注册 | 登录
点击这里给我发消息
所有产品
  • 所有产品
  • 一抗
  • 裂解液
>   首页   >   产品   >   一抗   >   其他   >   OTC/Ornithine Carbamoyltransferase Rabbit pAb   

OTC/Ornithine Carbamoyltransferase Rabbit pAb

     
  • 产品详情
  • 实验流程
Product Information
Application
  • Applications Legend:
  • E=ELISA
  • WB=Western Blotting
  • IHC=Immunohistochemistry
  • IHC-P=Immunohistochemistry (Paraffin)
  • IP=Immunoprecipitation
  • IF=Immunofluorescence
  • IC=Immunochemistry
  • ICC=Immunocytochemistry
  • FC=Flow Cytometry
  • DB=Dot Blot
WB, IHC-P, IHC-F, IF
Primary Accession P00480
Other Accession P00480
Host Rabbit
Clonality Polyclonal
Calculated MW 39935 Da
Physical State Liquid
Immunogen KLH conjugated synthetic peptide derived from human OTC/Ornithine Carbamoyltransferase
Epitope Specificity 31-130/354
Isotype IgG
Purity affinity purified by Protein A
Buffer 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
SIMILARITY Belongs to the ATCase/OTCase family.
SUBUNIT Homotrimer.
Post-translational modifications Acetylation at Lys-88 negatively regulates ornithine carbamoyltransferase activity in response to nutrient signals.
DISEASE 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.
Important Note This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
Background Descriptions This nuclear gene encodes a mitochondrial matrix enzyme. Missense, nonsense, and frameshift mutations in this enzyme lead to ornithine transcarbamylase deficiency, which causes hyperammonemia. Since the gene for this enzyme maps close to that for Duchenne muscular dystrophy, it may play a role in that disease also. [provided by RefSeq, Jul 2008]
Additional Information
Gene ID 5009
Other Names OCTD; OTC1; OTCD; OTCase; Sf; spf; OTC_BOVIN; OTC; Ornithine carbamoyltransferase, mitochondrial; 2.1.3.3; OTC_HUMAN; OTC_MOUSE; OTC_PIG; OTC_RAT; OTC_SHEEP; ornithine transcarbamylase; ornithine carbamoyltransferase; ornithine transcarbamylase deficiency
Dilution WB=1:500-2000,IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500,Flow-Cyt=1ug/Test
StorageStore at -20 °C for one year. Avoid repeated freeze/thaw cycles. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.

For Research Use Only. Not For Use In Diagnostic Procedures.

Protein Information
Name OTC (HGNC:8512)
Function Catalyzes the second step of the urea cycle, the condensation of carbamoyl phosphate with L-ornithine to form L-citrulline (PubMed:2556444, PubMed:6372096, PubMed:8112735). The urea cycle ensures the detoxification of ammonia by converting it to urea for excretion (PubMed:2556444).
Cellular Location Mitochondrion matrix
Tissue Location Mainly expressed in liver and intestinal mucosa.
Research Areas
FeedBack

终于等到您。ABCEPTA(百远生物)抗体产品。
点击下方“我要评价 ”按钮提交您的反馈信息,您的反馈和评价是我们最宝贵的财富之一,
我们将在1-3个工作日内处理您的反馈信息。

如有疑问,联系:0512-88856768 tech-china@abcepta.com.


我要评价