LMBRD1 Antikörper (AA 67-540)

Produktnummer ABIN7876379

Dieser Kaninchen Polyklonal Antikörper detektiert spezifisch LMBRD1 in ELISA, WB und FACS. Es zeigt Reaktivität gegenüber Proben von Human.

Kurzübersicht für LMBRD1 Antikörper (AA 67-540) (ABIN7876379)

Target: LMBRD1 (LMBR1 Domain Containing 1 (LMBRD1))

Reaktivität: Human

Wirt: Kaninchen

Klonalität: Polyklonal

Konjugat: Dieser LMBRD1 Antikörper ist unkonjugiert

Applikation: ELISA, Western Blotting (WB), Flow Cytometry (FACS)

Produktdetails anti-LMBRD1 Antikörper

Bindungsspezifität: AA 67-540

Verwendungszweck: LMBRD1 Antibody / Lysosomal cobalamin transport escort protein 1

Aufreinigung: Immunogen affinity purified

Immunogen: E.coli-derived human LMBRD1 recombinant protein (Position: D67-A540) was used as the immunogen for the LMBRD1 antibody.

Isotyp: IgG

Anwendungsinformationen

Applikationshinweise: Optimal dilution of the LMBRD1 antibody should be determined by the researcher.

Beschränkungen: Nur für Forschungszwecke einsetzbar

Handhabung

Format: Lyophilized

Rekonstitution: Adding 0.2 mL of distilled water will yield a concentration of 500 μg/mL

Buffer: Each vial contains 4 mg Trehalose, 0.9 mg NaCl, 0.2 mg Na2HPO4.

Lagerung: 4 °C,-20 °C

Informationen zur Lagerung: After reconstitution, the LMBRD1 antibody can be stored for up to one month at 4oC. For long-term, aliquot and store at -20oC. Avoid repeated freezing and thawing.

Details zu LMBRD1

Target: LMBRD1 (LMBR1 Domain Containing 1 (LMBRD1))

Andere Bezeichnung: LMBRD1

Hintergrund: LMBRD1 antibody detects Lysosomal cobalamin transport escort protein 1, an intracellular transporter essential for vitamin B12 metabolism. The UniProt recommended name is Lysosomal cobalamin transport escort protein 1 (LMBRD1). This membrane-associated protein facilitates the export of cobalamin (vitamin B12) from lysosomes into the cytoplasm, where it is converted into active cofactors for metabolic enzymes.

Functionally, LMBRD1 antibody identifies a 540-amino-acid transmembrane protein localized to the lysosomal membrane. LMBRD1 interacts with the lysosomal cobalamin transporter ABCD4 to mediate the transfer of cobalamin from endocytic vesicles to the cytosol. This process is crucial for the formation of methylcobalamin and adenosylcobalamin, cofactors required for methionine synthase and methylmalonyl-CoA mutase activity.

The LMBRD1 gene is located on chromosome 6q13 and is expressed in liver, kidney, and other metabolically active tissues. Its function links lysosomal transport to one-carbon metabolism and mitochondrial energy homeostasis. Proper LMBRD1 activity ensures efficient cobalamin utilization and cellular methylation balance.

Pathologically, mutations in LMBRD1 cause cobalamin deficiency type F (cblF), a metabolic disorder characterized by methylmalonic acidemia and homocystinuria. Defects in LMBRD1 disrupt lysosomal cobalamin release, leading to systemic metabolic dysfunction. Research using LMBRD1 antibody supports studies in lysosomal biology, vitamin metabolism, and inherited metabolic diseases.

LMBRD1 antibody is validated for western blotting, immunofluorescence, and immunohistochemistry to detect lysosomal transport proteins. NSJ Bioreagents provides LMBRD1 antibody reagents optimized for research in metabolism, intracellular trafficking, and lysosomal function.

Structurally, Lysosomal cobalamin transport escort protein 1 contains multiple transmembrane helices and a cytosolic domain that interacts with ABCD4. This antibody aids in exploring LMBRD1's mechanistic role in cobalamin metabolism and cellular nutrient transport.

UniProt: Q9NUN5