LMBRD1 Polyclonal Antibody
Purified Rabbit Polyclonal Antibody (Pab)
- 产品详情
- 实验流程
Application
| IHC-P, IHC-F, IF, ICC |
|---|---|
| Primary Accession | Q9NUN5 |
| Reactivity | Rat, Pig, Dog, Bovine |
| Host | Rabbit |
| Clonality | Polyclonal |
| Calculated MW | 61389 Da |
| Physical State | Liquid |
| Immunogen | KLH conjugated synthetic peptide derived from human LMBRD1 |
| Epitope Specificity | 21-120/540 |
| Isotype | IgG |
| Purity | affinity purified by Protein A |
| Buffer | 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| SUBCELLULAR LOCATION | Lysosome membrane. |
| SIMILARITY | Belongs to the LIMR family. LMBRD1 subfamily. |
| 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 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. |
| Important Note | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| Background Descriptions | 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] |
| Gene ID | 55788 |
|---|---|
| Other Names | Lysosomal cobalamin transport escort protein LMBD1, LMBD1, HDAg-L-interacting protein NESI, LMBR1 domain-containing protein 1, Nuclear export signal-interacting protein, LMBRD1 (HGNC:23038), C6orf209, NESI |
| Target/Specificity | Isoform 3 is expressed in liver. |
| Dilution | IHC-P=1:100-500,IHC-F=1:100-500,ICC=1:100-500,IF=1:100-500 |
| Storage | Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. 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. |
| Name | LMBRD1 (HGNC:23038) |
|---|---|
| Synonyms | C6orf209, NESI |
| Function | Lysosomal membrane chaperone required to export cobalamin (vitamin B12) from the lysosome to the cytosol, allowing its conversion to cofactors (PubMed:19136951). Targets ABCD4 transporter from the endoplasmic reticulum to the lysosome (PubMed:27456980). Then forms a complex with lysosomal ABCD4 and cytoplasmic MMACHC to transport cobalamin across the lysosomal membrane (PubMed:25535791). Acts as an adapter protein which plays an important role in mediating and regulating the internalization of the insulin receptor (INSR) (By similarity). Involved in clathrin-mediated endocytosis of INSR via its interaction with adapter protein complex 2 (By similarity). Essential for the initiation of gastrulation and early formation of mesoderm structures during embryogenesis (By similarity). |
| Cellular Location | Endoplasmic reticulum membrane. Lysosome membrane; Multi-pass membrane protein. Cell membrane {ECO:0000250|UniProtKB:Q8K0B2}; Multi-pass membrane protein. Cytoplasmic vesicle, clathrin-coated vesicle {ECO:0000250|UniProtKB:Q8K0B2} |
| Tissue Location | Isoform 3 is expressed in liver. |
Research Areas
For Research Use Only. Not For Use In Diagnostic Procedures.
Application Protocols
Provided below are standard protocols that you may find useful for product applications.
终于等到您。ABCEPTA(百远生物)抗体产品。
点击下方“我要评价 ”按钮提交您的反馈信息,您的反馈和评价是我们最宝贵的财富之一,
我们将在1-3个工作日内处理您的反馈信息。
如有疑问,联系:0512-88856768 tech-china@abcepta.com.
















癌症的基本特征包括细胞增殖、血管生成、迁移、凋亡逃避机制和细胞永生等。找到癌症发生过程中这些通路的关键标记物和对应的抗体用于检测至关重要。
为您推荐一个泛素化位点预测神器——泛素化分析工具,可以为您的蛋白的泛素化位点作出预测和评分。
细胞自噬受体图形绘图工具为你的蛋白的细胞受体结合位点作出预测和评分,识别结合到自噬通路中的蛋白是非常重要的,便于让我们理解自噬在正常生理、病理过程中的作用,如发育、细胞分化、神经退化性疾病、压力条件下、感染和癌症。