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Rabbit anti-Human CPS1 Monoclonal Antibody

Cat NoBMA-1257
Conjugate
Type重组兔多抗
SourceRabbit
Size50 uL
ApplicationImmunogen
FormatLiquid
ConcentrationPlease refer to the vial lable for the specific concentration.
BufferSupplied in PBS with 0.02% sodium azide,0.05% BSA,50% glycerol,pH7.3.
SpeciesHuman
StorageStore at -20℃. Avoid freeze / thaw cycles.
SynonymsCPSASE1;PHN
PurificationAffinity purification
MolecularWeight160KDa
Description
BackgroundThe 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.
We can offer labeled proteins/antibodies using a broad range of intensely fluorescent dyes and labels including FITC, Biotin, Alexa Fluor, Rhodamine B, Cy, Co-Au.
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