SLC19A3 Antikörper (C-Term)

Produktnummer ABIN5516361

Dieser Kaninchen Polyklonal Antikörper detektiert spezifisch SLC19A3 in WB. Es zeigt Reaktivität gegenüber Proben von Human.

Kurzübersicht für SLC19A3 Antikörper (C-Term) (ABIN5516361)

Target: SLC19A3 (Slc19a3) (Solute Carrier Family 19, Member 3 (Slc19a3))

Reaktivität: Human

Wirt: Kaninchen

Klonalität: Polyklonal

Konjugat: Dieser SLC19A3 Antikörper ist unkonjugiert

Applikation: Western Blotting (WB)

Produktdetails anti-SLC19A3 Antikörper

Bindungsspezifität: C-Term

Sequenz: VYGSYFAVIA GIFLMRSMYI TYSTKSQKDV QSPAPSENPD VSHPEEESNI

Aufreinigung: Affinity purified

Immunogen: The immunogen is a synthetic peptide directed towards the C terminal region of human SLC19A3

Anwendungsinformationen

Applikationshinweise: Optimal working dilution should be determined by the investigator.

Beschränkungen: Nur für Forschungszwecke einsetzbar

Handhabung

Format: Liquid

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

Konservierungsmittel: Sodium azide

Vorsichtsmaßnahmen: This product contains Sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.

Lagerung: -20 °C

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

Details zu SLC19A3

Target: SLC19A3 (Slc19a3) (Solute Carrier Family 19, Member 3 (Slc19a3))

Andere Bezeichnung: SLC19A3

Hintergrund: 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.

Alias Symbols: BBGD, THMD2, THTR2

Protein Size: 496

Gen-ID: 80704

NCBI Accession: NM_025243, NP_079519

UniProt: Q9BZV2

Pathways: Dicarboxylic Acid Transport