LMBRD1, Polyclonal Antibody

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

Polyclonal

Rabbit

Affinity purified

Liquid. Purified antibody supplied in 1x PBS buffer with 0.09% (w/v) sodium azide and 2% sucrose.

For short term use, store at 2-8 degree C up to 1 week. For long term storage, store at -20 degree C in small aliquots to prevent freeze-thaw cycles.

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.

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.

Polyclonal; Signal Proteins; Immunology; Membrane & Traffic; Disease Related;

Western Blot (WB)

Host: Rabbit
Target Name: LMBRD1
Sample Tissue: Human Ovary Tumor lysates
Antibody Dilution: 1ug/ml

51 kDa

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, multi-pass; Membrane protein, integral

Chromosomal Location of Human Ortholog: 6q13

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

Molecular Function: cobalamin binding; insulin receptor 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

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