Figure_6.tif (1002.03 kB)
Pantothenate metabolism.
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posted on 2014-09-29, 02:44 authored by Patricia Kuzaj, Joachim Kuhn, Ryan D. Michalek, Edward D. Karoly, Isabel Faust, Mareike Dabisch-Ruthe, Cornelius Knabbe, Doris Hendig(a) Measurements of pantothenate by LC-MS/MS in positive ionization mode (patient/control ratio:0.5, p<0.0003). (b) 3′-dephosphocoenzyme A (patient/control ratio:1.6, p<0.05) and (c) coenzyme A (patient/control ratio:1.2, p = 0.051) were detected by LC-MS/MS negative. No alterations in this metabolic pathway could be observed for siRNA-transfected cells. (d) Metabolic pathway of coenzyme A synthesis from pantothenate, including enzymatic steps of pantothenate kinases (PANK) and CoA synthase (COASY). Arrows point to the comparison of patients and healthy controls: red (significantly increased), light red (increased, by trend), green (significantly decreased).
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disease pathogenesisalterationmass spectrometrytissue mineralizationHealthy Controls Mutationsleucine dipeptidesPseudoxanthoma elasticum358 compoundsPXE pathogenesisABC transporter ABCC 6energy metabolitesPseudoxanthoma Elasticum PatientsABCC 6 deficiencyHuman Dermal Fibroblasts Derivedglycerophospholipid compositionoxidative stressguanine metabolismPXE patientsExtracellular matrix
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