: 201860 Rat Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
核输出信号相互作用蛋白抗体产品应用：WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
研究领域：肿瘤 细胞生物 **学 信号转导 新陈代谢
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid