#METABOLOMICS WORKBENCH spinelli_lab_20240520_111432 DATATRACK_ID:4850 STUDY_ID:ST003218 ANALYSIS_ID:AN005276 PROJECT_ID:PR001988 VERSION 1 CREATED_ON May 22, 2024, 7:58 pm #PROJECT PR:PROJECT_TITLE Rhodoquinone is an Electron Carrier in the Mammalian Electron Transport Chain PR:PROJECT_SUMMARY Ubiquinone (UQ), the only known electron carrier in the mammalian electron PR:PROJECT_SUMMARY transport chain (ETC), delivers electrons to both oxygen (O2) and fumarate as PR:PROJECT_SUMMARY terminal electron acceptors. As fumarate has a lower reduction potential than PR:PROJECT_SUMMARY UQ, fumarate reduction is only thermodynamically favorable when ubiquinol, the PR:PROJECT_SUMMARY reduced form of UQ, accumulates. Paradoxically, some tissues reduce fumarate PR:PROJECT_SUMMARY without ubiquinol buildup, suggesting another mechanism enables fumarate PR:PROJECT_SUMMARY reduction in mammals. Here, we identify rhodoquinone (RQ), a novel component of PR:PROJECT_SUMMARY the mammalian ETC that carries electrons to fumarate, instead of O2, as the PR:PROJECT_SUMMARY terminal electron acceptor. RQ, which is undetectable in cultured mammalian PR:PROJECT_SUMMARY cells, is enriched in mitochondria from mouse and human tissues that catalyze PR:PROJECT_SUMMARY high levels of fumarate reduction. UQ and RQ-directed ETC circuits support PR:PROJECT_SUMMARY unique programs of mitochondrial function. Through expression of a bacterial PR:PROJECT_SUMMARY enzyme that converts UQ into RQ and development of a novel RQ analog, we PR:PROJECT_SUMMARY demonstrate that reprogramming the mammalian ETC from the UQ to RQ circuit PR:PROJECT_SUMMARY renders cells highly resistant to hypoxia exposure in vitro and in vivo. Thus, PR:PROJECT_SUMMARY we establish RQ as a fundamental component of the mammalian ETC and unveil that PR:PROJECT_SUMMARY reprogramming the ETC to the RQ-circuit is a tractable strategy to ameliorate PR:PROJECT_SUMMARY hypoxia-related conditions. PR:INSTITUTE UMass Chan Medical School PR:LAST_NAME UMass Chan PR:FIRST_NAME Spinelli Lab PR:ADDRESS 55 Lake Avenue North, Worcester, Massachusetts, 01605, USA PR:EMAIL spinellilab@gmail.com PR:PHONE (508) 856-8989 ext. 68148 #STUDY ST:STUDY_TITLE Glutamine Tracing Assay in WT and SDHB KO 143B cells Treated with HKJS003 ST:STUDY_SUMMARY WT and succinate dehydrogenase iron sulfur subunit B(SDHB) KO 143B cells were ST:STUDY_SUMMARY treated with a small molecule analog of Rhodoquinone, HKJS003. Glutamine tracing ST:STUDY_SUMMARY was used to determine the fumarate reduction and succinate oxidation of the ST:STUDY_SUMMARY treated cells. Other metabolites such as ATP,ADP,AMP,Glutathione, Oxidized ST:STUDY_SUMMARY glutathione, UTP, lactic acid, and pyruvic acid were also measured. ST:INSTITUTE UMass Chan Medical School ST:LAST_NAME UMass Chan ST:FIRST_NAME Spinelli Lab ST:ADDRESS 55 Lake Avenue North, Worcester, Massachusetts, 01605, USA ST:EMAIL spinellilab@gmail.com ST:PHONE (508) 856-8989 ext. 68148 #SUBJECT SU:SUBJECT_TYPE Cultured cells SU:SUBJECT_SPECIES Homo sapiens SU:TAXONOMY_ID 9606 #FACTORS #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 - WT 1 Sample source:Bone sarcoma cells | Genotype:WT | Treatment:DMSO Cell line=143B; RAW_FILE_NAME(File Name )=JV121_WT_vs_SDHBKO_003_01_20240402125032.raw SUBJECT_SAMPLE_FACTORS - WT 2 Sample source:Bone sarcoma cells | Genotype:WT | Treatment:DMSO Cell line=143B; RAW_FILE_NAME(File Name )=JV121_WT_vs_SDHBKO_003_02.raw SUBJECT_SAMPLE_FACTORS - WT 3 Sample source:Bone sarcoma cells | Genotype:WT | Treatment:DMSO Cell line=143B; RAW_FILE_NAME(File Name )=JV121_WT_vs_SDHBKO_003_03.raw SUBJECT_SAMPLE_FACTORS - WT 25nM 003 1 Sample source:Bone sarcoma cells | Genotype:WT | Treatment:25nM HKJS003 Cell line=143B; RAW_FILE_NAME(File Name )=JV121_WT_vs_SDHBKO_003_04.raw SUBJECT_SAMPLE_FACTORS - WT 25nM 003 2 Sample source:Bone sarcoma cells | Genotype:WT | Treatment:25nM HKJS003 Cell line=143B; RAW_FILE_NAME(File Name )=JV121_WT_vs_SDHBKO_003_05.raw SUBJECT_SAMPLE_FACTORS - WT 25nM 003 3 Sample source:Bone sarcoma cells | Genotype:WT | Treatment:25nM HKJS003 Cell line=143B; RAW_FILE_NAME(File Name )=JV121_WT_vs_SDHBKO_003_06.raw SUBJECT_SAMPLE_FACTORS - WT 100nM 003 1 Sample source:Bone sarcoma cells | Genotype:WT | Treatment:100nM HKJS003 Cell line=143B; RAW_FILE_NAME(File Name )=JV121_WT_vs_SDHBKO_003_07.raw SUBJECT_SAMPLE_FACTORS - WT 100nM 003 2 Sample source:Bone sarcoma cells | Genotype:WT | Treatment:100nM HKJS003 Cell line=143B; RAW_FILE_NAME(File Name )=JV121_WT_vs_SDHBKO_003_08.raw SUBJECT_SAMPLE_FACTORS - WT 100nM 003 3 Sample source:Bone sarcoma cells | Genotype:WT | Treatment:100nM HKJS003 Cell line=143B; RAW_FILE_NAME(File Name )=JV121_WT_vs_SDHBKO_003_09.raw SUBJECT_SAMPLE_FACTORS - SDHB KO 1 Sample source:Bone sarcoma cells | Genotype:SDHB KO | Treatment:DMSO Cell line=143B; RAW_FILE_NAME(File Name )=JV121_WT_vs_SDHBKO_003_10.raw SUBJECT_SAMPLE_FACTORS - SDHB KO 2 Sample source:Bone sarcoma cells | Genotype:SDHB KO | Treatment:DMSO Cell line=143B; RAW_FILE_NAME(File Name )=JV121_WT_vs_SDHBKO_003_11.raw SUBJECT_SAMPLE_FACTORS - SDHB KO 3 Sample source:Bone sarcoma cells | Genotype:SDHB KO | Treatment:DMSO Cell line=143B; RAW_FILE_NAME(File Name )=JV121_WT_vs_SDHBKO_003_12.raw SUBJECT_SAMPLE_FACTORS - SDHB KO 25nM 003 1 Sample source:Bone sarcoma cells | Genotype:SDHB KO | Treatment:25nM HKJS003 Cell line=143B; RAW_FILE_NAME(File Name )=JV121_WT_vs_SDHBKO_003_13.raw SUBJECT_SAMPLE_FACTORS - SDHB KO 25nM 003 2 Sample source:Bone sarcoma cells | Genotype:SDHB KO | Treatment:25nM HKJS003 Cell line=143B; RAW_FILE_NAME(File Name )=JV121_WT_vs_SDHBKO_003_14.raw SUBJECT_SAMPLE_FACTORS - SDHB KO 25nM 003 3 Sample source:Bone sarcoma cells | Genotype:SDHB KO | Treatment:25nM HKJS003 Cell line=143B; RAW_FILE_NAME(File Name )=JV121_WT_vs_SDHBKO_003_15.raw SUBJECT_SAMPLE_FACTORS - SDHB KO 100nM 003 1 Sample source:Bone sarcoma cells | Genotype:SDHB KO | Treatment:100nM HKJS003 Cell line=143B; RAW_FILE_NAME(File Name )=JV121_WT_vs_SDHBKO_003_16.raw SUBJECT_SAMPLE_FACTORS - SDHB KO 100nM 003 2 Sample source:Bone sarcoma cells | Genotype:SDHB KO | Treatment:100nM HKJS003 Cell line=143B; RAW_FILE_NAME(File Name )=JV121_WT_vs_SDHBKO_003_17.raw SUBJECT_SAMPLE_FACTORS - SDHB KO 100nM 003 3 Sample source:Bone sarcoma cells | Genotype:SDHB KO | Treatment:100nM HKJS003 Cell line=143B; RAW_FILE_NAME(File Name )=JV121_WT_vs_SDHBKO_003_18.raw #COLLECTION CO:COLLECTION_SUMMARY Media was aspirated from the plates and then the cells were washed with 1x PBS CO:COLLECTION_SUMMARY twice. The plate was then transferred to dry ice and 500 µL of 80% HPLC-grade CO:COLLECTION_SUMMARY methanol (Sigma) 20% HPLC-grade water (Sigma) was added to each well. The wells CO:COLLECTION_SUMMARY were placed in a -80 freezer to incubate for 15 minutes. The plates are taken CO:COLLECTION_SUMMARY out of the freezer one at a time and placed back on dry ice. The cells were then CO:COLLECTION_SUMMARY scraped and transferred to a new tube. CO:SAMPLE_TYPE Cultured cells #TREATMENT TR:TREATMENT_SUMMARY The 143B cell line were cultured in Dulbecco's Modified Eagle Medium (DMEM) TR:TREATMENT_SUMMARY (ThermoFisher) supplemented with 10% Heat Inactivated Fetal Bovine Serum TR:TREATMENT_SUMMARY (ThermoFisher) and 1% penicillin and streptomycin (ThermoFisher), and 100 µg/mL TR:TREATMENT_SUMMARY uridine (Sigma). Cells were kept in a 37°C incubator (Baker Ruskinn) held at 5% TR:TREATMENT_SUMMARY CO2 and 90% relative humidity.Cells were then supplemented with either DMSO or TR:TREATMENT_SUMMARY HKJS003. For HKJS003, 20µL or 5µL of 100µM stock was added to 2mL of media TR:TREATMENT_SUMMARY for a final concentration of 100nM or 25nM. For DMSO, 20µL of a diluted 1:1000 TR:TREATMENT_SUMMARY DMSO was added to 2mL of media. The cells were incubated for 5-7 of days to TR:TREATMENT_SUMMARY proliferate and every 2 days the media was refreshed with the relevant TR:TREATMENT_SUMMARY treatments. #SAMPLEPREP SP:SAMPLEPREP_SUMMARY Once cells were collected into Eppendorf tubes, each well was washed with an SP:SAMPLEPREP_SUMMARY additional 300 µL of 80% HPLC-grade methanol (Sigma) 20% HPLC-grade water SP:SAMPLEPREP_SUMMARY (Sigma) and collected into the same tube as the initial lysis. The samples were SP:SAMPLEPREP_SUMMARY then vortexed at 4° C for 10 minutes and centrifuged at 21,300 x g for 10 SP:SAMPLEPREP_SUMMARY minutes at 4° C. Supernatants were transferred to a new tube and dried down in SP:SAMPLEPREP_SUMMARY a Refrigerated CentriVap Benchtop Vacuum Concentrator connected to a SP:SAMPLEPREP_SUMMARY CentriVap-105 Cold Trap (Labconco). After being dried down, pellets were stored SP:SAMPLEPREP_SUMMARY in a -20° C freezer until ready to run on the polar LC-MS method. #CHROMATOGRAPHY CH:CHROMATOGRAPHY_SUMMARY 20 min, 80% to 20% B; 0.5 min, 20% to 80% B; 7.5min, 80% B CH:CHROMATOGRAPHY_TYPE HILIC CH:INSTRUMENT_NAME Thermo Vanquish CH:COLUMN_NAME Merck SeQuant ZIC-HILIC (150 x 2.1mm,5um) CH:SOLVENT_A 100% water; 0.1% ammonium hydroxide; 20mM ammonium carbonate CH:SOLVENT_B 100% acetonitrile CH:FLOW_GRADIENT Gradient starts at 80% B. Subsequently, 80% to 20% B linearly in 20min; 20% to CH:FLOW_GRADIENT 80% B in 0.5min; 80% B for 7.5min CH:FLOW_RATE 0.15ml/min CH:COLUMN_TEMPERATURE 25 #ANALYSIS AN:ANALYSIS_TYPE MS #MS MS:INSTRUMENT_NAME Thermo Q Exactive Plus Orbitrap MS:INSTRUMENT_TYPE Orbitrap MS:MS_TYPE ESI MS:ION_MODE POSITIVE MS:MS_COMMENTS The mass spectrometer was set to full scan (70-1000 m/z), with the spray voltage MS:MS_COMMENTS set to 4.0 kV, heated capillary to 350°C, and the HESI probe at 30 °C. The MS:MS_COMMENTS sheath gas flow was set at 10 units, auxiliary gas at 1 units, and sweep gas MS:MS_COMMENTS flow at 1 unit. The resolution of scan was set to 70,000, AGC target to 1x106, MS:MS_COMMENTS and maximum injection time at 20 msec. Data acquired by Thermo Fisher's Xcalibur MS:MS_COMMENTS software and analyzed by their Tracefinder software. The raw files provided MS:MS_COMMENTS contain data from both positive and negative ion mode. #MS_METABOLITE_DATA MS_METABOLITE_DATA:UNITS peak area MS_METABOLITE_DATA_START Samples WT 1 WT 2 WT 3 WT 25nM 003 1 WT 25nM 003 2 WT 25nM 003 3 WT 100nM 003 1 WT 100nM 003 2 WT 100nM 003 3 SDHB KO 1 SDHB KO 2 SDHB KO 3 SDHB KO 25nM 003 1 SDHB KO 25nM 003 2 SDHB KO 25nM 003 3 SDHB KO 100nM 003 1 SDHB KO 100nM 003 2 SDHB KO 100nM 003 3 Factors Sample source:Bone sarcoma cells | Genotype:WT | Treatment:DMSO Sample source:Bone sarcoma cells | Genotype:WT | Treatment:DMSO Sample source:Bone sarcoma cells | Genotype:WT | Treatment:DMSO Sample source:Bone sarcoma cells | Genotype:WT | Treatment:25nM HKJS003 Sample source:Bone sarcoma cells | Genotype:WT | Treatment:25nM HKJS003 Sample source:Bone sarcoma cells | Genotype:WT | Treatment:25nM HKJS003 Sample source:Bone sarcoma cells | Genotype:WT | Treatment:100nM HKJS003 Sample source:Bone sarcoma cells | Genotype:WT | Treatment:100nM HKJS003 Sample source:Bone sarcoma cells | Genotype:WT | Treatment:100nM HKJS003 Sample source:Bone sarcoma cells | Genotype:SDHB KO | Treatment:DMSO Sample source:Bone sarcoma cells | Genotype:SDHB KO | Treatment:DMSO Sample source:Bone sarcoma cells | Genotype:SDHB KO | Treatment:DMSO Sample source:Bone sarcoma cells | Genotype:SDHB KO | Treatment:25nM HKJS003 Sample source:Bone sarcoma cells | Genotype:SDHB KO | Treatment:25nM HKJS003 Sample source:Bone sarcoma cells | Genotype:SDHB KO | Treatment:25nM HKJS003 Sample source:Bone sarcoma cells | Genotype:SDHB KO | Treatment:100nM HKJS003 Sample source:Bone sarcoma cells | Genotype:SDHB KO | Treatment:100nM HKJS003 Sample source:Bone sarcoma cells | Genotype:SDHB KO | Treatment:100nM HKJS003 Glutathione 292698418 328802279.1 347059528.3 373996007.1 373796867.5 294696223.6 264256565.6 369591153.2 356520964.6 489143105.5 398313414.5 464985477.2 386502434 392226487.6 513853489.9 228270296.2 393312164.7 378939255.4 Oxidized glutathione 6793757.65 5875048.016 6368457.519 8962317.544 6372007.147 3304381.612 2509261.029 5414605.288 5848973.377 11081976.32 7151053.296 7806179.079 10722281.68 8854580.138 11768190.19 3508012.008 6988012.485 7449209.352 MS_METABOLITE_DATA_END #METABOLITES METABOLITES_START metabolite_name Retention index Quantified m/z PubChem ID KEGG ID Glutathione 9.5 308.09108 124886 C00051 Oxidized glutathione 10.5 613.15924 65359 C00127 METABOLITES_END #END