Gene Ontology annotation through association of InterPro records with GO terms
Annotation inferences using phylogenetic trees
Gene Ontology annotation based on UniProtKB/Swiss-Prot Subcellular Location vocabulary mapping, accompanied by conservative changes to GO terms applied by UniProt
Gene Ontology annotation based on curation of immunofluorescence data
Automatic Gene Ontology annotation based on Rhea mapping
Combined Automated Annotation using Multiple IEA Methods
Human liver long-chain 3-hydroxyacyl-coenzyme A dehydrogenase is a multifunctional membrane-bound beta-oxidation enzyme of mitochondria.
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Purified the long-chain-specific multifunctional beta-oxidation enzyme from human liver mitochondrial membranes and showed it possesses hydratase, dehydrogenase and thiolase activities (maximal with C10-C16 substrates) that could not be separated. Did not assign activities to specific subunits; subunit assignment was later done by PMID:8135828.
Structural analysis of cDNAs for subunits of human mitochondrial fatty acid beta-oxidation trifunctional protein.
Mitochondrial trifunctional protein deficiency. Catalytic heterogeneity of the mutant enzyme in two patients.
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Assayed the trifunctional protein's hydratase, dehydrogenase and thiolase activities in fibroblasts from two LCHAD-deficiency patients, demonstrating that the disease is caused by abnormality of the trifunctional protein.
The layered structure of human mitochondrial DNA nucleoids.
Network organization of the human autophagy system.
Phosphoproteome analysis of functional mitochondria isolated from resting human muscle reveals extensive phosphorylation of inner membrane protein complexes and enzymes.
Acyl coenzyme A thioesterase Them5/Acot15 is involved in cardiolipin remodeling and fatty liver development.
Human trifunctional protein alpha links cardiolipin remodeling to beta-oxidation.
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Purified recombinant alpha subunit of TFP (alphaTFP) exhibits monolysocardiolipin acyltransferase activity, acylating MLCL to cardiolipin with linoleoyl-, oleoyl- and palmitoyl-CoA, and its expression increases incorporation of these fatty acids into CL in cells. Establishes an alpha-subunit-specific cardiolipin remodeling activity independent of the beta subunit.
Architecture of the human interactome defines protein communities and disease networks.
Cryo-EM structure of human mitochondrial trifunctional protein.
Crystal structure of human mitochondrial trifunctional protein, a fatty acid β-oxidation metabolon.
TFPa/HADHA is required for fatty acid beta-oxidation and cardiolipin re-modeling in human cardiomyocytes.
Interactome Mapping Provides a Network of Neurodegenerative Disease Proteins and Uncovers Widespread Protein Aggregation in Affected Brains.
Dual proteome-scale networks reveal cell-specific remodeling of the human interactome.
Quantitative high-confidence human mitochondrial proteome and its dynamics in cellular context.
Multimodal cell maps as a foundation for structural and functional genomics.
Molecular characterization of mitochondrial trifunctional protein deficiency: formation of the enzyme complex is important for stabilization of both alpha- and beta-subunits.
MLCL is acylated to CL by HADH (IM)
3-Oxotetradecanoyl-CoA+CoA-SH<=>Lauroyl-CoA
trans-Tetradec-2-enoyl-CoA+H2O<=>(S)-3-Hydroxytetradecanoyl-CoA
(S)-3-Hydroxytetradecanoyl-CoA+NAD<=>3-Oxotetradecanoyl-CoA+NADH+H
trans-Hexadec-2-enoyl-CoA+H2O<=>(S)-3-Hydroxyhexadecanoyl-CoA
(S)-3-Hydroxyhexadecanoyl-CoA+NAD<=>3-Oxopalmitoyl-CoA+NADH+H
3-Oxopalmitoyl-CoA+CoA-SH<=>myristoyl-CoA
3-Oxododecanoyl-CoA+CoA-SH<=>Decanoyl-CoA
3-Oxohexanoyl-CoA+CoA-SH<=>Butanoyl-CoA
3-Oxooctanoyl-CoA+CoA-SH<=>Hexanoyl-CoA
3-Oxodecanoyl-CoA+CoA-SH<=>Octanoyl-CoA