LMBR domain containing 1, LMBD1, nuclear export-signal interacting protein, NESI

For Research Use Only. Not for use in diagnostic procedures.

Polyclonal

Rabbit

Affinity chromatography purified via peptide column

Supplied in PBS containing 0.02% sodium azide.

Can be stored at -20 degree C, stable for one year. As with all antibodies care should be taken to avoid repeated freeze thaw cycles. Antibodies should not be exposed to prolonged high temperatures.

Small volumes of anti-LMBRD1 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

LMBRD1, also known as NESI (nuclear export signal-interacting protein, is a lysosomal membrane protein that is thought be involved in the transport and metabolism of cobalamin. LMBRD1 was initially identified as interacting 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 (cblF).

Total protein Ab

ELISA (EIA), Western Blot (WB), Immunohistochemistry (IHC)

Western blot analysis of LMBRD1 in human brain tissue lysate with LMBRD1 antibody at 1 ug/mL.

Immunohistochemistry of LMBRD1 in human brain tissue with LMBRD1 antibody at 2.5 ug/mL.

21,394 Da

LMBR1 domain containing 1

probable lysosomal cobalamin transporter

Probable lysosomal cobalamin transporter

C6orf209; NESI??[Similar Products]

LMBD1_HUMAN

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]

LMBRD1: 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). Defects in LMBRD1 are the cause of methylmalonic aciduria and homocystinuria type cblF (MMAHCF). 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. Belongs to the LIMR family. LMBRD1 subfamily. 4 isoforms of the human protein are produced by alternative splicing.

Protein type: Membrane protein, integral; Membrane protein, multi-pass

Chromosomal Location of Human Ortholog: 6q13

Cellular Component: membrane; lysosomal membrane; plasma membrane; integral to membrane; clathrin-coated endocytic vesicle

Molecular Function: insulin receptor binding; cobalamin binding

Biological Process: negative regulation of glucose import; negative regulation of protein kinase B signaling cascade; vitamin metabolic process; viral reproduction; cobalamin metabolic process; negative regulation of insulin receptor signaling pathway; water-soluble vitamin metabolic process

Disease: Methylmalonic Aciduria And Homocystinuria, Cblf Type

All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.

While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.

It is the responsibility of the customer to report product performance issues to MyBioSource within 30 days of receipt of the product. Please visit our Terms & Conditions page for more information.