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SLC19A3 Antikörper (C-Term)

Slc19a3 Reaktivität: Human WB Wirt: Kaninchen Polyclonal unconjugated
Produktnummer ABIN5516361
  • Target Alle SLC19A3 (Slc19a3) Antikörper anzeigen
    SLC19A3 (Slc19a3) (Solute Carrier Family 19, Member 3 (Slc19a3))
    Bindungsspezifität
    • 7
    • 6
    • 6
    • 6
    • 4
    • 1
    • 1
    • 1
    • 1
    C-Term
    Reaktivität
    • 22
    • 3
    • 2
    • 2
    • 2
    • 2
    • 2
    • 1
    Human
    Wirt
    • 20
    • 2
    Kaninchen
    Klonalität
    • 20
    • 2
    Polyklonal
    Konjugat
    • 9
    • 3
    • 3
    • 3
    • 2
    • 2
    Dieser SLC19A3 Antikörper ist unkonjugiert
    Applikation
    • 18
    • 17
    • 1
    • 1
    Western Blotting (WB)
    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
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    Discover our top product Slc19a3 Primärantikörper
  • Applikationshinweise
    Optimal working dilution should be determined by the investigator.
    Beschränkungen
    Nur für Forschungszwecke einsetzbar
  • 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.
  • Target
    SLC19A3 (Slc19a3) (Solute Carrier Family 19, Member 3 (Slc19a3))
    Andere Bezeichnung
    SLC19A3 (Slc19a3 Produkte)
    Synonyme
    BBGD antikoerper, THMD2 antikoerper, THTR2 antikoerper, thtr2 antikoerper, MGC52872 antikoerper, MGC89434 antikoerper, si:dkey-223n17.4 antikoerper, slc19a3 antikoerper, A230084E24Rik antikoerper, AI788884 antikoerper, ThTr2 antikoerper, solute carrier family 19 member 3 antikoerper, solute carrier family 19 member 3 L homeolog antikoerper, solute carrier family 19 (thiamine transporter), member 3 antikoerper, thiamine transporter 2 antikoerper, solute carrier family 19 (thiamine transporter), member 3b antikoerper, solute carrier family 19, member 3 antikoerper, thiamine transporter 2-like antikoerper, SLC19A3 antikoerper, Slc19a3 antikoerper, slc19a3.L antikoerper, slc19a3 antikoerper, LOC486151 antikoerper, slc19a3b antikoerper, LOC100230080 antikoerper
    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
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