We have investigated the palmitic acid (C16:0) and cerotic acid (C26:0) activating activities in rat-liver microsomes and peroxisomes. The activation of the two fatty acids showed similar dependencies on ATP and coenzyme A, reflected in about equal apparent Km values both in microsomes and peroxisomes. In microsomes and peroxisomes similar apparent Km values for palmitic acid were found (15 microM and 22.8 microM, respectively), whereas apparent Km values for cerotic acid were 8.4 microM and 1.0 microM in microsomes and peroxisomes, respectively. The activation of cerotic acid was found to be inhibited to a progressively greater extent by increasing concentrations of 1-pyrenedecanoic acid (P10) as compared to the activation of palmitic acid, both in microsomes and peroxisomes. The inhibition by P10 of palmitic acid activation and cerotic acid activation was non-competitive in both organelles. From the observation that P10 activation is not affected by palmitic acid and cerotic acid, we conclude that P10 is activated by a distinct enzyme. Furthermore, our results are in accordance with earlier suggestions that activation of cerotic acid is brought about by an enzyme distinct from the palmitoyl-CoA synthetase.
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http://dx.doi.org/10.1111/j.1432-1033.1991.tb15844.x | DOI Listing |
Radiol Case Rep
January 2025
Department of General Medicine, Jawaharlal Nehru Medical College, Datta Meghe Institute of Higher Education and Research, Wardha, India.
Nutrients
October 2024
Research Unit for Predictive and Preventive Medicine, Bambino Gesù Children's Hospital, Istituti di Ricovero e Cura a Carattere Scientifico, IRCCS, 00165 Rome, Italy.
: Adrenoleukodystrophy (X-ALD) is a metabolic disorder caused by dysfunctional peroxisomal beta-oxidation of very-long-chain fatty acids (VLCFAs). A VLCFA-restricted Mediterranean diet has been proposed for patients and carriers to reduce daily VLCFA intake. : We retrospectively evaluated plasma VLCFAs in a cohort of 36 patients and 20 carriers at baseline and after 1 year of restricted diet.
View Article and Find Full Text PDFMedicine (Baltimore)
April 2024
Department of Neurology, China-Japan Union Hospital of Jilin University, Changchun, China.
Rationale: X-linked adrenoleukodystrophy (X-ALD) is caused by mutations in the ABCD1 gene leading to very long chain fatty acid (VLCFA) accumulation. The disease demonstrates a spectrum of phenotypes including adrenomyeloneuropathy (AMN). We aimed to identify the genetic basis of disease in a patient presenting with AMN features in order to confirm the diagnosis, expand genetic knowledge of ABCD1 mutations, and elucidate potential genotype-phenotype associations to inform management.
View Article and Find Full Text PDFFront Mol Biosci
November 2023
Department of Clinical Genetics, Medical University of Lodz, Lodz, Poland.
Alström syndrome (ALMS) and Bardet-Biedl syndrome (BBS) are among the so-called ciliopathies and are associated with the development of multiple systemic abnormalities, including early childhood obesity and progressive neurodegeneration. Given the progressive deterioration of patients' quality of life, in the absence of defined causal treatment, it seems reasonable to identify the metabolic background of these diseases and search for their progression markers. The aim of this study was to find metabolites characteristic to ALMS and BBS, correlating with clinical course parameters, and related to the diseases progression.
View Article and Find Full Text PDFACS Omega
November 2023
P.G & Research, Department of Chemistry, Loyola College, Chennai 600 034, India.
The current study presents a simple and cost-competitive method for the development of high-performance superhydrophobic and superoleophilic cotton fabrics coated with cadmium oxide/cerotic acid (CdO/CE)-polycaprolactone (PCL)- and cadmium oxide/stearic acid (CdO/ST)-polycaprolactone-grafted hybrid composites. X-ray powder diffraction, scanning electron microscopy, and Fourier transform infrared spectroscopy are used to characterize the CdO/CE-PCL and CdO/ST-PCL and polycaprolactone-modified cotton fabrics. Using an optical contact angle meter, the wetting behavior of corrosive liquids such as coffee, milk, tea, water dyed with methylene blue, strong acids (HCl), strong alkali (NaOH), and saturated salt solution (NaCl) on the CdO-CE/ST/PCL-modified cotton fabrics is assessed as well as the durability of CdO-CE/ST/PCL-modified cotton fabrics in corrosive liquids.
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