NeoMass AAAC is used with Tandem Mass Spectrometry to detect concentrations of amino acid, free carnitine, acylcarnitines, succinylacetone and argininosuccinic acid.
As a first in the world achievement, Labsystems Diagnostics has established a methodology to measure OTC, CPS1 and NAGS deficiencies on dried blood spot along with the traditional detection of ASS, ASL and ARG1 enzyme deficiencies. NeoMass AAAC for tandem MS will therefore enable detection of all urea cycle abnormalities.
Patented innovation by Labsystems Diagnostics
NeoMass AAAC innovation enables detection of all urea cycle deficiencies, including proximal abnormalities along with classical distal deficiencies measurements.
The urea cycle is a metabolic pathway occurring in the liver and kidney. Ammonia, a product of protein metabolism, is removed from circulating blood through urea cycle by conversion into urea and its elimination in urine. Urea Cycle Disorders (UCD) occurs in some inborn errors of metabolism, resulting in accumulation of nitrogenous waste which leads to vomiting, increasing lethargy, seizures, hypotonia, respiratory distress, coma, and to death if untreated. Protein restriction is the mainstay of therapy for the management of urea cycle defects.
Unique Properties of NeoMass AAAC kit detection of OTC, CPS1 and NAGS deficiencies
Deficiencies of enzymes in the proximal part of the urea cycle have been challenging to detect in the past but the patented innovation in the new NeoMass AAAC kit changes this. As a first in the world achievement, Labsystems Diagnostics has established a methodology to measure OTC, CPS1 and NAGS deficiencies on dried blood spots along with the traditional detection of ASS, ASL and ARG1 enzyme deficiencies. NeoMass AAAC for tandem MS will therefore enable detection of all urea cycle abnormalities.
- The broadest available panel of analytes
- Easy sample preparation without derivatization
- More Analytes with less expenses
- Possibility of Automatic Sample Preparation
- 3 levels of blood spots controls - Low, Mid, High
- Unique endogenous level control
- Unraveling of Proximal & Distal Urea Cycle Disorder
- Includes Succinylacetone (SUAC) and Argininosuccinic Acid (ASA) standards
- Assay for screening more than 50 inherited metabolic disorders
- Ready-to-use kit for 960 tests
- 4 of steps: Punch, Extraction, Transfer, Injection into MS/MS
- Sample extraction without derivatization
- 4 sets of Isotope labelled internal Standards: 15 Amino Acids + 13 (Acyl) Carnitines + SUAC (Tyrosemia type-1) + ASA (Argininosuccinic Acidemia)
- 3 Dried Blood Spot Controls framing the decisional cut-off area for each of the matching internal standards
- Turnaround time for 96 samples approximately 3 hours
- High specificity and sensitivity by MS/MS detection
- Validation for multiple instrument applications
- Final concentrations calculated based on accurate peak area ratios compared to only peak intensities ratios