Mitochondrial trifunctional protein
Lack of mitochondrial trifunctional protein in mice causes neonatal hypoglycemia and sudden death
... Mitochondrial trifunctional protein (MTP) is a hetero-octamer of four α and four β subunits that catalyzes the final three steps of mitochondrial long chain fatty acid ...
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Common missense mutation G1528C in long chain 3 hydroxyacyl CoA dehydrogenase deficiency Characterization and expression of the mutant protein, mutation analysis on genomic DNA and chromosomal localization of the mitochondrial trifunctional protein alpha subunit gene
... Mitochondrial trifunctional protein (MTP) is a recently identified enzyme involved in mitochondrial b -oxidation, harboring long-chain enoyl-CoA hydratase, long-chain 3-hydroxyacyl-CoA ...
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Hepatitis C Virus Attenuates Mitochondrial Lipid β-Oxidation by Downregulating Mitochondrial Trifunctional-Protein Expression
... the mitochondrial trifunctional protein (MTP), an enzyme complex which catalyzes the last 3 steps of mitochon- drial lipid  -oxidation for cellular energy ...MTP protein and mRNA levels were ...
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Evaluation of earlier versus later dietary management in long-chain 3-hydroxyacyl-CoA dehydrogenase or mitochondrial trifunctional protein deficiency: a systematic review
... and mitochondrial trifunctional protein defi- ciency (MTPD) are rare autosomal recessive fatty acid β- oxidation ...the mitochondrial trifunctional protein (MTP) which is ...
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Hepatitis C virus attenuates mitochondrial lipid β-oxidation by downregulating mitochondrial trifunctional-protein expression
... on mitochondrial lipid  -oxi- dation, apart from one study suggesting that HCV might impair this process in vivo ...Since mitochondrial lipid  -oxidation yields a greater amount of ATP than carbohydrate ...
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Fatal pitfalls in newborn screening for mitochondrial trifunctional protein (MTP)/long-chain 3-Hydroxyacyl-CoA dehydrogenase (LCHAD) deficiency
... At the time of first admission to a metabolic center, the concentrations of C16-OH (0.045 μmol/l; reference < 0.01 μ mol/l) and C18:1-OH (0.055 μ mol/l; reference < 0.01 μ mol/l) in plasma were elevated. Molecular ...
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Mitochondrial trifunctional protein deficiency Catalytic heterogeneity of the mutant enzyme in two patients
... enzyme protein and biosynthesis of human trifunctional protein harboring enoyl-CoA hydratase, 3-hydroxyacyl-CoA dehydrogenase, and 3-ketoacyl-CoA thiolase activity in cultured skin fibroblasts from ...
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Child Neurology: Recurrent rhabdomyolysis due to a fatty acid oxidation disorder
... range from sudden infant death to Reye-like syn- drome, nonketotic hypoglycemia, skeletal myopathy, peripheral neuropathy, and progressive cardiomyopa- thy. Here, we describe an 18-month-old child pre- senting with ...
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Mild trifunctional protein deficiency is associated with progressive neuropathy and myopathy and suggests a novel genotype phenotype correlation
... Human mitochondrial trifunctional protein (TFP) is a het- erooctamer of four a - and four b -subunits that catalyzes three steps in the b -oxidation spiral of long-chain fatty ac- ...
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Long-Chain 3-Hydroxyacyl-CoA Dehydrogenase Deficiency: Clinical Presentation and Follow-Up of 50 Patients
... MTP, mitochondrial trifunctional protein; MCAD, medium-chain acyl-CoA dehydrogenase; VLCAD, very long- chain acyl-CoA dehydrogenase; CPT, carnitine palmitoyl-CoA transferase; HELLP, hemolysis, ...
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Mitochondrial Heat Shock Protein Machinery Hsp70/Hsp40 Is Indispensable for Proper Mitochondrial DNA Maintenance and Replication
... paired protein import would deplete the kDNA replication and maintenance enzymes; (ii) overall protein stability might be com- FIG 4 The ultrastructure of kDNA is severely altered in mtHsp70- and ...
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The Rubella Virus Capsid Protein Inhibits Mitochondrial Import
... RV-encoded protein known to associate with mitochondria (2, 3, ...the mitochondrial marker cytochrome c (Fig. 2). The E2 glyco- protein, which is the binding partner of E1, exhibits a localiza- tion ...
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Mitochondrial Protein Synthesis, Import, and Assembly
... The TIM23 complex must respond to signals in the sub- strate precursors that direct either their lateral insertion into the inner membrane or their complete translocation into the matrix. There is currently some dispute ...
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A polymorphism, R653Q, in the trifunctional enzyme methylenetetrahydrofolate dehydrogenase/methenyltetrahydrofolate cyclohydrolase/formyltetrahydrofolate synthetase is a maternal genetic risk factor for neural tube defects: report of the Birth Defects Research Group
... phosphate–dependent, trifunctional enzyme methylenetetrahydrofolate dehydrogenase/methenyltetrahydro- folate cyclohydrolase/formyltetrahydrofolate synthetase (MTHFD1) for an association with NTDs in the Irish ...
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Mitochondrial translocation of EGFR regulates mitochondria dynamics and promotes metastasis in NSCLC
... the mitochondrial translocation of EGFR; further, it leads to mitochondrial fission and redistribution in the lamellipodia, upregulates cellular ATP production, and enhances motility in vitro and in ...
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Cellular and proteomic studies of the mitochondrial ABAD/Aβ complex : investigating its role in Alzheimer's disease
... of protein spots displayed on a two- dimensional gel (Liao et ...the mitochondrial 2D gel with the three spots identified as succinate dehydrogenase (Figure ...
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Human Immunodeficiency Virus Type 1 Vpr Interacts with Antiapoptotic Mitochondrial Protein HAX-1
... a protein that associates with HS-1 (for hematopoietic lin- eage cell-specific protein ...signaling protein and is a substrate for intracellular protein tyrosine kinases involved in the immune ...
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Mitochondrial protein hyperacetylation in the failing heart
... tuned mitochondrial system ...for mitochondrial fuel oxidation and ATP production is causally linked to the development of heart failure ...in mitochondrial fatty acid oxi- dation (FAO), the chief ...
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Differential Proteomics Analyses Reveal Anxiety-Associated Molecular and Cellular Mechanisms in Cingulate Cortex Synapses
... ATP is hydrolyzed by cellular ATPases pumps into extra-mitochondrial ADP and inorganic phosphate (Pi). In the presence of creatine and ATP, MtCKs, functionally coupled to ATP synthase via adenine nucleotide ...
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The “fossilized” mitochondrial genome of Liriodendron tulipifera: ancestral gene content and order, ancestral editing sites, and extraordinarily low mutation rate
... To scale the substitutions per site to absolute substitution rates, a chronogram was estimated using the same 13 taxa and a concatenated three-plastid-gene alignment (3,936 bp) with BEAST ver. 1.61 [87] and the following ...
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