Metabolism, Inborn ErrorsEndoplasmic ReticulumLipid MetabolismLipidsAmino Acid Metabolism, Inborn ErrorsEndoplasmic Reticulum StressEndoplasmic Reticulum, RoughSarcoplasmic ReticulumLipid Metabolism, Inborn ErrorsEndoplasmic Reticulum, SmoothGolgi ApparatusLiverLipid ASteroid Metabolism, Inborn ErrorsMolecular Sequence DataNeonatal ScreeningPurine-Pyrimidine Metabolism, Inborn ErrorsMembrane ProteinsLipid Metabolism DisordersUnfolded Protein ResponseMutationCarbohydrate Metabolism, Inborn ErrorsFatty AcidsProtein TransportEnergy MetabolismCholesterolLipid BilayersAmino Acid SequenceTriglyceridesCalciumMolecular ChaperonesCell LineUrea Cycle Disorders, InbornIntracellular MembranesBiological TransportBrain Diseases, Metabolic, InbornSarcoplasmic Reticulum Calcium-Transporting ATPasesTunicamycinCell MembraneCalcium-Transporting ATPasesMicrosomesTranscription Factor CHOPCalnexinArgininosuccinic AciduriaModels, BiologicalCalreticulinMicroscopy, ElectronThapsigarginHyperammonemiaLipid PeroxidationProtein FoldingBrefeldin ACells, CulturedPhospholipidsGlucoseActivating Transcription Factor 6GlycosylationKineticsHeat-Shock ProteinsBase SequenceProtein Processing, Post-TranslationalTime FactorsPhenylketonuriasSignal TransductionSmith-Lemli-Opitz SyndromeRNA, MessengerCaspase 12Protein Sorting SignalsSubcellular FractionsStress, PhysiologicalGene Expression RegulationProtein BindingMitochondriaCarrier ProteinsOxidation-ReductionCytosolCOP-Coated VesiclesHomeostasisCricetinaeSaccharomyces cerevisiaeMicrosomes, LiverMice, Inbred C57BLInfant, NewbornProtein Disulfide-IsomerasesMice, KnockoutAdipose TissueInsulinMetabolic DiseasesProtein Structure, TertiaryTransfectionReceptors, Cytoplasmic and NuclearCarnitineRefractive ErrorsMicroscopy, Fluorescencealpha-GalactosidaseCalcium SignalingHomogentisate 1,2-DioxygenaseRabbitsSaccharomyces cerevisiae ProteinsCOS Cells