ArticleJournal of inherited metabolic disease1995
Measurement of peroxisomal fatty acid beta-oxidation in cultured human skin fibroblasts.
Article in Journal of inherited metabolic disease, 1995. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 31 papers.
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
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Who cites it
31 citing papers in PubMed, 98 citations in OpenAlex.
- Genetic heterogeneity in patients with a disorder of peroxisomal beta-oxidation: a complementation study based on pristanic acid beta-oxidation suggesting different enzyme defects.Journal of inherited metabolic disease · 1997Trial
- Protein Kinase C promotes peroxisome biogenesis and peroxisome-endoplasmic reticulum interaction.The Journal of cell biology · 2025Article
- HSDL2 links nutritional cues to bile acid and cholesterol homeostasis.Science advances · 2024Article
- Fluorescent fatty acid conjugates for live cell imaging of peroxisomes.Nature communications · 2024Article
- Autophagy Inhibitors Do Not Restore Peroxisomal Functions in Cells With the Most Common Peroxisome Biogenesis Defect.Frontiers in cell and developmental biology · 2021Article
- Article
- Peroxisomes can oxidize medium- and long-chain fatty acids through a pathway involving ABCD3 and HSD17B4.FASEB journal : official publication of the Federation of American Societies for Experimental Biology · 2019Article
- Case report of dysregulation of primary bile acid synthesis in a family with X-linked adrenoleukodystrophy.Medicine · 2018Article
- Neonatal detection of Aicardi Goutières Syndrome by increased C26:0 lysophosphatidylcholine and interferon signature on newborn screening blood spots.Molecular genetics and metabolism · 2017Article
- Peroxisomal abnormalities in the immortalized human hepatocyte (IHH) cell line.Histochemistry and cell biology · 2017Article
- The important role of biochemical and functional studies in the diagnostics of peroxisomal disorders.Journal of inherited metabolic disease · 2016Review
- Dataset for a case report of a homozygous PEX16 F332del mutation.Data in brief · 2016Article
- Zellweger spectrum disorders: clinical manifestations in patients surviving into adulthood.Journal of inherited metabolic disease · 2016Article
- A homozygous mutation inMolecular genetics and metabolism reports · 2015Article
- Heimler Syndrome Is Caused by Hypomorphic Mutations in the Peroxisome-Biogenesis Genes PEX1 and PEX6.American journal of human genetics · 2015Article
- Arginine improves peroxisome functioning in cells from patients with a mild peroxisome biogenesis disorder.Orphanet journal of rare diseases · 2013Article
- Bezafibrate lowers very long-chain fatty acids in X-linked adrenoleukodystrophy fibroblasts by inhibiting fatty acid elongation.Journal of inherited metabolic disease · 2012Article
- Substrate specificity overlap and interaction between adrenoleukodystrophy protein (ALDP/ABCD1) and adrenoleukodystrophy-related protein (ALDRP/ABCD2).The Journal of biological chemistry · 2011Article
- Mechanisms of disease: Inborn errors of bile acid synthesis.Nature clinical practice. Gastroenterology & hepatology · 2008Review
- X-linked adreno leukodistrophy: Profiles of very long chain fatty acids in plasma and fibroblasts in eigth Serbian patients.Indian journal of clinical biochemistry : IJCB · 2007Article
Corrections and comments
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Authors and funding
6 authors at 1 institution in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
One of the main functions of mammalian peroxisomes is the beta-oxidation of a variety of fatty acids and fatty acid derivatives, including very long-chain fatty acids. Oxidation of these fatty acids is deficient in a number of different peroxisomal disorders, including the disorders of peroxisome biogenesis (Zellweger syndrome, neonatal adrenoleukodystrophy and infantile Refsum disease), X-linked adrenoleukodystrophy and a number of other disorders of peroxisomal beta-oxidation of known and unknown aetiology. Accurate measurement of peroxisomal fatty acid oxidation is of utmost importance for correct postnatal and prenatal diagnosis of these disorders. In this paper we describe a straightforward and accurate assay method to measure the beta-oxidation of palmitic acid (C16:0), hexacosanoic acid (C26:0) and pristanic acid in intact fibroblasts.
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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.