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ORGANIC ACID IN URINE

Organic Acid in Urine LC-MS/MS Analysis Kit

Human urine is a complex biofluid of diverse small polar metabolites such as organic acids. Organic acidurias are inborn errors of metabolism given rise to by defections of inherited enzymes which lead to accumulation excess amounts of normal organic acids in human urine. For this reason qualitative and quantitative analyses of urinary organic acids are prominent diagnostic tools for the interpretation of metabolite profile that reflects inherited metabolic disorders.

“Jasem-Organic Acids” method overcomes abovementioned drawbacks by using liquid chromatography tandem mass spectrometry (LC-MS/MS). Jasem method ensures quantification of 54 organic acids by absence of derivatization simplified sample preperation to diluting. Jasem offers two panels achieving sufficient chromatographic separation for crucial isomers by LC-MS/MS.

Highlights

12 minutes run for each panel (Total analysis time 24 minutes-Panel 1&2).

Analysis of 54 organic acids without derivatization.

Quick and easy sample preparation; just dilution!

Successful chromatographic separation of isomers.

Consuming small volume of patient’s sample.

Parameters

2-Methyl citrate, 2-OH-Phenylacetic acid, 2-OH-Butyric acid, 2-Oxoadipic acid, 3-Methylglutaconic acid, 3-OH-2-Methylbutanoic acid, 3-OH-3-Methylglutaric acid, 3-OH-Butyric acid, 3-OH-Isobutyrate, 3-Phenyllactic acid, 4-OH-Phenylacetic acid, Fumaric acid, Glycolic acid, Hexanoylglycine, Homogentisic acid, Malic acid, Malonic acid, N-(3-phenyl propionyl) glycine, N-Acetylaspartic acid, N-Acetyltyrosine, N-isovaleryl glycine, Oxoproline, 4-OH-Phenyl lactic acid, Propionylglycine, Sebacic acid, Suberic acid, Suberylglycine, Succinylacetone, 2-OH-Glutaric acid, 2-OH-3-Methylpentanoic acid, 2-OH-Isocaproic acid, 2-OH-Isovaleric acid, 3-OH-Glutaric acid, 3-OH-Propanoic acid, 3-Methylcrotonyl glycine, 3-Methylglutaric acid, 3-Methyl-2-oxovaleric acid, 3-OH-Isovaleric acid, 3-OH-pentanoic acid, 4-Methyl-2-oxovaleric acid, 4-OH-Phenylpyruvic acid, Adipic acid, Alpha-Ketoglutaric acid, Citric acid, Ethylmalonic acid, Glutaconic acid, Glutaric acid, Lactic acid, Methylmalonic acid, Orotic acid, Phenylpyruvic acid, Pyruvic acid, Succinic acid, Tiglylglycine

Matrix

Urine

Sample Preparation

  • Step 1
    Dilute urine sample 5 times using Reagent-1
  • Step 2
    Transfer 50 µl of diluted urine sample to a vial
  • Step 3
    Add 25 µl of IS mix and 425 µl of Reagent-1 into the sample and inject to LC-MS/MS system

Chromatogram

METHOD PERFORMANCE

NO

COMPOUNDS

LOQ(ppm) 6460 QQQ

LOQ (ppm) Ultivo

Linearity

Recovery (%)6460 QQQ

Repeatability

R2 6460 QQQ

RSD (%) 6460 QQQ

1

Alpha-Ketoglutaric acid

0,61

1,65

0,9979

118

1,74

2

2-Methyl citrate

0,72

1,22

0,9969

95,7

16,03

3

2-OH-3-Methylpentanoic acid

0,08

0,12

0,9986

104,2

6,98

4

2-OH-Butyric acid

0,06

0,28

0,9987

120

4,97

5

2-OH-Glutaric acid

0,37

0,84

0,9992

106,6

1,97

6

2-OH-Isocaproic acid

0,07

0,22

0,9992

108,4

5,66

7

2-OH-Isovaleric acid

0,07

0,06

0,9976

90,7

2,59

8

2-OH-Phenylacetic acid

0,07

0,06

0,9994

105,8

1,59

9

2-Oxoadipic acid

0,06

0,89

0,9983

111,8

7,78

10

3-Methyl -2-oxovaleric acid

0,13

1,88

0,9953

91,7

6,13

11

3-Methylcrotonylglycine

0,1

0,13

0,9998

96,9

2,99

12

3-Methylglutaconic acid

0,05

0,03

0,9987

104,2

2,8

13

3-Methylglutaric acid

0,08

0,04

0,999

87,1

3,38

14

3-OH-2-Methylbutanoic acid

0,09

0,51

0,9856

99,4

13,27

15

3-OH-3-Methylglutaric acid

0,18

0,99

0,9994

82,5

1,85

16

3-OH-Butyric acid

0,26

0,09

0,9991

113,5

8,84

17

3-OH-Glutaric acid

0,05

0,21

0,9996

115

2,7

18

3-OH-Isobutyrate

0,55

0,45

0,9994

94,4

1,74

19

3-OH-Isovaleric  acid

0,11

0,16

0,9983

101,8

3,24

20

3-OH-Pentanoic acid

0,16

0,12

0,9996

94,14

6,95

21

3-OH-Propanoic acid

1,49

2,51

0,9983

88,4

4,55

22

3-Phenyllactic acid

0,06

0,07

0,999

115,9

6,34

23

4-Methyl-2-oxovaleric acid

0,15

1,92

0,9999

111,3

6,46

24

4-OH-Phenyl lactic acid

0,08

0,08

0,9968

77,3

10,34

25

4-OH-Phenylacetic acid

1,4

1,67

0,9997

110,1

2,01

26

4-OH-Phenylpyruvic acid

0,39

2,19

0,9977

115,8

6,03

27

Adipic acid

0,08

0,14

0,9993

100,2

6,01

28

Citric acid

0,39

1,91

0,9957

101,5

8,85

29

Ethylmalonic acid

0,05

0,2

0,9984

86,4

1,98

30

Fumaric acid

0,06

0,49

0,999

75,9

10,75

31

Glutaconic acid

0,2

0,5

0,9992

101,8

11,58

32

Glutaric acid

0,23

0,51

0,9999

93,6

2,45

33

Glycolic acid

0,72

5,51

0,9977

101,6

11,9

34

Hexanoylglycine

0,09

0,09

0,9985

116,5

3,08

35

Homogentisic acid

0,04

0,02

0,9993

78,7

3,19

36

Lactic acid

3,54

3,32

0,9997

106,4

10,8

37

Malic acid

0,06

0,18

0,9952

115

4,04

38

Malonic acid

0,07

0,36

0,9996

115

6,15

39

Methylmalonic acid

0,34

0,41

0,9998

87,6

3,27

40

N-(3-phenyl propionyl)glycine

0,05

0,07

0,9984

106,4

2,03

41

N-Acetylaspartic acid

0,1

0,1

0,9994

119

5,89

42

N-acetyltyrosine

0,13

0,06

0,9995

86,3

6,03

43

N-isovalerylglycine

0,07

0,11

0,9997

96

2,61

44

Orotic acid

0,04

0,82

0,9991

73,1

2,36

45

Oxoproline

0,05

0,07

0,9984

79,5

3,72

46

Phenylpyruvic acid

0,1

0,09

0,9995

98,3

6,3

47

Propionylglycine

0,18

0,15

0,9995

100,3

3,51

48

Pyruvic acid

0,22

4,98

0,9965

119

17,09

49

Sebacic acid

0,07

0,12

0,9998

94,5

2,4

50

Suberic acid

0,08

0,11

0,9993

94,9

1,99

51

Suberylglycine

0,13

0,09

0,9998

111,8

3,14

52

Succinic acid

0,1

0,11

0,9996

92,9

3,25

53

Succinylacetone

0,05

0,06

0,9999

102,9

4,24

54

Tiglylglycine

0,05

0,16

0,9999

93,5

4,74
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