Human Ornithine carbamoyltransferase, mitochondrial, OTC ELISA Kit | MyBioSource MBS900262 product information

product summary

company name :

MyBioSource

product type :

ELISA/assay

product name :

Human Ornithine carbamoyltransferase, mitochondrial, OTC ELISA Kit

catalog :

MBS900262

quantity :

48-Strip-Wells

price :

510 USD

more info or order :

MyBioSource product webpage

product information

catalog number :

MBS900262

products type :

ELISA Kit

products full name :

Human Ornithine carbamoyltransferase, mitochondrial, OTC ELISA Kit

products short name :

ornithine carbamoyltransferase

products name syn :

Human Ornithine carbamoyltransferase; mitochondrial (OTC) ELISA kit; MGC129967; MGC129968; MGC138856; OCTD; ornithine transcarbamylase; ornithine carbamoyltransferase

other names :

ornithine carbamoyltransferase, mitochondrial; Ornithine carbamoyltransferase, mitochondrial; ornithine carbamoyltransferase, mitochondrial; OTCase; ornithine transcarbamylase; ornithine carbamoyltransferase; Ornithine transcarbamylase; OTCase

products gene name :

OTC

other gene names :

OTC; OTC; OCTD; OTCase

uniprot entry name :

OTC_HUMAN

reactivity :

Human

sequence length :

354

specificity :

This assay has high sensitivity and excellent specificity for detection of human OTC. No significant cross-reactivity or interference between human OTC and analogues was observed.

storage stability :

Unopened test kits should be stored at 2 to 8 degree C upon receipt. Please refer to pdf manual for further storage instructions.

other info1 :

Samples: Serum, plasma, tissue homogenates and Cell lysates. Assay Type: Sandwich. Detection Range: 1. 25 mU/ml -80 mU/ml. Sensitivity: The minimum detectable dose of human OTC is typically less than 0.312 mU/ml. The sensitivity of this assay, or Lower Limit of Detection (LLD) was defined as the lowest protein concentration that could be differentiated from zero. It was determined the mean O.D value of 20 replicates of the zero standard added by their three standard deviations.

other info2 :

Intra-assay Precision: Intra-assay Precision (Precision within an assay): CV%<8%. Three samples of known concentration were tested twenty times on one plate to assess. Inter-assay Precision: Inter-assay Precision (Precision between assays): CV%<10%. Three samples of known concentration were tested in twenty assays to assess.

products description :

Princple of the assay: This assay employs the quantitative sandwich enzyme immunoassay technique. Antibody specific for OTC has been pre-coated onto a microplate. Standards and samples are pipetted into the wells and any OTC present is bound by the immobilized antibody. After removing any unbound substances, a biotin-conjugated antibody specific for OTC is added to the wells. After washing, avidin conjugated Horseradish Peroxidase (HRP) is added to the wells. Following a wash to remove any unbound avidin-enzyme reagent, a substrate solution is added to the wells and color develops in proportion to the amount of OTC bound in the initial step. The color development is stopped and the intensity of the color is measured.

ncbi gi num :

38788445

ncbi acc num :

NP_000522.3

ncbi gb acc num :

NM_000531.5

uniprot acc num :

P00480

ncbi mol weight :

39,935 Da

ncbi pathways :

Arginine And Proline Metabolism Pathway (82957); Arginine And Proline Metabolism Pathway (323); Biosynthesis Of Amino Acids Pathway (790012); Biosynthesis Of Amino Acids Pathway (795174); Metabolic Pathways (132956); Metabolism Pathway (477135); Metabolism Of Amino Acids And Derivatives Pathway (106169); Urea Cycle Pathway (413351); Urea Cycle Pathway (106171); Urea Cycle Pathway (468222)

ncbi summary :

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 summary :

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: EC 2.1.3.3; Amino Acid Metabolism - arginine and proline; Transferase; Mitochondrial. Chromosomal Location of Human Ortholog: Xp21.1. Cellular Component: mitochondrion; mitochondrial matrix; mitochondrial inner membrane. Molecular Function: amino acid binding; ornithine carbamoyltransferase activity; phospholipid binding; phosphate binding. Biological Process: response to drug; citrulline biosynthetic process; response to zinc ion; midgut development; arginine biosynthetic process via ornithine; liver development; ornithine catabolic process; response to insulin stimulus; urea cycle. Disease: Ornithine Transcarbamylase Deficiency, Hyperammonemia Due To

size1 :

48-Strip-Wells

price1 :

510 USD

size2 :

96-Strip-Wells

price2 :

725

size3 :

5x96-Strip-Wells

price3 :

2565

size4 :

10x96-Strip-Wells

price4 :

4800

more info or order :

MyBioSource product webpage