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OTC Polyclonal Antibody

CAT: 0498-bs-6724RSize: 100 µLDry Ice: NoHazardous: No
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CAT#:0498-bs-6724RSize:100 µL
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24/48H Stock Items & 2 to 6 Weeks non Stock Items.
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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.
Synonyms
OCTD; Ornithine carbamoyltransferase, mitochondrial; Ornithine transcarbamylase; OTCase; OTC
Gene ID
5009
Swiss Prot
P00480
Cellular Locus
Cytoplasm
Host
Rabbit
Cross Reactivity
Human
Immunogen
51-150/354
Target
OTC
Clonality
Polyclonal
Isotype
IgG
Conjugation
Unconjugated
Source
KLH conjugated synthetic peptide derived from human OTC
Applications
WB, ELISA
Purification
Purified by Protein A.
Concentration
1µg/µl
Dilution
WB (1:300-5000), ELISA (1:500-1000)
Buffer
0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
Modification
Unmodified
Storage Conditions
Shipped at 4C. Store at -20C for one year. Avoid repeated freeze/thaw cycles.
Gene ID URL
5009
Predicted Cross Reactivity
Mouse, Rat, Dog, Cow, Pig, Horse

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