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| Reactivity: | H,M,R |
| Applications: | WB |
| Host Species: | Rabbit |
| Clonality: | Polyclonal |
| Full Name: | ASS1 rabbit polyclonal antibody |
Gene Name: | Argininosuccinate synthase |
Synonyms: | Citrulline--aspartate ligase, ASS1, Ass, CTLN1, ASSA |
Immunogen: | KLH conjugated Synthetic peptide corresponding to Mouse ASS1 |
Isotype: | IgG |
Purity: | Affinity purification |
Predicted MW. | 47 kDa |
Observed MW. | 47 kDa |
Uniprot ID: | P00966, P16460, P09034 |
Product Usage Information
Applications | Species | Dilution | Positive tissue |
WB | Human, Mouse, Rat | 1: 500-1: 1000 | liver, kidney |
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.
Images
| Western blot analysis of ASS1 (GB111882) at dilution of 1: 500 |
Storage
| Storage | Store at -20°C for one year. Avoid repeated freeze/thaw cycles. |
| Storage Buffer | PBS with 0.02%sodium azide,100 μg/ml BSA and 50% glycerol. |
