產(chǎn)品詳情
簡單介紹：
Anti-OTC抗體產(chǎn)品質(zhì)量穩(wěn)定，實(shí)驗(yàn)效果明顯，貨期快，價(jià)格優(yōu)惠，歡迎垂詢訂購！我公司長期供應(yīng)**組化抗體、WB抗體、**組化試劑盒和抗體試驗(yàn)所需全部相關(guān)試劑、熒光標(biāo)記抗體、單克隆抗體、多克隆抗體、各種標(biāo)記的二抗IgG/IgM/IgD/IgA等科研實(shí)驗(yàn)抗體。Anti-OTC抗體用于**組化實(shí)驗(yàn),WB實(shí)驗(yàn),相應(yīng)的標(biāo)記抗體有HRP標(biāo)記抗體,FITC標(biāo)記,BIO等。
詳情介紹：
Rabbit Anti-OTC
Cat. Number:
Anti-OTC抗體KL-6724R
Quantity size:
0.2ml
Concentration:
1mg/ml Buffer = 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
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 Anti-OTC抗體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.
Also known as:
OCTD; Ornithine Anti-OTC抗體carbamoyltransferase mitochondrial; Ornithine Carbamoyltransferase; Ornithine carbamoyltransferase, mitochondrial; Ornithine transcarbamylase; Otc; OTC_HUMAN; OTCase.
Specificity:
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Rabbit Polyclonal IgG, affinity purified by Protein A.
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Reacts with: Human, Anti-OTC抗體Mouse, Rat, Dog, Pig, Cow, Horse, .
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Immunogen: KLH conjugated synthetic peptide derived from human OTC (33-80aa).
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Predicted Molecular Weight: 40kDa.
Storage:
Shipped at 4℃, Store at -20℃ (Avoid repeated freeze/thaw cycles).
Application:
WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:100-500
Not yet tested in other applications. Anti-OTC抗體
Optimal working dilutions must be determined by the end user.
Not yet tested in other applications. Anti-OTC抗體
Optimal working dilutions must be determined by the end user.
