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14-September-2008 18:38:50 - Argininosuccinate synthetase Redirected from ASS gene Argininosuccinate synthetase Identifiers Symbol ASS Entrez 445 HUGO 758 OMIM 603470 RefSeq NM_000050 UniProt P00966 Other data EC number 6.3.4.5 Locus Chr. 9 q34.1 Argininosuccinate synthetase ASS is an enzyme that catalyzes the synthesis of argininosuccinate from citrulline and aspartate ASS is responsible for the third step of the urea cycle and one of the reactions of the Citrulline-NO cycle. Gene The gene that encodes for this enzyme, ASS, is located on chromosome 9. In humans, ASS is expressed mostly in the cells of liver and kidney. Pathology Citrullinemia: At least 50 mutations that cause type I citrullinemia have been identified in the ASS gene. Most of these mutations substitute one amino acid for another in ASS. These mutations likely affect the structure of the enzyme and its ability to bind to citrulline, aspartate, and other molecules. A few mutations lead to the production of an abnormally short enzyme that cannot effectively play its role in the urea cycle. Defects in ASS disrupt the third step of the urea cycle, preventing the liver from processing excess nitrogen into urea. As a result, nitrogen in the form of ammonia and other byproducts of the urea cycle such as citrulline build up in the bloodstream. Ammonia is toxic, particularly to the nervous system. An accumulation of ammonia during the first few days of life leads to poor feeding, vomiting, seizures, and the other signs and symptoms of type I citrullinemia. See also Citrullinemia Urea cycle Synthetase v d e Metabolism: amino acid metabolism - urea cycle enzymes Main cycle mitochondrial matrix: Carbamoyl phosphate synthetase I - Ornithine transcarbamylase cytosol: Argininosuccinate synthetase - Argininosuccinate lyase - Arginase Regulatory/transport N-Acetylglutamate synthase - Ornithine translocase see also disorders v d e Enzymes: ligases EC 6 6.1 - Carbon-Oxygen Aminoacyl tRNA synthetase 6.2 - Carbon-Sulfur Succinyl coenzyme A synthetase - Acetyl Co-A synthetase - Long fatty acyl CoA synthetase 6.3 - Carbon-Nitrogen Glutamine synthetase - Ubiquitin ligase Von Hippel-Lindau tumor suppressor, UBE3A, Mdm2, Anaphase-promoting complex - Glutathione synthetase - CTP synthase - Adenylosuccinate synthase - Argininosuccinate synthetase - Holocarboxylase synthetase - GMP synthase - Asparagine synthetase - Carbamoyl phosphate synthetase I, II - Gamma-glutamylcysteine synthetase 6.4 - Carbon-Carbon Carbon-carbon ligases 6.5 - Phosphoric Ester DNA ligase Retrieved from http://en..org/wiki/Argininosuccinate_synthetase Categories: Genes on chromosome 9 | EnzymesHidden category: Protein pages needing a picture Views Article Discussion this page History Personal tools Log in / create account Navigation Main page Contents Featured content Current events Random article Search Go Search Interaction Community portal Recent changes Contact Donate to Help Toolbox What links here Related changes Upload file Special pages Printable version Permanent link Cite this page Languages 中文 This page was last modified on 3 September 2008, at 22:15
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