#METABOLOMICS WORKBENCH silviaradenkovic_20230418_143045 DATATRACK_ID:3870 STUDY_ID:ST002566 ANALYSIS_ID:AN004227 PROJECT_ID:PR001653 VERSION 1 CREATED_ON April 18, 2023, 5:28 pm #PROJECT PR:PROJECT_TITLE Metabolomic profiling of PMM2-CDG patient fibroblasts PR:PROJECT_SUMMARY Abnormal polyol metabolism has been predominantly associated with diabetes, PR:PROJECT_SUMMARY where excess glucose is converted to sorbitol by aldose reductase (AR). PR:PROJECT_SUMMARY Recently, abnormal polyol metabolism has also been implicated in PR:PROJECT_SUMMARY phosphomannomutase 2-congenital disorder of glycosylation (PMM2-CDG), and PR:PROJECT_SUMMARY epalrestat, an AR inhibitor, proposed as a potential therapy for this disorder. PR:PROJECT_SUMMARY Given that the PMM enzyme is not closely connected to polyol metabolism, and, PR:PROJECT_SUMMARY unlike in diabetes, PMM2-CDG does not present with hyperglycemia in blood, the PR:PROJECT_SUMMARY increased polyol production, and the therapeutic mechanism of epalrestat in PR:PROJECT_SUMMARY PMM2-CDG remained largely elusive. PMM2-CDG is caused by deficiency of the PMM PR:PROJECT_SUMMARY enzyme and results in a depletion of mannose-1-P and guanosine diphosphate PR:PROJECT_SUMMARY mannose (GDP-mannose), which is essential for glycosylation. Here, we show that PR:PROJECT_SUMMARY apart from glycosylation abnormalities, PMM2 deficiency also leads to changes in PR:PROJECT_SUMMARY intracellular glucose flux, which results in an increase in intracellular PR:PROJECT_SUMMARY polyols. PR:INSTITUTE Mayo Clinic PR:LAST_NAME Radenkovic PR:FIRST_NAME Silvia PR:ADDRESS 200 2nd Ave SW Rochester MN PR:EMAIL radenkovic.silvia@mayo.edu PR:PHONE 507(77) 6-6107 PR:FUNDING_SOURCE NIH, KU Leuven #STUDY ST:STUDY_TITLE Metabolomic profiling of PMM2-CDG patient fibroblasts ST:STUDY_SUMMARY Abnormal polyol metabolism has been predominantly associated with diabetes, ST:STUDY_SUMMARY where excess glucose is converted to sorbitol by aldose reductase (AR). ST:STUDY_SUMMARY Recently, abnormal polyol metabolism has also been implicated in ST:STUDY_SUMMARY phosphomannomutase 2-congenital disorder of glycosylation (PMM2-CDG), and ST:STUDY_SUMMARY epalrestat, an AR inhibitor, proposed as a potential therapy for this disorder. ST:STUDY_SUMMARY Given that the PMM enzyme is not closely connected to polyol metabolism, and, ST:STUDY_SUMMARY unlike in diabetes, PMM2-CDG does not present with hyperglycemia in blood, the ST:STUDY_SUMMARY increased polyol production, and the therapeutic mechanism of epalrestat in ST:STUDY_SUMMARY PMM2-CDG remained largely elusive. PMM2-CDG is caused by deficiency of the PMM ST:STUDY_SUMMARY enzyme and results in a depletion of mannose-1-P and guanosine diphosphate ST:STUDY_SUMMARY mannose (GDP-mannose), which is essential for glycosylation. Here, we show that ST:STUDY_SUMMARY apart from glycosylation abnormalities, PMM2 deficiency also leads to changes in ST:STUDY_SUMMARY intracellular glucose flux, which results in an increase in intracellular ST:STUDY_SUMMARY polyols. ST:INSTITUTE Mayo Clinic ST:LAST_NAME Radenkovic ST:FIRST_NAME Silvia ST:ADDRESS 200 2nd Ave SW Rochester MN, USA ST:EMAIL radenkovic.silvia@mayo.edu ST:PHONE 507(77) 6-6107 #SUBJECT SU:SUBJECT_TYPE Cultured cells SU:SUBJECT_SPECIES Homo sapiens SU:TAXONOMY_ID 9606 SU:GENOTYPE_STRAIN WT/PMM2-CDG SU:AGE_OR_AGE_RANGE 5-45 SU:GENDER Male and female #SUBJECT_SAMPLE_FACTORS: SUBJECT(optional)[tab]SAMPLE[tab]FACTORS(NAME:VALUE pairs separated by |)[tab]Raw file names and additional sample data SUBJECT_SAMPLE_FACTORS P1 SR01 (1) Genotype:PMM2-CDG Cell type=Skin fibroblasts; RAW_FILE_NAME=SR01 (1); Medium=5.5mM 13C GLU SUBJECT_SAMPLE_FACTORS P1 SR01 (2) Genotype:PMM2-CDG Cell type=Skin fibroblasts; RAW_FILE_NAME=SR01 (2); Medium=5.5mM 13C GLU SUBJECT_SAMPLE_FACTORS P1 SR01 (3) Genotype:PMM2-CDG Cell type=Skin fibroblasts; RAW_FILE_NAME=SR01 (3); Medium=5.5mM 13C GLU SUBJECT_SAMPLE_FACTORS P1 SR01 (4) Genotype:PMM2-CDG Cell type=Skin fibroblasts; RAW_FILE_NAME=SR01 (4); Medium=5.5mM GLU + SUBJECT_SAMPLE_FACTORS P2 SR01 (5) Genotype:PMM2-CDG Cell type=Skin fibroblasts; RAW_FILE_NAME=SR01 (5); Medium=5.5mM 13C GLU SUBJECT_SAMPLE_FACTORS P2 SR01 (6) Genotype:PMM2-CDG Cell type=Skin fibroblasts; RAW_FILE_NAME=SR01 (6); Medium=5.5mM 13C GLU SUBJECT_SAMPLE_FACTORS P2 SR01 (7) Genotype:PMM2-CDG Cell type=Skin fibroblasts; RAW_FILE_NAME=SR01 (7); Medium=5.5mM 13C GLU SUBJECT_SAMPLE_FACTORS P2 SR01 (8) Genotype:PMM2-CDG Cell type=Skin fibroblasts; RAW_FILE_NAME=SR01 (8); Medium=5.5mM GLU + SUBJECT_SAMPLE_FACTORS P4 SR01 (9) Genotype:PMM2-CDG Cell type=Skin fibroblasts; RAW_FILE_NAME=SR01 (9); Medium=5.5mM 13C GLU SUBJECT_SAMPLE_FACTORS P4 SR01 (10) Genotype:PMM2-CDG Cell type=Skin fibroblasts; RAW_FILE_NAME=SR01 (10); Medium=5.5mM 13C GLU SUBJECT_SAMPLE_FACTORS P4 SR01 (11) Genotype:PMM2-CDG Cell type=Skin fibroblasts; RAW_FILE_NAME=SR01 (11); Medium=5.5mM 13C GLU SUBJECT_SAMPLE_FACTORS P4 SR01 (12) Genotype:PMM2-CDG Cell type=Skin fibroblasts; RAW_FILE_NAME=SR01 (12); Medium=5.5mM GLU + SUBJECT_SAMPLE_FACTORS P5 SR01 (13) Genotype:PMM2-CDG Cell type=Skin fibroblasts; RAW_FILE_NAME=SR01 (13); Medium=5.5mM 13C GLU SUBJECT_SAMPLE_FACTORS P5 SR01 (14) Genotype:PMM2-CDG Cell type=Skin fibroblasts; RAW_FILE_NAME=SR01 (14); Medium=5.5mM 13C GLU SUBJECT_SAMPLE_FACTORS P5 SR01 (15) Genotype:PMM2-CDG Cell type=Skin fibroblasts; RAW_FILE_NAME=SR01 (15); Medium=5.5mM 13C GLU SUBJECT_SAMPLE_FACTORS P5 SR01 (16) Genotype:PMM2-CDG Cell type=Skin fibroblasts; RAW_FILE_NAME=SR01 (16); Medium=5.5mM GLU + SUBJECT_SAMPLE_FACTORS P6 SR01 (17) Genotype:PMM2-CDG Cell type=Skin fibroblasts; RAW_FILE_NAME=SR01 (17); Medium=5.5mM 13C GLU SUBJECT_SAMPLE_FACTORS P6 SR01 (18) Genotype:PMM2-CDG Cell type=Skin fibroblasts; RAW_FILE_NAME=SR01 (18); Medium=5.5mM 13C GLU SUBJECT_SAMPLE_FACTORS P6 SR01 (19) Genotype:PMM2-CDG Cell type=Skin fibroblasts; RAW_FILE_NAME=SR01 (19); Medium=5.5mM 13C GLU SUBJECT_SAMPLE_FACTORS P6 SR01 (20) Genotype:PMM2-CDG Cell type=Skin fibroblasts; RAW_FILE_NAME=SR01 (20); Medium=5.5mM GLU + SUBJECT_SAMPLE_FACTORS C1 SR01 (21) Genotype:WT Cell type=Skin fibroblasts; RAW_FILE_NAME=SR01 (21); Medium=5.5mM 13C GLU SUBJECT_SAMPLE_FACTORS C1 SR01 (22) Genotype:WT Cell type=Skin fibroblasts; RAW_FILE_NAME=SR01 (22); Medium=5.5mM 13C GLU SUBJECT_SAMPLE_FACTORS C1 SR01 (23) Genotype:WT Cell type=Skin fibroblasts; RAW_FILE_NAME=SR01 (23); Medium=5.5mM 13C GLU SUBJECT_SAMPLE_FACTORS C1 SR01 (24) Genotype:WT Cell type=Skin fibroblasts; RAW_FILE_NAME=SR01 (24); Medium=5.5mM GLU + SUBJECT_SAMPLE_FACTORS C3 SR01 (25) Genotype:WT Cell type=Skin fibroblasts; RAW_FILE_NAME=SR01 (25); Medium=5.5mM 13C GLU SUBJECT_SAMPLE_FACTORS C3 SR01 (26) Genotype:WT Cell type=Skin fibroblasts; RAW_FILE_NAME=SR01 (26); Medium=5.5mM 13C GLU SUBJECT_SAMPLE_FACTORS C3 SR01 (27) Genotype:WT Cell type=Skin fibroblasts; RAW_FILE_NAME=SR01 (27); Medium=5.5mM 13C GLU SUBJECT_SAMPLE_FACTORS C3 SR01 (28) Genotype:WT Cell type=Skin fibroblasts; RAW_FILE_NAME=SR01 (28); Medium=5.5mM GLU + SUBJECT_SAMPLE_FACTORS C4 SR01 (29) Genotype:WT Cell type=Skin fibroblasts; RAW_FILE_NAME=SR01 (29); Medium=5.5mM 13C GLU SUBJECT_SAMPLE_FACTORS C4 SR01 (30) Genotype:WT Cell type=Skin fibroblasts; RAW_FILE_NAME=SR01 (30); Medium=5.5mM 13C GLU SUBJECT_SAMPLE_FACTORS C4 SR01 (31) Genotype:WT Cell type=Skin fibroblasts; RAW_FILE_NAME=SR01 (31); Medium=5.5mM 13C GLU SUBJECT_SAMPLE_FACTORS C4 SR01 (32) Genotype:WT Cell type=Skin fibroblasts; RAW_FILE_NAME=SR01 (32); Medium=5.5mM GLU + SUBJECT_SAMPLE_FACTORS P1 SR02 (1) Genotype:PMM2-CDG Cell type=Skin fibroblasts; RAW_FILE_NAME=SR02 (1); Medium=5.5 mM 13 GLU SUBJECT_SAMPLE_FACTORS P1 SR02 (2) Genotype:PMM2-CDG Cell type=Skin fibroblasts; RAW_FILE_NAME=SR02 (2); Medium=5.5 mM 13 GLU SUBJECT_SAMPLE_FACTORS P1 SR02 (3) Genotype:PMM2-CDG Cell type=Skin fibroblasts; RAW_FILE_NAME=SR02 (3); Medium=5.5 mM 13 GLU SUBJECT_SAMPLE_FACTORS P1 SR02 (4) Genotype:PMM2-CDG Cell type=Skin fibroblasts; RAW_FILE_NAME=SR02 (4); Medium=5.5 mM 12 GLU SUBJECT_SAMPLE_FACTORS P2 SR02 (5) Genotype:PMM2-CDG Cell type=Skin fibroblasts; RAW_FILE_NAME=SR02 (5); Medium=5.5 mM 13 GLU SUBJECT_SAMPLE_FACTORS P2 SR02 (6) Genotype:PMM2-CDG Cell type=Skin fibroblasts; RAW_FILE_NAME=SR02 (6); Medium=5.5 mM 13 GLU SUBJECT_SAMPLE_FACTORS P2 SR02 (7) Genotype:PMM2-CDG Cell type=Skin fibroblasts; RAW_FILE_NAME=SR02 (7); Medium=5.5 mM 13 GLU SUBJECT_SAMPLE_FACTORS P2 SR02 (8) Genotype:PMM2-CDG Cell type=Skin fibroblasts; RAW_FILE_NAME=SR02 (8); Medium=5.5 mM 12 GLU SUBJECT_SAMPLE_FACTORS P3 SR02 (9) Genotype:PMM2-CDG Cell type=Skin fibroblasts; RAW_FILE_NAME=SR02 (9); Medium=5.5 mM 13 GLU SUBJECT_SAMPLE_FACTORS P3 SR02 (10) Genotype:PMM2-CDG Cell type=Skin fibroblasts; RAW_FILE_NAME=SR02 (10); Medium=5.5 mM 13 GLU SUBJECT_SAMPLE_FACTORS P3 SR02 (11) Genotype:PMM2-CDG Cell type=Skin fibroblasts; RAW_FILE_NAME=SR02 (11); Medium=5.5 mM 13 GLU SUBJECT_SAMPLE_FACTORS P3 SR02 (12) Genotype:PMM2-CDG Cell type=Skin fibroblasts; RAW_FILE_NAME=SR02 (12); Medium=5.5 mM 12 GLU SUBJECT_SAMPLE_FACTORS C2 SR02 (13) Genotype:WT Cell type=Skin fibroblasts; RAW_FILE_NAME=SR02 (13); Medium=5.5 mM 13 GLU SUBJECT_SAMPLE_FACTORS C2 SR02 (14) Genotype:WT Cell type=Skin fibroblasts; RAW_FILE_NAME=SR02 (14); Medium=5.5 mM 13 GLU SUBJECT_SAMPLE_FACTORS C2 SR02 (15) Genotype:WT Cell type=Skin fibroblasts; RAW_FILE_NAME=SR02 (15); Medium=5.5 mM 13 GLU SUBJECT_SAMPLE_FACTORS C2 SR02 (16) Genotype:WT Cell type=Skin fibroblasts; RAW_FILE_NAME=SR02 (16); Medium=5.5 mM 12 GLU SUBJECT_SAMPLE_FACTORS C3 SR02 (17) Genotype:WT Cell type=Skin fibroblasts; RAW_FILE_NAME=SR02 (17); Medium=5.5 mM 13 GLU SUBJECT_SAMPLE_FACTORS C3 SR02 (18) Genotype:WT Cell type=Skin fibroblasts; RAW_FILE_NAME=SR02 (18); Medium=5.5 mM 13 GLU SUBJECT_SAMPLE_FACTORS C3 SR02 (19) Genotype:WT Cell type=Skin fibroblasts; RAW_FILE_NAME=SR02 (19); Medium=5.5 mM 13 GLU SUBJECT_SAMPLE_FACTORS C3 SR02 (20) Genotype:WT Cell type=Skin fibroblasts; RAW_FILE_NAME=SR02 (20); Medium=5.5 mM 12 GLU SUBJECT_SAMPLE_FACTORS P1 SR03 (1) Genotype:PMM2-CDG Cell type=Skin fibroblasts; RAW_FILE_NAME=SR03 (1); Medium=5.5 mM 13 GLU SUBJECT_SAMPLE_FACTORS P1 SR03 (2) Genotype:PMM2-CDG Cell type=Skin fibroblasts; RAW_FILE_NAME=SR03 (2); Medium=5.5 mM 13 GLU SUBJECT_SAMPLE_FACTORS P1 SR03 (3) Genotype:PMM2-CDG Cell type=Skin fibroblasts; RAW_FILE_NAME=SR03 (3); Medium=5.5 mM 13 GLU SUBJECT_SAMPLE_FACTORS P1 SR03 (4) Genotype:PMM2-CDG Cell type=Skin fibroblasts; RAW_FILE_NAME=SR03 (4); Medium=5.5 mM 12 GLU SUBJECT_SAMPLE_FACTORS P2 SR03 (5) Genotype:PMM2-CDG Cell type=Skin fibroblasts; RAW_FILE_NAME=SR03 (5); Medium=5.5 mM 13 GLU SUBJECT_SAMPLE_FACTORS P2 SR03 (6) Genotype:PMM2-CDG Cell type=Skin fibroblasts; RAW_FILE_NAME=SR03 (6); Medium=5.5 mM 13 GLU SUBJECT_SAMPLE_FACTORS P2 SR03 (7) Genotype:PMM2-CDG Cell type=Skin fibroblasts; RAW_FILE_NAME=SR03 (7); Medium=5.5 mM 13 GLU SUBJECT_SAMPLE_FACTORS P2 SR03 (8) Genotype:PMM2-CDG Cell type=Skin fibroblasts; RAW_FILE_NAME=SR03 (8); Medium=5.5 mM 12 GLU SUBJECT_SAMPLE_FACTORS P3 SR03 (9) Genotype:PMM2-CDG Cell type=Skin fibroblasts; RAW_FILE_NAME=SR03 (9); Medium=5.5 mM 13 GLU SUBJECT_SAMPLE_FACTORS P3 SR03 (10) Genotype:PMM2-CDG Cell type=Skin fibroblasts; RAW_FILE_NAME=SR03 (10); Medium=5.5 mM 13 GLU SUBJECT_SAMPLE_FACTORS P3 SR03 (11) Genotype:PMM2-CDG Cell type=Skin fibroblasts; RAW_FILE_NAME=SR03 (11); Medium=5.5 mM 13 GLU SUBJECT_SAMPLE_FACTORS P3 SR03 (12) Genotype:PMM2-CDG Cell type=Skin fibroblasts; RAW_FILE_NAME=SR03 (12); Medium=5.5 mM 12 GLU SUBJECT_SAMPLE_FACTORS C5 SR03 (13) Genotype:WT Cell type=Skin fibroblasts; RAW_FILE_NAME=SR03 (13); Medium=5.5 mM 13 GLU SUBJECT_SAMPLE_FACTORS C5 SR03 (14) Genotype:WT Cell type=Skin fibroblasts; RAW_FILE_NAME=SR03 (14); Medium=5.5 mM 13 GLU SUBJECT_SAMPLE_FACTORS C5 SR03 (15) Genotype:WT Cell type=Skin fibroblasts; RAW_FILE_NAME=SR03 (15); Medium=5.5 mM 13 GLU SUBJECT_SAMPLE_FACTORS C5 SR03 (16) Genotype:WT Cell type=Skin fibroblasts; RAW_FILE_NAME=SR03 (16); Medium=5.5 mM 12 GLU SUBJECT_SAMPLE_FACTORS C3 SR03 (17) Genotype:WT Cell type=Skin fibroblasts; RAW_FILE_NAME=SR03 (17); Medium=5.5 mM 13 GLU SUBJECT_SAMPLE_FACTORS C3 SR03 (18) Genotype:WT Cell type=Skin fibroblasts; RAW_FILE_NAME=SR03 (18); Medium=5.5 mM 13 GLU SUBJECT_SAMPLE_FACTORS C3 SR03 (19) Genotype:WT Cell type=Skin fibroblasts; RAW_FILE_NAME=SR03 (19); Medium=5.5 mM 13 GLU SUBJECT_SAMPLE_FACTORS C3 SR03 (20) Genotype:WT Cell type=Skin fibroblasts; RAW_FILE_NAME=SR03 (20); Medium=5.5 mM 12 GLU #COLLECTION CO:COLLECTION_SUMMARY Cells were washed with PBS, cells were incubated with extraction buffer for 2min CO:COLLECTION_SUMMARY before scraping and transferring to a fresh eppendorf. Samples were precipitated CO:COLLECTION_SUMMARY overnight at -80, then they were centrifuged at max rpm, 20min, 4 degrees C and CO:COLLECTION_SUMMARY supernatant transferred to an M/S vial. CO:SAMPLE_TYPE Fibroblasts CO:STORAGE_CONDITIONS -80℃ #TREATMENT TR:TREATMENT_SUMMARY Cells were grown in medium with 13c glucose or 12c glucose #SAMPLEPREP SP:SAMPLEPREP_SUMMARY Metabolites were extracted with 80% Methanol and IS (d27 myristic acid) SP:PROCESSING_STORAGE_CONDITIONS -80℃ SP:EXTRACT_STORAGE -80℃ #CHROMATOGRAPHY CH:CHROMATOGRAPHY_SUMMARY C18 iP REVERSE PHASE CH:CHROMATOGRAPHY_TYPE Reversed phase CH:INSTRUMENT_NAME Waters Acquity CH:COLUMN_NAME Waters ACQUITY UPLC HSS T3 (150 x 2.1mm,1.8um) CH:SOLVENT_A 100% water; 10mM tributylamine; 15mM acetic acid CH:SOLVENT_B 100% methanol CH:FLOW_GRADIENT The gradient started with 5% of solvent B and 95% solvent A and remained at 5% B CH:FLOW_GRADIENT until 2 min post injection. A linear gradient to 37% B was carried out until 7 CH:FLOW_GRADIENT min and increased to 41% until 14 min. Between 14 and 26 minutes the gradient CH:FLOW_GRADIENT increased to 95% of B and remained at 95% B for 4 minutes. At 30 min the CH:FLOW_GRADIENT gradient returned to 5% B. The chromatography was stopped at 40 min CH:FLOW_RATE 0.25 ml/min CH:COLUMN_TEMPERATURE 40 #ANALYSIS AN:ANALYSIS_TYPE MS #MS MS:INSTRUMENT_NAME Thermo Q Exactive Orbitrap MS:INSTRUMENT_TYPE Orbitrap MS:MS_TYPE ESI MS:ION_MODE NEGATIVE MS:MS_COMMENTS El-Maven polly, ThermoFisher Xcalibur, Metabolites were annotated based on the MS:MS_COMMENTS in-house metabolite library- elution time and m/z values #MS_METABOLITE_DATA MS_METABOLITE_DATA:UNITS AUC MS_METABOLITE_DATA_START Samples SR01 (1) SR01 (2) SR01 (3) SR01 (4) SR01 (5) SR01 (6) SR01 (7) SR01 (8) SR01 (9) SR01 (10) SR01 (11) SR01 (12) SR01 (13) SR01 (14) SR01 (15) SR01 (16) SR01 (17) SR01 (18) SR01 (19) SR01 (20) SR01 (21) SR01 (22) SR01 (23) SR01 (24) SR01 (25) SR01 (26) SR01 (27) SR01 (28) SR01 (29) SR01 (30) SR01 (31) SR01 (32) SR02 (1) SR02 (2) SR02 (3) SR02 (4) SR02 (5) SR02 (6) SR02 (7) SR02 (8) SR02 (9) SR02 (10) SR02 (11) SR02 (12) SR02 (13) SR02 (14) SR02 (15) SR02 (16) SR02 (17) SR02 (18) SR02 (19) SR02 (20) SR03 (1) SR03 (2) SR03 (3) SR03 (4) SR03 (5) SR03 (6) SR03 (7) SR03 (8) SR03 (9) SR03 (10) SR03 (11) SR03 (12) SR03 (13) SR03 (14) SR03 (15) SR03 (16) SR03 (17) SR03 (18) SR03 (19) SR03 (20) Factors Genotype:PMM2-CDG Genotype:PMM2-CDG Genotype:PMM2-CDG Genotype:PMM2-CDG Genotype:PMM2-CDG Genotype:PMM2-CDG Genotype:PMM2-CDG Genotype:PMM2-CDG Genotype:PMM2-CDG Genotype:PMM2-CDG Genotype:PMM2-CDG Genotype:PMM2-CDG Genotype:PMM2-CDG Genotype:PMM2-CDG Genotype:PMM2-CDG Genotype:PMM2-CDG Genotype:PMM2-CDG Genotype:PMM2-CDG Genotype:PMM2-CDG Genotype:PMM2-CDG Genotype:WT Genotype:WT Genotype:WT Genotype:WT Genotype:WT Genotype:WT Genotype:WT Genotype:WT Genotype:WT Genotype:WT Genotype:WT Genotype:WT Genotype:PMM2-CDG Genotype:PMM2-CDG Genotype:PMM2-CDG Genotype:PMM2-CDG Genotype:PMM2-CDG Genotype:PMM2-CDG Genotype:PMM2-CDG Genotype:PMM2-CDG Genotype:PMM2-CDG Genotype:PMM2-CDG Genotype:PMM2-CDG Genotype:PMM2-CDG Genotype:WT Genotype:WT Genotype:WT Genotype:WT Genotype:WT Genotype:WT Genotype:WT Genotype:WT Genotype:PMM2-CDG Genotype:PMM2-CDG Genotype:PMM2-CDG Genotype:PMM2-CDG Genotype:PMM2-CDG Genotype:PMM2-CDG Genotype:PMM2-CDG Genotype:PMM2-CDG Genotype:PMM2-CDG Genotype:PMM2-CDG Genotype:PMM2-CDG Genotype:PMM2-CDG Genotype:WT Genotype:WT Genotype:WT Genotype:WT Genotype:WT Genotype:WT Genotype:WT Genotype:WT Sorbitol 7.62E+07 7.76E+07 9.96E+07 1.08E+08 1.09E+08 9.76E+07 1.20E+08 1.23E+08 1.18E+08 9.75E+07 1.23E+08 1.26E+08 1.29E+08 9.83E+07 1.00E+08 1.10E+08 1.20E+08 1.04E+08 9.63E+07 1.23E+08 1.16E+08 1.00E+08 1.15E+08 1.23E+08 1.35E+08 8.62E+07 9.40E+07 1.04E+08 1.13E+08 9.55E+07 1.01E+08 1.15E+08 1.20E+08 21444749 31418179 38778389 34113777 31572489 35110003 34505794 31363448 32547115 42107360 31611473 26006661 28299994 34991180 33076376 35854777 35909283 43402918 55537612 2.95E+07 2.71E+07 3.67E+07 3.47E+07 1.82E+07 2.36E+07 2.35E+07 2.43E+07 2.37E+07 2.41E+07 2.41E+07 2.62E+07 3.04E+07 2.11E+07 2.69E+07 2.93E+07 1.21E+07 7.76E+06 1.63E+07 7.45E+06 MS_METABOLITE_DATA_END #METABOLITES METABOLITES_START metabolite_name parent formula M/Z HMDB ID isotopeLabel isotope count Sorbitol C6H14O6 181.07164 HMDB00247 C12 PARENT m0-6 METABOLITES_END #END