| scholarly article | Q13442814 |
| P819 | ADS bibcode | 1984PNAS...81.4203S |
| P356 | DOI | 10.1073/PNAS.81.13.4203 |
| P932 | PMC publication ID | 345397 |
| P698 | PubMed publication ID | 6588384 |
| P5875 | ResearchGate publication ID | 16608699 |
| P2093 | author name string | H W Moser | |
| S Goldfischer | |||
| I Singh | |||
| A E Moser | |||
| P2860 | cites work | Adrenoleukodystrophy: evidence for X linkage, inactivation, and selection favoring the mutant allele in heterozygous cells | Q24633620 |
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| The large-scale separation of peroxisomes, mitochondria, and lysosomes from the livers of rats injected with triton WR-1339. Improved isolation procedures, automated analysis, biochemical and morphological properties of fractions | Q34205999 | ||
| Conversion of 3 alpha, 7 alpha, 12 alpha-trihydroxy-5 beta-cholestanoic acid into cholic acid by rat liver peroxisomes | Q34288021 | ||
| Adrenoleukodystrophy: increased plasma content of saturated very long chain fatty acids | Q34716419 | ||
| Neonatal adrenoleukodystrophy: clinical, pathologic, and biochemical delineation of a syndrome affecting both males and females | Q35885011 | ||
| Alpha hydroxylation of lignoceric acid in brain. Subcellular localization of alpha hydroxylation and the requirement for heat-stable and heat-labile factors and sphingosine | Q39264337 | ||
| Cytochemistry of human catalase. The demonstration of hepatic and renal peroxisomes by a high temperature procedure | Q39578294 | ||
| Light and electron microscopic liver changes in the cerebro-hepato-renal syndrome of Zellweger (Peroxisome deficiency) (author's transl) | Q39903439 | ||
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| Metabolic pathways in peroxisomes and glyoxysomes | Q40312979 | ||
| The mechanism of arrest of neuronal migration in the Zellweger malformation: An hypothesis based upon cytoarchitectonic analysis | Q41038903 | ||
| Defects of Bile Acid Synthesis in Zellweger's Syndrome | Q41466334 | ||
| A metabolic disorder similar to Zellweger syndrome with hepatic acatalasia and absence of peroxisomes, altered content and redox state of cytochromes, and infantile cirrhosis with hemosiderosis | Q44689247 | ||
| Glycerolipid biosynthesis in peroxisomes via the acyl dihydroxyacetone phosphate pathway | Q46184824 | ||
| Peroxisomal and mitochondrial defects in the cerebro-hepato-renal syndrome | Q47974070 | ||
| The evaluation of infants with the Zellweger (cerebro-hepato-renal) syndrome* | Q48462168 | ||
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| The cerebrohepatorenal (Zellweger) syndrome. Increased levels and impaired degradation of very-long-chain fatty acids and their use in prenatal diagnosis | Q48670157 | ||
| Severe plasmalogen deficiency in tissues of infants without peroxisomes (Zellweger syndrome). | Q48715988 | ||
| Biochemical studies in the liver and muscle of patients with Zellweger syndrome. | Q48797042 | ||
| Cerebro-hepato-renal (Zellweger) syndrome and neonatal adrenoleukodystrophy: similarities in phenotype and accumulation of very long chain fatty acids. | Q48862242 | ||
| Cerebro-hepato-renal syndrome of Zellweger: an inherited disorder of neuronal migration. | Q50644403 | ||
| Cerebro-hepato-renal syndrome. A newly recognized hereditary disorder of multiple congenital defects, including sudanophilic leukodystrophy, cirrhosis of the liver, and polycystic kidneys. | Q51203911 | ||
| Rat liver peroxisomes catalyze the beta oxidation of fatty acids | Q67276534 | ||
| A new method for simultaneous purification of cytochrome b5 and NADPH-cytochrome c reductase from rat liver microsomes | Q68743785 | ||
| Adrenoleukodystrophy: impaired oxidation of very long chain fatty acids in white blood cells, cultured skin fibroblasts, and amniocytes | Q71369133 | ||
| Adrenoleukodystrophy: impaired oxidation of long chain fatty acids in cultured skin fibroblasts an adrenal cortex | Q71580591 | ||
| The degradation of urate in liver peroxisomes. Association of allantoinase with allantoicase in amphibian liver but not in fish and invertebrate liver | Q71687987 | ||
| Peroxisomal beta-oxidation of long fatty acids: effects of high fat diets | Q72042317 | ||
| [Measurement of catalase activity] | Q74073174 | ||
| P433 | issue | 13 | |
| P407 | language of work or name | English | Q1860 |
| P1104 | number of pages | 5 | |
| P304 | page(s) | 4203-4207 | |
| P577 | publication date | 1984-07-01 | |
| P1433 | published in | Proceedings of the National Academy of Sciences of the United States of America | Q1146531 |
| P1476 | title | Lignoceric acid is oxidized in the peroxisome: implications for the Zellweger cerebro-hepato-renal syndrome and adrenoleukodystrophy | |
| P478 | volume | 81 |
| Q37389923 | 25-hydroxycholesterol contributes to cerebral inflammation of X-linked adrenoleukodystrophy through activation of the NLRP3 inflammasome |
| Q33733733 | A missense point mutation (Ser515Phe) in the adrenoleukodystrophy gene in a family with adrenomyeloneuropathy: a clinical, biochemical, and genetic study |
| Q35245375 | A new peroxisomal disorder with enlarged peroxisomes and a specific deficiency of acyl-CoA oxidase (pseudo-neonatal adrenoleukodystrophy) |
| Q69568391 | A prenatal test for the cerebro-hepato-renal (Zellweger) syndrome by demonstration of the absence of catalase-containing particles (peroxisomes) in cultured amniotic fluid cells |
| Q77731452 | A yeast strain defective in oleic acid utilization has a mutation in the RML2 gene |
| Q35417932 | ABCD1 deletion-induced mitochondrial dysfunction is corrected by SAHA: implication for adrenoleukodystrophy |
| Q45156613 | Abnormal profiles of polyunsaturated fatty acids in the brain, liver, kidney and retina of patients with peroxisomal disorders |
| Q28338863 | Accumulation of pristanic acid (2, 6, 10, 14 tetramethylpentadecanoic acid) in the plasma of patients with generalised peroxisomal dysfunction |
| Q38623776 | Adrenoleukodystrophy and other peroxisomal disorders that affect the nervous system, including new observations on L-pipecolic acid oxidase in primates |
| Q30660504 | Adrenoleukodystrophy and related disorders |
| Q33629275 | Adrenoleukodystrophy in a mother and son. |
| Q48544570 | Adrenoleukodystrophy without adrenal insufficiency and its magnetic resonance imaging |
| Q39598547 | Adrenoleukodystrophy: biochemical procedures in diagnosis, prevention and treatment |
| Q45889150 | Adrenoleukodystrophy: from bedside to molecular biology |
| Q28270437 | Adrenoleukodystrophy: molecular genetics, pathology, and Lorenzo's oil |
| Q24671055 | Altered expression of ALDP in X-linked adrenoleukodystrophy |
| Q90428649 | Augmenter of liver regeneration protein deficiency promotes hepatic steatosis by inducing oxidative stress and microRNA-540 expression |
| Q34418889 | Biochemistry of peroxisomes in health and disease |
| Q33742431 | Biology of senescent liver peroxisomes: role in hepatocellular aging and disease |
| Q37395755 | Biosynthesis and maturation of peroxisomal beta-oxidation enzymes in fibroblasts in relation to the Zellweger syndrome and infantile Refsum disease |
| Q72406532 | Blood polyunsaturated fatty acids in patients with peroxisomal disorders. A multicenter study |
| Q36713344 | Caffeic acid phenethyl ester induces adrenoleukodystrophy (Abcd2) gene in human X-ALD fibroblasts and inhibits the proinflammatory response in Abcd1/2 silenced mouse primary astrocytes |
| Q42855382 | Chlorpromazine and carnitine-dependency of rat liver peroxisomal beta-oxidation of long-chain fatty acids |
| Q38719077 | Comparative aspects of the mammalian cytochrome P450 IV gene family |
| Q69941476 | Compartmentation of dicarboxylic acid beta-oxidation in rat liver: importance of peroxisomes in the metabolism of dicarboxylic acids |
| Q48376668 | Conserved expression of alternative splicing variants of peroxisomal acyl-CoA oxidase 1 in vertebrates and developmental and nutritional regulation in fish. |
| Q41855406 | Detection of a homologous series of C26-C38 polyenoic fatty acids in the brain of patients without peroxisomes (Zellweger's syndrome). |
| Q68488219 | Detection of peroxisomes in human liver and kidney fixed with formalin and embedded in paraffin: The use of catalase and lipid β-oxidation enzymes as immunocytochemical markers |
| Q38770261 | Detection of unusual very-long-chain fatty acid and ether lipid derivatives in the fibroblasts and plasma of patients with peroxisomal diseases using liquid chromatography-mass spectrometry |
| Q24338719 | Distribution and cellular localization of adrenoleukodystrophy protein in human tissues: implications for X-linked adrenoleukodystrophy |
| Q53738596 | Effect of ciprofibrate on the activation and oxidation of very long chain fatty acids. |
| Q41389708 | Effect of hypoxia-reoxygenation on peroxisomal functions in cultured human skin fibroblasts from control and Zellweger syndrome patients |
| Q48409183 | Effects of aging on the content, composition and synthesis of sphingomyelin in the central nervous system |
| Q71602675 | Effects of clofibrate on lipids and fatty acids of mouse liver |
| Q44514379 | Effects of the testosterone metabolite dihydrotestosterone and 5 alpha-androstan-3 alpha,17 beta-diol on very long chain fatty acid metabolism in X-adrenoleukodystrophic fibroblasts |
| Q71479021 | Eicosapentaenoic acid, but not docosahexaenoic acid, increases mitochondrial fatty acid oxidation and upregulates 2,4-dienoyl-CoA reductase gene expression in rats |
| Q35771255 | Evaluation of therapy of X-linked adrenoleukodystrophy |
| Q28372514 | Fatty acid composition of brain glycerophospholipids in peroxisomal disorders |
| Q26744147 | Fatty acid metabolism and the basis of brown adipose tissue function |
| Q67959190 | Fatty acid metabolism in renal ischemia |
| Q69834543 | First trimester prenatal diagnosis of adrenoleukodystrophy by determination of very long chain fatty acid levels and by linkage analysis to a DNA probe |
| Q28551048 | Functional Characterization of IPSC-Derived Brain Cells as a Model for X-Linked Adrenoleukodystrophy |
| Q41308119 | Functions and organization of peroxisomal beta-oxidation |
| Q24561552 | Genetic heterogeneity in the cerebrohepatorenal (Zellweger) syndrome and other inherited disorders with a generalized impairment of peroxisomal functions. A study using complementation analysis |
| Q35380239 | HDAC inhibitor SAHA normalizes the levels of VLCFAs in human skin fibroblasts from X-ALD patients and downregulates the expression of proinflammatory cytokines in Abcd1/2-silenced mouse astrocytes |
| Q41822915 | High-affinity binding of very-long-chain fatty acyl-CoA esters to the peroxisomal non-specific lipid-transfer protein (sterol carrier protein-2) |
| Q34907267 | Histone deacetylase inhibitor upregulates peroxisomal fatty acid oxidation and inhibits apoptotic cell death in abcd1-deficient glial cells |
| Q24293596 | Human peroxisomal 3-oxoacyl-coenzyme A thiolase deficiency |
| Q34248207 | Hydroxyeicosatetraenoic acid metabolism in cultured human skin fibroblasts. Evidence for peroxisomal beta-oxidation |
| Q40839143 | Identification of the pathway of alpha-oxidation of cerebronic acid in peroxisomes |
| Q33892786 | Impaired degradation of leukotrienes in patients with peroxisome deficiency disorders |
| Q35796087 | Impaired mitochondrial fat oxidation induces adaptive remodeling of muscle metabolism |
| Q72478657 | Impairment of peroxisomal beta-oxidation system by endotoxin treatment |
| Q77952731 | Increased peroxisomal fatty acid beta-oxidation and enhanced expression of peroxisome proliferator-activated receptor-alpha in diabetic rat liver |
| Q42817044 | Induced pluripotent stem cell models from X-linked adrenoleukodystrophy patients |
| Q68976247 | Infantile Refsum disease: deficiency of catalase-containing particles (peroxisomes), alkyldihydroxyacetone phosphate synthase and peroxisomal beta-oxidation enzyme proteins |
| Q68291224 | Inhibition of 3 alpha,7 alpha,12 alpha-trihydroxy-5 beta-cholestanoic acid oxidation and of bile acid secretion in rat liver by fatty acids |
| Q41972280 | Interactions of a very long chain fatty acid with model membranes and serum albumin. Implications for the pathogenesis of adrenoleukodystrophy |
| Q22011096 | Intraperoxisomal localization of very-long-chain fatty acyl-CoA synthetase: implication in X-adrenoleukodystrophy |
| Q73726123 | Isolation and biochemical characterization of peroxisomes from cultured rat glial cells |
| Q40379234 | Komrower Lecture. Adrenoleukodystrophy: natural history, treatment and outcome |
| Q24628708 | Large deletion of the peroxisomal acyl-CoA oxidase gene in pseudoneonatal adrenoleukodystrophy |
| Q36002780 | Lipid-lowering drugs |
| Q42542201 | Lovastatin and sodium phenylacetate normalize the levels of very long chain fatty acids in skin fibroblasts of X- adrenoleukodystrophy |
| Q41308266 | Mammalian peroxisomes: metabolism of oxygen and reactive oxygen species |
| Q73117534 | Measurement of peroxisomal fatty acid beta-oxidation in cultured human skin fibroblasts |
| Q72376119 | Measurement of plasma very long-chain fatty acids as a preliminary screening procedure for the diagnosis of peroxisomal disorders |
| Q49167746 | Measurement of very long-chain fatty acids, phytanic and pristanic acid in plasma and cultured fibroblasts by gas chromatography |
| Q42050577 | Metabolism of saturated and polyunsaturated fatty acids by normal and Zellweger syndrome skin fibroblasts |
| Q73211506 | Metabolism of trideuterated iso-lignoceric acid in rats in vivo and in human fibroblasts in culture |
| Q53276010 | MicroRNA Profiling Identifies miR-196a as Differentially Expressed in Childhood Adrenoleukodystrophy and Adult Adrenomyeloneuropathy. |
| Q73830075 | Mitochondrial, but not peroxisomal, beta-oxidation of fatty acids is conserved in coenzyme A-deficient rat liver |
| Q28569176 | Molecular cloning of cDNA encoding rat very long-chain acyl-CoA synthetase |
| Q24294056 | Molecular organization of peroxisomal enzymes: protein-protein interactions in the membrane and in the matrix |
| Q28507035 | Mouse very long-chain acyl-CoA synthetase in X-linked adrenoleukodystrophy |
| Q41806915 | Multiple peroxisomal enzymatic deficiency disorders. A comparative biochemical and morphologic study of Zellweger cerebrohepatorenal syndrome and neonatal adrenoleukodystrophy |
| Q33971574 | Myelin and disorders that affect the formation and maintenance of this sheath |
| Q35888833 | Novel subtype of peroxisomal acyl-CoA oxidase deficiency and bifunctional enzyme deficiency with detectable enzyme protein: identification by means of complementation analysis |
| Q46324842 | Overexpression of PGC-1α increases peroxisomal activity and mitochondrial fatty acid oxidation in human primary myotubes. |
| Q39073343 | Peroxisomal Dysfunction in Age-Related Diseases. |
| Q34602966 | Peroxisomal beta-oxidation enzyme proteins in adrenoleukodystrophy: distinction between X-linked adrenoleukodystrophy and neonatal adrenoleukodystrophy |
| Q34567743 | Peroxisomal bifunctional enzyme deficiency |
| Q67803021 | Peroxisomal degradation of leukotrienes by beta-oxidation from the omega-end |
| Q41308196 | Peroxisomal diseases: a microscopist looks through the retrospectroscope |
| Q68563955 | Peroxisomal disorders |
| Q68953015 | Peroxisomal disorders |
| Q38179029 | Peroxisomal disorders. A review of a recently recognized group of clinical entities |
| Q39730039 | Peroxisomal disorders: a newly recognised group of genetic diseases |
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| Q41308204 | Peroxisomal disorders: overview |
| Q41016301 | Peroxisomal fatty acid beta-oxidation in relation to the accumulation of very long chain fatty acids in cultured skin fibroblasts from patients with Zellweger syndrome and other peroxisomal disorders |
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| Q34488327 | Plasma very long chain fatty acids in 3,000 peroxisome disease patients and 29,000 controls |
| Q48750294 | Postnatal development and isolation of peroxisomes from brain |
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| Q48352091 | Presence of heterogeneous peroxisomal populations in the rat nervous tissue |
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| Q42857172 | Structure and lipid distribution of polyenoic very-long-chain fatty acids in the brain of peroxisome-deficient patients (Zellweger syndrome). |
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