Product info:

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.

Alternative names:

CPS1; CPSASE1; PHN

Species reactivity:

Human, Mouse, Rat

Host:

Rabbit

Cellular localisation:

Mitochondrion, Nucleus, nucleolus

Isotype:

IgG

Immunogen:

Recombinant fusion protein containing a sequence corresponding to amino acids 820-930 of human CPS1 (NP_001116105.1).

Positive control:

HepG2, NCI-H460, HeLa, Mouse liver, Rat liver

AA Sequence:

RMCHPSIEGFTPRLPMNKEWPSNLDLRKELSEPSSTRIYAIAKAIDDNMSLDEIEKLTYIDKWFLYKMRDILNMEKTLKGLNSESMTEETLKRAKEIGFSDKQISKCLGLT

Purification:

Affinity purification

Molecular weight:

Calculated MW: 116kDa/164kDa/165kDa Observed MW: 165kDa

Form:

Liquid

Applications:

IF (Homo sapiens) WB (Homo sapiens)

Dilution:

WB 1:1000 - 1:2000 IF 1:50 - 1:200

Storage buffer:

Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.

Storage:

Store at 4°C short term. For long-term storage, aliquot and store at -20°C or below. Stable for 12 months at -20°C. Avoid repeated freeze-thaw cycles.

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