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Rabbit Anti-LMBRD1/BF647 Conjugated antibody (bs-18309R-BF647)
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產品編號 bs-18309R-BF647
英文名稱 Rabbit Anti-LMBRD1/BF647 Conjugated antibody
中文名稱 BF647標記的核輸出信號相互作用蛋白抗體
別    名 HDAg-L-interacting protein NESI; LMBD1_HUMAN; LMBR1 domain-containing protein 1; lmbrd1; Nuclear export signal-interacting protein; Probable lysosomal cobalamin transporter.  
規(guī)格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領域 腫瘤  細胞生物  免疫學  信號轉導  新陳代謝  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 Mouse,  (predicted: Human, Rat, Chicken, Dog, Pig, Cow, Horse, Rabbit, Sheep, )
產品應用 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 61kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human LMBRD1
亞    型 IgG
純化方法 affinity purified by Protein A
儲 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存條件 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.
產品介紹 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 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.

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



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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