CPS1 Rabbit polyclonal antibody

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£152.00

SKU:: A8080
Zusätzliche Namen:: CPS1|CPSASE1|GATD6|PHN
Anwendung:: ELISA, WB, IF, ICC
Molekulargewicht:: 165kDa
Spezies-Reaktivität:: Human
Aufreinigung:: Affinity Purified
Lagerbedingungen:: -20[o]C Avoid freeze/thaw cycles.
Hersteller:: Abclonal
Host:: Rabbit
Reaktivitäten:: Human, Mouse, Rat
Immunogen:: Recombinant protein (or fragment).This information is considered to be commercially sensitive.
Formulierung:: Unmodified
Sequenz:: IKNVVSGKTSACFEPSLDYMVTKIPRWDLDRFHGTSSRIGSSMKSVGEVMAIGRTFEESFQKALRMCHPSIEGFTPRLPMNKEWPSNLDLRKELSEPSSTRIYAIAKAIDDNMSLDEIEKLTYIDKWFLYKMRDILNMEKTLKGLNSESMTEETLKRAKEIGFSDKQISKCLGLTEAQTRELRLKKNIHPWVKQIDTLAAEYPSVTNYLYVTYNGQEHDVN
Uniprot:: P31327
Synonyme:: carbamoyl-phosphate synthase (ammonia);carbamoyl-phosphate synthase [ammonia], mitochondrial;carbamoyl-phosphate synthase 1, mitochondrial;Carbamoyl-phosphate synthetase I;carbamoylphosphate synthetase I;CPSASE1;GATD6;PHN
Weitere Details:: The mitochondrial enzyme encoded by this gene catalyzes synthesis of carbamoyl phosphate from ammonia and bicarbonate. This reaction is the first committed step of the urea cycle, which is important in the removal of excess urea from cells. The encoded protein may also represent a core mitochondrial nucleoid protein. Three transcript variants encoding different isoforms have been found for this gene. The shortest isoform may not be localized to the mitochondrion. Mutations in this gene have been associated with carbamoyl phosphate synthetase deficiency, susceptibility to persistent pulmonary hypertension, and susceptibility to venoocclusive disease after bone marrow transplantation.

Versandbedingungen:: Blue Ice