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ASS1 Polyclonal Antibody

规格: / 20μL / 60μL / 120μL / 200μL
价格: / ¥420 / ¥1010 / ¥1640 / ¥2365

货号:E-AB-18521

宿主: Rabbit

反应性: H,M,R

应用: WB,IHC

  • 详情
  • Overview

    Synonyms Argininosuccinate synthase 1,Argininosuccinate synthase,Argininosuccinate synthetase 1,ASS,Ass-1,ass1,ASSA,ASSY,Citrulline aspartate ligase,Citrulline--aspartate ligase,CTLN1,ASS1抗体
    Swissprot P00966
    Source Rabbit
    Reactivity Human,Mouse,Rat
    Immunogen Fusion protein of human ASS1
    Application WB(Detection kit: E-IR-R304),IHC(Detection kit: E-IR-R213)
    Recommended dilution WB,,1:500-1:2000;IHC,,1:25-1:100;
    Concentration 0.5mg/mL
    Clonality Polyclonal

    Properties

    Cellular localization Cytosol,Extracellular region or secreted,extracellular exosome,Mitochondrion,Other locations:cytoplasm,myelin sheath
    Isotype IgG
    Purification Affinity purification
    Conjugation Unconjugated
    Storage instructions Store at -20℃. Avoid freeze / thaw cycles.
    Storage buffer PBS with 0.05% NaN3 and 40% Glycerol,pH7.4
    Background ASS1 is a rate-limiting enzyme in the urea cycle. Citrullinemia type I is an autosomal recessive disorder that is caused by a deficiency of the urea cycle enzyme argininosuccinate synthetase (ASS1). Deficiency of ASS1 shows various clinical manifestations encompassing severely affected patients with fatal neonatal hyperammonemia as well as asymptomatic individuals with only a biochemical phenotype.
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