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Compound active in mitochondria
the cells in three enzymatic reactions of the carnitine shuttle. The first reaction of the carnitine shuttle is a two-step process catalyzed by a family
Carnitine
Biochemical shuttle reaction
The Carnitine Shuttle is a biochemical reaction used to transport long chain fatty acids (LCFAs) from the cytosol into the matrix of the mitochondria
Carnitine_shuttle
Mammalian protein found in Homo sapiens
across the inner mitochondrial membrane as part of the carnitine shuttle system. Fatty acyl–carnitine can diffuse from the cytosol across the porous outer
Carnitine-acylcarnitine translocase
Carnitine-acylcarnitine_translocase
Enzyme found in humans
Carnitine palmitoyltransferase I (CPT1) also known as carnitine acyltransferase I, CPTI, CAT1, CoA:carnitine acyl transferase (CCAT), or palmitoylCoA transferase
Carnitine palmitoyltransferase I
Carnitine_palmitoyltransferase_I
Medical condition
Carnitine palmitoyltransferase II deficiency, sometimes shortened to CPT-II or CPT2, is an autosomal recessively inherited genetic metabolic disorder
Carnitine palmitoyltransferase II deficiency
Carnitine_palmitoyltransferase_II_deficiency
Chemical compound
intermediate products of the carnitine shuttle. In the mitochondria themselves, meldonium also competitively inhibits the carnitine shuttle protein SLC22A5. This
Meldonium
Medical condition
primary carnitine deficiency (SPCD) is an inborn error of fatty acid transport caused by a defect in the transporter responsible for moving carnitine across
Systemic primary carnitine deficiency
Systemic_primary_carnitine_deficiency
Metabolism via Carnitine Shuttle Pathway (with Animation) Fraenkel, G.; Friedman, S. Carnitine. Vitam. Horm. 1957, 15, 73–118. Bremer, J. Carnitine – metabolism
Carnitine_biosynthesis
Chemical compound
acid and can be transported into the mitochondrial matrix by the carnitine shuttle system (which transports fatty acyl-CoA molecules into mitochondria)
Palmitoyl-CoA
Mammalian protein found in humans
Carnitine O-palmitoyltransferase 2, mitochondrial is an enzyme that in humans is encoded by the CPT2 gene. Carnitine palmitoyltransferase II precursor
Carnitine palmitoyltransferase II
Carnitine_palmitoyltransferase_II
Process of fatty acid breakdown
membranes. Acyl-carnitine is shuttled inside by a carnitine-acylcarnitine translocase, as a carnitine is shuttled outside. Acyl-carnitine is converted back
Beta_oxidation
Set of biological processes
membranes. Acyl-carnitine is shuttled inside by a carnitine-acylcarnitine translocase, as a carnitine is shuttled outside. Acyl-carnitine is converted back
Fatty_acid_metabolism
Protein-coding gene in the species Homo sapiens
cytoskeleton fibrillar center cytoplasm cytosol Biological process carnitine shuttle malonyl-CoA biosynthetic process lipid metabolism fatty acid metabolic
ACACA
Acyl CoA with a fatty acid tail
acyltransferase II reverses the process, producing fatty acyl-CoA and carnitine. This shuttle mechanism is required only for longer chain fatty acids. Once inside
Fatty_acyl-CoA_esters
Class of enzymes
mitochondria (Carnitine Shuttle System) Carnitine-acylcarnitine translocase (CACT) catalyzes both unidirectional transport of carnitine and carnitine/acylcarnitine
Translocase
British Metabolomic expert and Mass Spectrometrist
Nazroo J, Goodacre R (2019). "Metabolic dysregulation in vitamin E and carnitine shuttle energy mechanisms identified as drivers behind human frailty". Nature
Roy_Goodacre
Protein-coding gene in humans
fatty acid oxidation by means of the ability of malonyl-CoA to inhibit carnitine palmitoyltransferase I, the rate-limiting step in fatty acid uptake and
ACACB
Protein-coding gene in the species Homo sapiens
nucleotide-activated protein kinase complex extracellular space Biological process carnitine shuttle intracellular signal transduction sterol biosynthetic process regulation
PRKAG2
Protein-coding gene in the species Homo sapiens
catalytic activity positive regulation of cold-induced thermogenesis carnitine shuttle regulation of macroautophagy regulation of signal transduction by
PRKAB2
Protein-coding gene in the species Homo sapiens
lipid metabolism regulation of triglyceride biosynthetic process carnitine shuttle response to bacterium Sources:Amigo / QuickGO Orthologs Databases
THRSP
Metabolic process
conjugated to carnitine by carnitine acyltransferase I (palmitoyltransferase) I located on the outer mitochondrial membrane Acyl carnitine is shuttled inside
Fatty_acid_degradation
Protein-coding gene in the species Homo sapiens
cytoplasmic stress granule axon dendrite soma Biological process carnitine shuttle steroid metabolic process intracellular signal transduction cellular
PRKAA2
Cell structure
cleavage enzyme Protein tyrosine phosphatase Carnitine O-palmitoyltransferase Carnitine O-acetyltransferase Carnitine O-octanoyltransferase Cytochrome P450 Translocase
Inner_mitochondrial_membrane
Phosphatidylcholine biosynthetic process
low-affinity, sodium-independent organic cation transport proteins (OCTs) and/or carnitine/organic cation transporters (OCTNs), and does not require ATP. Lastly
CDP-choline_pathway
Changes in metabolism that occur in response to a lack of food
liver cells. Fatty acids are transported into the mitochondria as an acyl-carnitine via the action of the enzyme CAT-1. This step controls the metabolic flux
Starvation_response
Protein-coding gene in mammals
phosphoenol pyruvate carboxykinase, 3-hydroxybutyrate dehydrogenase type 1, and carnitine palmitoyltransferase 1α. In addition, injection of FGF21 was associated
Fibroblast_growth_factor_21
Peptide hormone
formed by ACC during denovo synthesis and an allosteric inhibitor of carnitine palmitoyltransferase I (CPT1), a mitochondrial enzyme important for bringing
Glucagon
Chemical synthesis of ketone bodies
production of malonyl-CoA from acetyl-CoA. Malonyl-CoA reduces the activity of carnitine palmitoyltransferase I, an enzyme that brings fatty acids into the mitochondria
Ketogenesis
Specialized technique associated with MRI
Participates in gluconeogenesis Essential to malate-aspartate shuttle None None Carnitine 3.21 Transporting long-chain fatty acids across the mitochondrial
In vivo magnetic resonance spectroscopy
In_vivo_magnetic_resonance_spectroscopy
carbon-monoxide dehydrogenase (cytochrome b-561) - carboxyl terminus - carcinoma - carnitine dehydratase - carrier - carveol dehydrogenase - Catalog of MCA Control
Index of molecular biology articles
Index_of_molecular_biology_articles
Protein-coding gene in the species Homo sapiens
uncoupling and fatty acid oxidation, such as uncoupling protein 1 (UCP1), carnitine palmitoyltransferase I (CPT1b), and the peroxisome proliferator‑activated
NRIP1
CARNITINE SHUTTLE
CARNITINE SHUTTLE
Boy/Male
Indian
Air; Space Shuttle
Surname or Lastname
English
English : habitational name from any of several places so called (in Lancashire, Derbyshire, and West Yorkshire), which are named from Old English scyttel(s) ‘bar’, ‘bolt’ + worð ‘enclosure’. Reaney and Wilson give also Shuttlewood in Bolsover, Derbyshire, as a source of the surname.
Surname or Lastname
English
English : from Middle English slaye (Old English slege, from slēan ‘to strike’), a metonymic occupational name for a slay maker, an implement used in weaving to push the weft thread tightly against the thread of the preceding pass of the shuttle.English : topographic name from Middle English slay ‘grassy slope’.
Surname or Lastname
English
English : variant of Shuttleworth.
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