Product Information
66036-1-PBS targets ASS1 in WB, IHC, IF/ICC, Indirect ELISA applications and shows reactivity with human, pig samples.
Tested Reactivity | human, pig |
Host / Isotype | Mouse / IgG1 |
Class | Monoclonal |
Type | Antibody |
Immunogen | ASS1 fusion protein Ag9314 Predict reactive species |
Full Name | argininosuccinate synthetase 1 |
Calculated Molecular Weight | 412 aa, 47 kDa |
Observed Molecular Weight | 40-45 kDa |
GenBank Accession Number | BC009243 |
Gene Symbol | ASS1 |
Gene ID (NCBI) | 445 |
RRID | AB_11042883 |
Conjugate | Unconjugated |
Form | Liquid |
Purification Method | Protein G purification |
UNIPROT ID | P00966 |
Storage Buffer | PBS only , pH 7.3 |
Storage Conditions | Store at -80°C. |
Background Information
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(PMID:19006241).