MinireviewCarnitine Transport by Organic Cation Transporters and Systemic Carnitine Deficiency
References (57)
- et al.
Clinical and neurochemical effects of acetyl--carnitine in Alzheimer's disease
Neurobiol Aging
(1995) - et al.
Cloning and functional characterization of a potential-sensitive, polyspecific organic cation transporter (OCT3) most abundantly expressed in placenta
J Biol Chem
(1998) - et al.
Cloning and characterization of a novel human pH-dependent organic cation transporter, OCTN1
FEBS Lett
(1997) - et al.
Molecular and functional identification of sodium ion-dependent, high affinity human carnitine transporter OCTN2
J Biol Chem
(1998) - et al.
cDNA sequence, transport function, and genomic organization of human OCTN2, a new member of the organic cation transporter family
Biochem Biophys Res Commun
(1998) - et al.
Molecular and functional characterization of organic cation/carnitine transporter family in mice
J Biol Chem
(2000) - et al.
cDNA cloning and functional expression of a novel rat kidney organic cation transporter, OCT2
Biochem Biophys Res Commun
(1996) - et al.
Membrane localization of the electrogenic cation transporter rOCT1 in rat liver
Biochem Biophys Res Commun
(1998) - et al.
Identity of the organic cation transporter OCT3 as the extraneuronal monoamine transporter (uptake 2) and evidence for the expression of the transporter in the brain
J Biol Chem
(1998) - et al.
Structural and functional characteristics and tissue distribution pattern of rat OCTN1, an organic cation transporter, cloned from placenta
Biochim Biophys Acta
(2000)
β-lactam antibiotics as substrates for OCTN2, an organic cation/carnitine transporter
J Biol Chem
Molecular basis of organic anion and cation transport
Kidney Int
Carnitine uptake defect: Frameshift mutations in the human plasmalemmal carnitine transporter gene
Biochem Biophys Res Commun
Carnitine transporter OCTN2 mutations in systemic primary carnitine deficiency: A novel Arg169Gln mutation and a recurrent Arg282ter mutation associated with an unconventional splicing abnormality
Biochem Biophys Res Commun
Abnormal sodium stimulation of carnitine transport in primary carnitine deficiency
J Biol Chem
GFP-human high-affinity carnitine transporter OCTN2 protein: Subcellular localization and functional restoration of carnitine uptake in mutant cell lines with the carnitine transporter defect
Biochem Biophys Res Commun
Mutations in novel organic cation transporter (OCTN2), an organic cation/carnitine transporter, with differential effects on the organic cation transport function and the carnitine transport function
J Biol Chem
A missense mutation of mouse OCTN2, a sodium-dependent carnitine cotransporter, in the juvenile visceral steatosis mouse
Biochem Biophys Res Commun
Urea cycle disorder in C3H-H-2° mice with juvenile steatosis of viscera
FEBS Lett
Abnormal expression of urea cycle enzyme genes in juvenile visceral steatosis (jvs) mice
Biochim Biophys Acta
Cardiac hypertrophy in juvenile visceral steatosis (jvs) mice with systemic carnitine deficiency
FEBS Lett
Animal model of systemic carnitine deficiency: Analysis in C3H-H-2° strain of mouse associated with juvenile visceral steatosis
Biochem Biophys Res Commun
Carnitine administration to juvenile visceral steatosis mice corrects the suppressed expression of urea cycle enzymes by normalizing their transcription
J Biol Chem
Primary defect of juvenile visceral steatosis (jvs) mouse with systemic carnitine deficiency is probably in renal carnitine transport system
Biochim Biophys Acta
Carnitine transport defect in fibroblasts of juvenile visceral steatosis (JVS) mouse
Biochem Biophys Res Commun
Gene-dose effect on carnitine transport activity in embryonic fibroblasts of jvs mice as a model of human carnitine transporter deficiency
Biochem Pharmacol
Definition of the locus responsible for systemic carnitine deficiency within a 1.6cM region of mouse chromosome 11 by detailed linkage analysis
Genomics
Role of organic cation transporters in drug absorption and elimination
Annu Rev Pharmacol Toxicol
Cited by (128)
Longitudinal changes in fatty acid metabolism and in the mitochondrial protein import system in overconditioned and normal conditioned cows: A transcriptional study using microfluidic quantitative PCR
2021, Journal of Dairy ScienceCitation Excerpt :Furthermore, Zhu et al. (2019) demonstrated that the hepatic mRNA abundance of FABP1 was greater in the cows with subclinical (3.0 > BHB ≥1.2 mM) or clinical (BHB ≥3.0 mM) ketosis relative to control cows (BHB ≤0.6 mM) in early lactation. The SLC22A5 (OCTN2) transporter facilitates the import of carnitine into the hepatocyte (Lahjouji et al., 2001). The upregulation of SLC22A5 and SLC25A20 after parturition was more pronounced in HBCS cows, suggesting a need for increasing the capacity of FA uptake, and FA transport into the hepatocyte.
Nutrition and anemia in chronic kidney disease
2021, Nutritional Management of Renal Disease, Fourth EditionThe inhibitory effect of antiretroviral drugs on the L-carnitine uptake in human placenta
2019, Toxicology and Applied PharmacologyThe pro-inflammatory cytokine tumor necrosis factor α stimulates expression of the carnitine transporter OCTN2 (novel organic cation transporter 2) and carnitine uptake via nuclear factor-κB in Madin-Darby bovine kidney cells
2015, Journal of Dairy ScienceCitation Excerpt :The main finding of the present study is that the pro-inflammatory cytokine TNFα stimulates gene expression of OCTN2 and carnitine uptake in the bovine MDBK cell line, and co-treatment of cells with an NF-κB inhibitor blocks the TNFα-induced stimulation of OCTN2 gene expression and carnitine uptake in this cell line. The OCTN2 protein is essential for carnitine homeostasis through its important role in carnitine distribution between tissues and the tubular carnitine reabsorption process in the kidney, which is evidenced by the fact that mutations in the OCTN2 gene lead to systemic carnitine deficiency, at least in humans (Lahjouji et al., 2001). Given this important role of OCTN2 for carnitine uptake, it is likely that the TNFα-stimulated carnitine uptake observed in this study was due to upregulation of OCTN2.
A cause of dilated cardiomyopathy in child: Primary carnitine deficiency
2014, Annales de Cardiologie et d'Angeiologie
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