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
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. |