Table 1 Global metabolic profiling.

From: Metabolomics Analysis of Skeletal Muscles from FKRP-Deficient Mice Indicates Improvement After Gene Replacement Therapy

Metabolic Pathway

Metabolites Assayed

aFKRPP448L versus C57BL/6

aAAV9-hFKRP versus FKRPP448L

aAAV9-hFKRP versus C57BL/6

Amino Acid

111

49 (32)

42

22

Peptide

11

3 (3)

2

1

Carbohydrate

43

25 (14)

11

6

Energy

13

4 (3)

3

2

Lipid

282

132 (74)

111

54

Nucleotide

32

19 (11)

10

8

Cofactor/Vitamin

20

8 (8)

4

2

Xenobiotic

12

7 (7)

4

4

Total

524

247

187

99

Downregulated

 

103

134

61

Upregulated

 

144

53

38

  1. aStatistically significant (p ≤ 0.05, q ≤ 0.10) metabolites detected in global metabolic profiling experiments which are determined from comparison tests of the quadriceps muscles derived from 52-week old C57BL/6, untreated FKRPP448L, and AAV9-hFKRP-treated FKRPP448L mice. Values in parentheses refer to the number of metabolites in a given pathway that showed cumulative p ≤ 0.05.
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