bs-6724R [Primary Antibody]
OTC Polyclonal Antibody
www.biossusa.com
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Host: Rabbit

Target Protein: OTC

Immunogen Range: 51-150/354


Clonality: Polyclonal

Isotype: IgG

Entrez Gene: 5009

Swiss Prot: P00480

Source: KLH conjugated synthetic peptide derived from human OTC

Purification: Purified by Protein A.

Storage Buffer: 0.01M TBS(pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.

Storage: Shipped at 4°C. Store at -20°C for one year. Avoid repeated freeze/thaw cycles.

Background:

Defects in OTC are the cause of ornithine carbamoyltransferase deficiency (OTCD) [MIM:311250]. OTCD is an X-linked disorder of the urea cycle which causes a form of hyperammonemia. Mutations with no residual enzyme activity are always expressed in hemizygote males by a very severe neonatal hyperammonemic coma that generally proves to be fatal. Heterozygous females are either asymptomatic or express orotic aciduria spontaneously or after protein intake. The disorder is treatable with supplemental dietary arginine and low protein diet. The arbitrary classification of patients into the 'neonatal' group (clinical hyperammonemia in the first few days of life) and 'late' onset (clinical presentation after the neonatal period) has been used to differentiate severe from mild forms.

Size: 100ul

Concentration: 1ug/ul

Applications: WB(1:300-5000)
ELISA(1:500-1000)

Predicted Molecular Weight: 40


Cross Reactive Species: Human

Predicted Cross Reactive Species: Mouse
Rat
Dog
Cow
Pig
Horse

For research use only. Not intended for diagnostic or therapeutic use.

VALIDATION IMAGES

Human HepG2 cells probed with OTC Polyclonal Antibody, unconjugated (bs-6724R) at 1:300 overnight at 4°C followed by a conjugated secondary antibody at 1:10000 for 90 minutes at 37°C.