Product Name	SLC19A3
Chinese Name	溶质载体家族19成员3抗体
Alias	Solute carrier family 19 member 3; Thiamine transporter 2; thTr 2; THTR2.
Research Area	Cell biology  Neurobiology  Signal transduction  Transporter  Transmembrane protein
Immunogen Species	Rabbit
Clonality	Polyclonal
React Species	Mouse,  (predicted: Human, Rat, )
Applications	WB=1:500-2000 ELISA=1:5000-10000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 （Paraffin sections need antigen repair） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
Theoretical molecular weight	56kDa
Cellular localization	The cell membrane
Form	Liquid
Concentration	1mg/ml
immunogen	KLH conjugated synthetic peptide derived from human SLC19A3: 261-360/496 <Extracellular>
Lsotype	IgG
Purification	affinity purified by Protein A
Buffer Solution	Preservative: 15mM Sodium Azide, Constituents: 1% BSA, 0.01M PBS, pH 7.4
Storage	Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
Attention	This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed	PubMed
Product Detail	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] Function: Mediates high affinity thiamine uptake, propably via a proton anti-port mechanism. Has no folate transport activity. Subcellular Location: Plasma membrane. Tissue Specificity: Widely expressed but most abundant in placenta, kidney and liver. DISEASE: The disease is caused by mutations affecting the gene represented in this entry. Disease description:An autosomal recessive metabolic disorder characterized by episodic encephalopathy, often triggered by febrile illness, presenting as confusion, seizures, external ophthalmoplegia, dysphagia, and sometimes coma and death. If untreated, encephalopathies can result in permanent dystonia. Brain imaging may show characteristic bilateral lesions of the basal ganglia. Similarity: Belongs to the reduced folate carrier (RFC) transporter (TC 2.A.48) family. SWISS: Q9BZV2 Gene ID: 80704 Database links: Entrez Gene: 80704 Human Omim: 606152 Human SwissProt: Q9BZV2 Human Unigene: 221597 Human
Product Picture	Sample: Liver (Mouse) Lysate at 40 ug Primary: Anti-SLC19A3 (SL8702R) at 1/300 dilution Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution Predicted band size: 56 kD Observed band size: 56 kD

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