Ornithine-delta-aminotransferase catalyzes the major catalytic reaction for ornithine.
Ornithinemia presumably due to deficiency of ornithine ketoacid aminotransferase (OAT) was found in 9 patients with gyrate atrophy of the choroid and retina.
The clinical history of gyrate atrophy is usually night blindness that begins in late childhood, accompanied by sharply demarcated circular areas of chorioretinal atrophy.
During the second and third decades the areas of atrophy enlarge.
Ornithine levels were 10 to 20 times higher than normal in plasma, urine, spinal fluid and aqueous humor.
No consistent clinical abnormality other than the ocular one was found.
Hyperammonemia was not found in the fasting state or after meals or stress testing.
All the patients' parents were from the same geographic area of Finland.
Applications:Suitable for use in ELISA, Western Blot, Immunohistochemistry, Immunocytochemistry.
Other applications not tested.
Recommended Dilution:ELISA: 1:100-1:5000 Western Blot: 1:50-400 Immunohistochemistry (frozen): 1:50-500 Immunohistochemistry (paraffin): 1:10-100 Immunocytochemistry: 1:50-500 Optimal dilutions to be determined by the researcher.
Storage and Stability:May be stored at 4°C for short-term only.
Aliquot to avoid repeated freezing and thawing.
Store at -20°C.
Aliquots are stable for 12 months after receipt.
For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
仕様
Size：200ul
Host：rabbit
Source Antibody：human
Grade：Affinity Purified
Purity：Purified by immunoaffinity chromatography.
Form：Supplied as a liquid in PBS, pH 7.4, 0.02% sodium azide, 50% glycerol.
Specificity：Recognizes human OAT.
Isotype：IgG
Calc Applications Abbrev：E IC IHC WB
Calc Crossreactivity：Hu
Immunogen：Recombinant protein corresponding to Met1-Phe301of human OAT, fused to two N-terminal Tags, His-Tag and T7-tag, expressed in E. coli.
