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Quantitative Determination of Phenylketonuria

 Quantitative Determination of Phenylketonuria
Zivak PKU HPLC-UV Analysis Kit was developed for sensitive, rapid and accurate quantitative detection of Phenylketonuria in human dried blood spot & serum samples. Main methods and procedures that have been selected are based on EN ISO 13485 and 98/79/EC.

Phenylketonuria (PKU) is an autosomal recessive genetic disorder characterized by a deficiency in the enzyme phenylalanine hydroxylase (PAH). This enzyme is necessary to metabolise the amino acid phenylalanine to the amino acid tyrosine. When PAH is deficient, phenylalanine accumulates on the brain tissue and is converted into phenyl pyruvate (also known as phenyl ketone), which is detected in the urine. PAH is found on chromosome number 12. If left untreated, this condition can cause problems with brain development, leading to progressive mental retardation and seizures. However, PKU can be controlled by a special diet with low in phenylalanine and high in tyrosine can be a very effective treatment. There is no cure. The disorder is only detectable after irreversible damage is done hence early diagnosis is crucial. Most babies in developed countries are screened for PKU soon after birth. PKU is usually detected using the HPLC test, but some clinics still use the Guthrie test, as a part of national biochemical screening programs.

 Quantitative Determination of Phenylketonuria 0

HPLC Parameters (for serum sample ) : contact for more details

Storage & Stability: contact for more details

Preparation of Sample , Calibrators & Controls (Serum): contact for more details

 

Materials Supplied (for serum samples)

Order No.

Volume

Component

ZV-4003-02R1-10

1 x 150 ml

Reagent 1, Contains internal standard

ZV-4003-02MP-10

2 x 2 L

Mobile Phase, Contains organic solvent

  ZV-4003-02WB-10

  1 x 0,5 L

Washing solution, Contains organic solvent

ZV-4003-KK-10

1 pc

User Guide

 
 
Materials Required But Not Supplied (for serum samples)

Order No.

Volume

Component

ZV-4003-02S1-10

1 x 3 ml

 Dried Blood Spot Calibrator Level 1

ZV-4003-02K1-10

1 x 3 ml

          Dried Blood Spot Control Level 1

ZV-4003-02K2-10

1 x 3 ml

          Dried Blood Spot Control Level 2

ZV-4003-02C1-10

1 pcs

Zivak PKU Serum HPLC Analytical Column

 
 
Materials Supplied (for dried blood samples)

Order No.

Volume

Component

ZV-4003-05R1-15

1 x 150 ml

Reagent 1, Contains internal standard

ZV-4003-05MP-15

2 x 2 L

Mobile Phase, Contains organic solvent

  ZV-4003-05WB-15

1 x 0,5 L

Washing solution, Contains organic solvent

ZV-4003-KK-Eng-15

1 pc

User Guide

 
 
Materials Required But Not Supplied (for dried blood samples)

Order No.

Volume

Component

ZV-4003-02S1-15

1 x 1 Spot

  Dried Blood Spot Calibrator Level 1

ZV-4003-02K1-15

1 x 1 Spot

     Dried Blood Spot Control Level 1

ZV-4003-02K2-15

1 x 1 Spot

     Dried Blood Spot Control Level 2

ZV-4003-02C1-15

       1 pcs

     Zivak PKU Dried Blood Spots HPLC Analytical Column

 

Zivak General Catalog

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