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

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Catalog # MBS1494494
Unit / Price
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  0.05 mg  /  $160 +1 FREE 8GB USB
  0.1 mg  /  $235 +1 FREE 8GB USB
Product Name

Ornithine carbamoyltransferase (OTC), Polyclonal Antibody

Popular Item
Full Product Name

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

Product Synonym Names
Ornithine transcarbamylase; OTCase
Product Gene Name
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
OMIM
300461
3D Structure
ModBase 3D Structure for P00480
Clonality
Polyclonal
Isotype
IgG
Host
Rabbit
Species Reactivity
Human
Purity/Purification
Caprylic Acid Ammonium Sulfate Precipitation Purified
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
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.
Applications Tested/Suitable for anti-OTC antibody
ELISA (EIA), Immunohistochemistry (IHC)

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
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
NCBI/Uniprot data below describe general gene information for OTC. It may not necessarily be applicable to this product.
NCBI GI #
NCBI GeneID
NCBI Accession #
NCBI GenBank Nucleotide #
UniProt Primary Accession #
UniProt Secondary Accession #
UniProt Related Accession #
Molecular Weight
39,935 Da
NCBI Official Full Name
ornithine carbamoyltransferase, mitochondrial
NCBI Official Synonym Full Names
ornithine carbamoyltransferase
NCBI Official Symbol
NCBI Official Synonym Symbols
NCBI Protein Information
ornithine carbamoyltransferase, mitochondrial
UniProt Protein Name
Ornithine carbamoyltransferase, mitochondrial
UniProt Synonym Protein Names
Ornithine transcarbamylase; OTCase
UniProt Gene Name
UniProt Synonym Gene Names
OTCase  [Similar Products]
UniProt Entry Name
OTC_HUMAN
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]
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
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.
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