CPS1 Rabbit polyclonal antibody

Size

£152.00

SKU:: A8080
Additional Names:: CPS1|CPSASE1|GATD6|PHN
Application:: ELISA, WB, IF, ICC
Molecular Weight:: 165kDa
Species Reactivity:: Human
Purification:: Affinity Purified
Storage Conditions:: -20[o]C Avoid freeze/thaw cycles.
Supplier:: Abclonal
Host:: Rabbit
Reactivities:: Human, Mouse, Rat
Immunogen:: Recombinant protein (or fragment).This information is considered to be commercially sensitive.
Formulation:: Unmodified
Sequence:: IKNVVSGKTSACFEPSLDYMVTKIPRWDLDRFHGTSSRIGSSMKSVGEVMAIGRTFEESFQKALRMCHPSIEGFTPRLPMNKEWPSNLDLRKELSEPSSTRIYAIAKAIDDNMSLDEIEKLTYIDKWFLYKMRDILNMEKTLKGLNSESMTEETLKRAKEIGFSDKQISKCLGLTEAQTRELRLKKNIHPWVKQIDTLAAEYPSVTNYLYVTYNGQEHDVN
Uniprot:: P31327
Synonyms:: carbamoyl-phosphate synthase (ammonia);carbamoyl-phosphate synthase [ammonia], mitochondrial;carbamoyl-phosphate synthase 1, mitochondrial;Carbamoyl-phosphate synthetase I;carbamoylphosphate synthetase I;CPSASE1;GATD6;PHN
Extra 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.

Shipping Conditions:: Blue Ice