The SLC19A3 Polyclonal Antibody (PACO59377) is a valuable tool for researchers studying SLC19A3, a solute carrier protein involved in the transport of thiamine (Vitamin B1) across cell membranes. This antibody, developed in rabbits, exhibits high specificity and sensitivity for human samples, making it ideal for Western blotting applications.SLC19A3 plays a crucial role in maintaining thiamine homeostasis in the body, essential for proper cellular function and metabolism. Mutations in the SLC19A3 gene have been linked to thiamine-responsive megaloblastic anemia, a rare disorder characterized by an inability to effectively utilize thiamine.
By using the SLC19A3 Polyclonal Antibody, researchers can study the expression and localization of SLC19A3 in various cell types, aiding in the understanding of thiamine transport mechanisms and the pathophysiology of related disorders. This antibody is a valuable tool for investigations into thiamine metabolism, nutritional deficiencies, and potential therapeutic interventions.
Western Blot. Positive WB detected in: SH-SY5Y whole cell lysate, HepG2 whole cell lysate, 293 whole cell lysate, Hela whole cell lysate. All lanes: SLC19A3 antibody at 3.6µg/ml. Secondary. Goat polyclonal to rabbit IgG at 1/50000 dilution. Predicted band size: 56 kDa. Observed band size: 56 kDa.
Immunofluorescence staining of HepG2 cells with PACO59377 at 1:166, counter-stained with DAPI. The cells were fixed in 4% formaldehyde, permeabilized using 0.2% Triton X-100 and blocked in 10% normal Goat Serum. The cells were then incubated with the antibody overnight at 4°C. The secondary antibody was Alexa Fluor 488-congugated AffiniPure Goat Anti-Rabbit IgG(H+L).
Background:
Mediates high affinity thiamine uptake, probably via a proton anti-port mechanism. Has no folate transport activity.
Synonyms:
Thiamine transporter 2 (ThTr-2) (ThTr2) (Solute carrier family 19 member 3), SLC19A3
UniProt Protein Function:
Mediates high affinity thiamine uptake, probably via a proton anti-port mechanism. Has no folate transport activity.
NCBI Summary:
This gene encodes a ubiquitously expressed transmembrane thiamine transporter that lacks folate transport activity. Mutations in this gene cause biotin-responsive basal ganglia disease (BBGD); a recessive disorder manifested in childhood that progresses to chronic encephalopathy, dystonia, quadriparesis, and death if untreated. Patients with BBGD have bilateral necrosis in the head of the caudate nucleus and in the putamen. Administration of high doses of biotin in the early progression of the disorder eliminates pathological symptoms while delayed treatment results in residual paraparesis, mild cognitive disability, or dystonia. Administration of thiamine is ineffective in the treatment of this disorder. Experiments have failed to show that this protein can transport biotin. Mutations in this gene also cause a Wernicke's-like encephalopathy.[provided by RefSeq, Jan 2010]
