Antibody Protein ELISA Kit from MyBioSource header Telephone 1.858.633.0165          
1.855.MyBioSource
Fax 1.858.633.0166    Email sales@mybiosource.com 
tel/fax
menu1 Home Products Ordering Company Help menu7
bottom
   MyBioSource rightarrow Antibody rightarrow OTC rightarrow Polyclonal OTC  rightarrow LOG IN  rightarrow MY ACCOUNT  rightarrow CART CONTENTS  rightarrow CHECKOUT 
horiz bar
MyBioSource Menu separator
separator
ELISA Kit ELISA Kit
CLIA Kit CLIA Kit
PCR Kit PCR Kit
Monoclonal Antibody Monoclonal Antibody
Polyclonal Antibody Polyclonal Antibody
Secondary Antibody Secondary Antibody
Antigen Antigen
Biochemical Biochemical
cDNA Clone cDNA Clone
siRNA siRNA
Peptide Peptide
Recombinant/Purified Protein Rec./Purified Protein

Custom ELISA Kit Custom ELISA Kit
Custom Protein Custom Protein
Custom Antibody Custom Antibody
Antibody Matched Pairs Antibody Matched Pairs
Antibody & Corresponding Blocking Peptide Pairs Antibody Peptide Pairs
Phospho-Specific Antibodies Phospho Antibodies
Products by Disease Products by Disease
Products by Pathway Products by Pathway
Products by Tissue Products by Tissue

arrow Advanced Search
arrow Submit Technical Q&A
arrow International Distributors
arrow Contact Us
separator
Our Best Sellers moreseparator
separator
 • Sarcoplasmic-endoplasmic reticulum calcium ATPase 1 (ATP2A1) ELISA Kit
 • Fructose-bisphosphate aldolase A (ALDOA) Recombinant Protein
 • MIA2 Antibody
 • interferon regulatory factor 4 (IRF4) Antibody
 • Plasminogen Activator Inhibitor 1 (PAI 1) ELISA Kit
 • Defensin alpha4 (DEFA4) Antibody
 • Cytochrome P450 3A4-5 (CYP3A4) Antibody
 • CD1 Antibody
 • 1,3-betaD Glucosidase (1,3-betaD-Glu) ELISA Kit
 • PLAU-uPA ELISA Kit
 • Heat Shock Protein 65, Mycobacterial Antibody
 • Sex Determining Region Y Box Protein 6 (SOX6) ELISA Kit
 • mucin-5AC (MUC5AC) ELISA Kit
 • 3-hydroxyacyl-CoA dehydrogenase type-2 (HSD17B10) ELISA Kit
 downarrow more ...
separator
separator
DatasheetFull DatasheetPrinter Friendly DatasheetPrint This DatasheetAdd to Compare ListHave Questions? Ask UsRequest for a Quotation today

anti-OTC antibody :: Rabbit anti-Human Ornithine carbamoyltransferase Polyclonal Antibody

Scan QR to view Datasheet Catalog #    MBS1494494
Unit / Price
0.05 mg  /  $160 +1 FREE 8GB USB
0.1 mg  /  $235 +1 FREE 8GB USB
 
 Go to:   rightarrow  Product Names   rightarrow Product Info   rightarrow Accession #s   rightarrow Product Desc   rightarrow Diseases/Tissues/Pathways   rightarrow Applications   rightarrow References 
 Product Name   

Ornithine carbamoyltransferase (OTC), Polyclonal Antibody

★Popular Item★
 Also Known As   

Rabbit anti-human Ornithine carbamoyltransferase, mitochondrial polyclonal Antibody(OTC)

 Product Synonym Names    Ornithine transcarbamylase; OTCase
 Product Gene Name   

anti-OTC antibody

[Similar Products]
 Research Use Only    For Research Use Only. Not for use in diagnostic procedures.
Table BarTOPTable Bar
 OMIM    300461
 3D Structure    ModBase 3D Structure for P00480
Section Bar
 Clonality    Polyclonal
 Isotype    IgG
 Host    Rabbit
 Species Reactivity    Human
Section Bar
 Purity/Purification    Caprylic Acid Ammonium Sulfate Precipitation Purified
Section Bar
 Storage Buffer    Preservative: 0.03% Proclin 300
Constituents: 50% Glycerol, 0.01M PBS, PH 7.4
 Conjugate    Non-conjugated
 Immunogen    Recombinant human Ornithine carbamoyltransferase, mitochondrial protein(33-354AA)
 Santa Cruz Alternative    Potential replacement for Santa Cruz Biotechnology antibody catalog# sc-132401 / sc-102051 / sc-102051
Section Bar
 Preparation and Storage    Shipped at 4 degree C. Upon delivery aliquot and store at -20 degree C or -80 degree C. Avoid repeated freeze.
 ISO Certification    Manufactured in an ISO 9001:2008 Certified Laboratory.
 Other Notes    Small volumes of anti-OTC antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.
Table BarTOPTable Bar
 Applications Tested/Suitable for anti-OTC antibody   

ELISA (EIA), Immunohistochemistry (IHC)

Section Bar
 Immunohistochemistry (IHC) of anti-OTC antibody    Immunohistochemistry of paraffin-embedded human liver tissue using MBS1494494 at dilution 1:100
anti-OTC antibody Immunohistochemistry (IHC) (IHC) image
Section Bar
 Immunohistochemistry (IHC) of anti-OTC antibody    Immunohistochemistry of paraffin-embedded human colon cancer using MBS1494494 at dilution 1:100
anti-OTC antibody Immunohistochemistry (IHC) (IHC) image
Table BarTOPTable Bar
NCBI/Uniprot data below describe general gene information for OTC. It may not necessarily be applicable to this product.
 NCBI GI #    38788445
 NCBI GeneID    5009
 NCBI Accession #    NP_000522.3 [Other Products]
 NCBI GenBank Nucleotide #    NM_000531.5 [Other Products]
Section Bar
 UniProt Primary Accession #    P00480 [Other Products]
 UniProt Secondary Accession #    Q3KNR1; Q6B0I1; Q9NYJ5; A8K9P2; D3DWB0 [Other Products]
 UniProt Related Accession #    P00480 [Other Products]
 Molecular Weight    39,935 Da
Table BarTOPTable Bar
 NCBI Official Full Name    ornithine carbamoyltransferase, mitochondrial
 NCBI Official Synonym Full Names    ornithine carbamoyltransferase
 NCBI Official Symbol    OTC [Similar Products]
 NCBI Official Synonym Symbols   
OCTD
[Similar Products]
 NCBI Protein Information    ornithine carbamoyltransferase, mitochondrial
 UniProt Protein Name    Ornithine carbamoyltransferase, mitochondrial
 UniProt Synonym Protein Names   
Ornithine transcarbamylase; OTCase
 Protein Family    Ornithine carbamoyltransferase
 UniProt Gene Name    OTC [Similar Products]
 UniProt Synonym Gene Names    OTCase [Similar Products]
 UniProt Entry Name    OTC_HUMAN
Section Bar
 NCBI Summary for OTC    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]
Section Bar
 UniProt Comments for OTC    OTC: Defects in OTC are the cause of ornithine carbamoyltransferase deficiency (OTCD). 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. Belongs to the ATCase/OTCase family.

Protein type: Amino Acid Metabolism - arginine and proline; Mitochondrial; EC 2.1.3.3; Transferase

Chromosomal Location of Human Ortholog: Xp21.1

Cellular Component: mitochondrial inner membrane; mitochondrial matrix; mitochondrion

Molecular Function: amino acid binding; ornithine carbamoyltransferase activity; phosphate binding; phospholipid binding

Biological Process: arginine biosynthetic process via ornithine; citrulline biosynthetic process; liver development; midgut development; ornithine catabolic process; polyamine metabolic process; response to drug; response to insulin stimulus; response to zinc ion; urea cycle

Disease: Ornithine Transcarbamylase Deficiency, Hyperammonemia Due To
Table BarTOPTable Bar
 Research Articles on OTC    1. In Korean patients with OTC deficiency, mutations in OTC are genetically heterogeneous.
Table BarTOPTable Bar
 Precautions    All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.
 Disclaimer    While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.

It is the responsibility of the customer to report product performance issues to MyBioSource within 30 days of receipt of the product. Please visit our Terms & Conditions page for more information.
Table BarTOPTable Bar
Products associated with anti-OTC antibodyPathways associated with anti-OTC antibody
 Reference Product  PubMed Publications
 ASL antibody  >17 publications with OTC and ASL
 CPS1 antibody  >14 publications with OTC and CPS1
 NAGS antibody  >7 publications with OTC and NAGS
 ARG1 antibody  >5 publications with OTC and ARG1
 ASS1 antibody  >4 publications with OTC and ASS1
 OAT antibody  >3 publications with OTC and OAT
 CAD antibody  >2 publications with OTC and CAD
 ARG2 antibody  >2 publications with OTC and ARG2
 Products by Pathway  Pathway Diagram
 Arginine Biosynthesis Pathway antibodies  Arginine Biosynthesis Pathway Diagram
 Arginine Biosynthesis Pathway antibodies  Arginine Biosynthesis Pathway Diagram
 Biosynthesis Of Amino Acids Pathway antibodies  Biosynthesis Of Amino Acids Pathway Diagram
 Biosynthesis Of Amino Acids Pathway antibodies  Biosynthesis Of Amino Acids Pathway Diagram
 L-citrulline Biosynthesis Pathway antibodies  L-citrulline Biosynthesis Pathway Diagram
 Metabolic Pathways antibodies  Metabolic Pathways Diagram
 Metabolism Pathway antibodies  Metabolism Pathway Diagram
 Metabolism Of Amino Acids And Derivatives Pathway antibodies  Metabolism Of Amino Acids And Derivatives Pathway Diagram
 Metabolism Of Polyamines Pathway antibodies  Metabolism Of Polyamines Pathway Diagram
 Urea Cycle Pathway antibodies  Urea Cycle Pathway Diagram
Diseases associated with anti-OTC antibody
 Disease Name  Pubmed Publications
 Brain Diseases Antibodies  >392 publications with OTC and Brain Diseases
 ORNITHINE TRANSCARBAMYLASE DEFICIENCY, HYPERAMMONEMIA DUE TO Antibodies  >335 publications with OTC and ORNITHINE TRANSCARBAMYLASE DEFICIENCY, HYPERAMMONEMIA DUE TO
 Ornithine Carbamoyltransferase Deficiency Disease Antibodies  >311 publications with OTC and Ornithine Carbamoyltransferase Deficiency Disease
 Hyperammonemia Antibodies  >165 publications with OTC and Hyperammonemia
 Liver Diseases Antibodies  >89 publications with OTC and Liver Diseases
 Drug-Related Side Effects and Adverse Reactions Antibodies  >65 publications with OTC and Drug-Related Side Effects and Adverse Reactions
 Disease Models, Animal Antibodies  >57 publications with OTC and Disease Models, Animal
 Inflammation Antibodies  >52 publications with OTC and Inflammation
 Coma Antibodies  >49 publications with OTC and Coma
 Heart Diseases Antibodies  >47 publications with OTC and Heart Diseases
Table BarTOPTable Bar
horiz bar
 SSL   Follow us on Facebook Follow us onTwitter Follow us on Google Plus Connect us on LinkedIn Subscribe to our RSS Feed for latest products and special promotions