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LMBRD1 Rabbit pAb (bs-18309R)  
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產(chǎn)品編號(hào) bs-18309R
英文名稱 LMBRD1 Rabbit pAb
中文名稱 核輸出信號(hào)相互作用蛋白抗體
別    名 HDAg-L-interacting protein NESI; LMBD1_HUMAN; LMBR1 domain-containing protein 1; lmbrd1; Nuclear export signal-interacting protein; Probable lysosomal cobalamin transporter.  
研究領(lǐng)域 腫瘤  細(xì)胞生物  免疫學(xué)  信號(hào)轉(zhuǎn)導(dǎo)  新陳代謝  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應(yīng) Mouse (predicted: Human,Rat,Rabbit,Pig,Sheep,Cow,Chicken,Dog,Horse)
產(chǎn)品應(yīng)用 IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 61 kDa
檢測(cè)分子量
細(xì)胞定位 細(xì)胞漿 
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human LMBRD1: 21-120/540 <Extracellular>
亞    型 IgG
純化方法 affinity purified by Protein A
緩 沖 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存條件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事項(xiàng) This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
產(chǎn)品介紹 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 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.

SWISS:
Q9NUN5

Gene ID:
55788

Database links:

Entrez Gene: 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



產(chǎn)品圖片
Paraformaldehyde-fixed, paraffin embedded (Mouse brain); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (LMBRD1) Polyclonal Antibody, Unconjugated (bs-18309R) at 1:400 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB staining.
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