Thiamine transporter 2; ThTr-2; ThTr2; Solute carrier family 19 member 3; SLC19A3

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

Polyclonal

Rabbit Ig

Rabbit

This SLC19A3 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 217-246 amino acids from the Central region of human SLC19A3.

Peptide Affinity Purified Rabbit Polyclonal Antibody (Pab)

Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is purified through a protein A column, followed by peptide affinity purification.

Vial Concentration: 0.5 (lot specific)

Maintain refrigerated at 2-8 degree C for up to 6 months. For long term storage store at -20 degree C in small aliquots to prevent freeze-thaw cycles.

Small volumes of anti-SLC19A3 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.

SLC19A3 encodes a ubiquitously expressed transmembrane thiamine transporter that lacks folate transport activity.

Signal Transduction

Western Blot (WB), ELISA (EIA)

WB~~1:1000

SLC19A3 Antibody (Center) western blot analysis in NCI-H292 cell line lysates (35ug/lane).This demonstrates the SLC19A3 antibody detected the SLC19A3 protein (arrow).

55665

solute carrier family 19 (thiamine transporter), member 3

thiamine transporter 2

Thiamine transporter 2

ThTr-2; ThTr2??[Similar Products]

S19A3_HUMAN

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 mental retardation, 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]

SLC19A3: Mediates high affinity thiamine uptake, propably via a proton anti-port mechanism. Has no folate transport activity. Defects in SLC19A3 are the cause of biotin-responsive basal ganglia disease (BBGD). BBGD is a recessive disorder with childhood onset that presents as a subacute encephalopathy, with confusion, dysarthria, and dysphagia, and that progresses to severe rigidity, dystonia, quadriparesis and death if not treated. Belongs to the reduced folate carrier (RFC) transporter (TC 2.A.48) family.

Protein type: Transporter; Membrane protein, multi-pass; Membrane protein, integral; Transporter, SLC family

Chromosomal Location of Human Ortholog: 2q37

Cellular Component: integral to membrane; plasma membrane

Molecular Function: thiamin uptake transmembrane transporter activity

Biological Process: vitamin metabolic process; thiamin and derivative metabolic process; water-soluble vitamin metabolic process

Disease: Thiamine Metabolism Dysfunction Syndrome 2 (biotin- Or Thiamine-responsive Type)

Mee, L., et al. Am. J. Physiol. Gastrointest. Liver Physiol. 297 (1), G197-G206 (2009)
Liu, X., et al. Tumour Biol. 30 (5-6), 242-248 (2009)
Subramanian, V.S., et al. J. Physiol. (Lond.) 582 (PT 1), 73-85 (2007)

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