{"doi":"10.1128/mcb.23.2.744-753.2003","title":"Role of ALDP (ABCD1) and Mitochondria in X-Linked Adrenoleukodystrophy","abstract":null,"journal":"Molecular and Cellular Biology","year":2003,"id":651902,"datarank":0.750591945891819,"base_score":5.003946305945459,"endowment":5.003946305945459,"self_citation_contribution":0.750591945891819,"citation_network_contribution":0.0,"self_endowment_contribution":0.750591945891819,"citer_contribution":0.0,"corpus_percentile":null,"corpus_rank":null,"citation_count":148,"citer_count":0,"citers_with_citation_signal":0,"citers_with_endowment":0,"datacite_reuse_total":0,"is_dataset":false,"is_dataset_confidence":null,"is_data_producer":false,"deposit_databanks":null,"is_oa":false,"file_count":0,"downloads":0,"has_version_chain":false,"published_date":null,"fair_score":null,"fair_percentile":null,"algorithm_id":"datarank_citation_only_1hop_v6","ranking_scope":"data_only","authors":[{"id":1700358,"name":"J.-F. Lu","orcid":null,"position":1,"is_corresponding":false},{"id":1700359,"name":"H.-P. Zhang","orcid":null,"position":2,"is_corresponding":false},{"id":1700360,"name":"G.-X. Dong","orcid":null,"position":3,"is_corresponding":false},{"id":1700361,"name":"A. K. Heinzer","orcid":null,"position":4,"is_corresponding":false},{"id":1700362,"name":"P. A. Watkins","orcid":null,"position":5,"is_corresponding":false},{"id":872786,"name":"J. Powers","orcid":"0000-0003-2033-2427","position":6,"is_corresponding":false},{"id":1700363,"name":"K. D. Smith","orcid":null,"position":7,"is_corresponding":false},{"id":1700357,"name":"M. C. McGuinness","orcid":null,"position":0,"is_corresponding":false}],"reference_count":0,"raw_metadata":{"has_enrichment":true,"resolved":true,"title":"Role of ALDP (ABCD1) and Mitochondria in X-Linked Adrenoleukodystrophy","abstract":"Peroxisomal disorders have been associated with malfunction of peroxisomal metabolic pathways, but the pathogenesis of these disorders is largely unknown. X-linked adrenoleukodystrophy (X-ALD) is associated with elevated levels of very-long-chain fatty acids (VLCFA; C(>22:0)) that have been attributed to reduced peroxisomal VLCFA beta-oxidation activity. Previously, our laboratory and others have reported elevated VLCFA levels and reduced peroxisomal VLCFA beta-oxidation in human and mouse X-ALD fibroblasts. In this study, we found normal levels of peroxisomal VLCFA beta-oxidation in tissues from ALD mice with elevated VLCFA levels. Treatment of ALD mice with pharmacological agents resulted in decreased VLCFA levels without a change in VLCFA beta-oxidation activity. These data indicate that ALDP does not determine the rate of VLCFA beta-oxidation and that VLCFA levels are not determined by the rate of VLCFA beta-oxidation. The rate of peroxisomal VLCFA beta-oxidation in human and mouse fibroblasts in vitro is affected by the rate of mitochondrial long-chain fatty acid beta-oxidation. We hypothesize that ALDP facilitates the interaction between peroxisomes and mitochondria, resulting, when ALDP is deficient in X-ALD, in increased VLCFA accumulation despite normal peroxisomal VLCFA beta-oxidation in ALD mouse tissues. In support of this hypothesis, mitochondrial structural abnormalities were observed in adrenal cortical cells of ALD mice.","is_dataset_classified":null,"base_score":5.003946305945459,"endowment":5.003946305945459,"datacite_reuse_total":0,"file_count":0,"downloads":0,"views":0,"has_version_chain":false,"is_dataset":false,"is_oa":false,"pmid":"12509471","pmcid":"PMC151532","openalex_id":"https://openalex.org/W2002316432","authors":[],"funders":[{"funder_name":"NICHD NIH HHS","grant_id":"HD 24061","title":null},{"funder_name":"NINDS NIH HHS","grant_id":"NS 37355","title":null},{"funder_name":"NINDS NIH HHS","grant_id":"R01 NS037355","title":null},{"funder_name":"NICHD NIH HHS","grant_id":"HD10981","title":null},{"funder_name":"NICHD NIH HHS","grant_id":"P01 HD010981","title":null},{"funder_name":"NICHD NIH HHS","grant_id":"P30 HD024061","title":null}],"total_grants":6,"fwci":4.9908,"citation_percentile":0.96283437,"influential_citations":0,"citation_trend":[{"year":2012,"count":4},{"year":2013,"count":7},{"year":2014,"count":3},{"year":2015,"count":5},{"year":2016,"count":1},{"year":2017,"count":5},{"year":2018,"count":6},{"year":2020,"count":4},{"year":2021,"count":8},{"year":2022,"count":9},{"year":2023,"count":3},{"year":2024,"count":6},{"year":2025,"count":3},{"year":2026,"count":1}],"oa_status":"green","license":"https://journals.asm.org/non-commercial-tdm-license","oa_locations":[{"url":"https://www.ncbi.nlm.nih.gov/pmc/articles/151532","host_type":"repository"},{"url":"https://www.ncbi.nlm.nih.gov/pmc/articles/151532","host_type":"repository"},{"url":"https://journals.asm.org/doi/pdf/10.1128/MCB.23.2.744-753.2003","host_type":"publisher"},{"url":"https://www.tandfonline.com/doi/pdf/10.1128/MCB.23.2.744-753.2003","host_type":"publisher"},{"url":"https://doi.org/10.1128/mcb.23.2.744-753.2003","host_type":"journal"},{"url":"https://pubmed.ncbi.nlm.nih.gov/12509471","host_type":"repository"}],"fields_of_study":["Peroxisome Proliferator-Activated Receptors","Metabolism and Genetic Disorders","Drug Transport and Resistance Mechanisms"],"mesh_terms":["ATP Binding Cassette Transporter, Subfamily D, Member 1","Adrenal Glands","Adrenoleukodystrophy","Animals","Cell Line","Cell Separation","Cells, Cultured","Fatty Acids","Fibroblasts","Flow Cytometry","Humans","Microscopy, Electron","Mitochondria","Mutation","Oxygen","Time Factors","Tissue Distribution","ATP-Binding Cassette Transporters","Peroxisomes","Mice"],"keywords":["Adrenoleukodystrophy","Peroxisome","Peroxisomal disorder","Biology","Mitochondrion","Phytanic acid","Biochemistry","Internal medicine","Endocrinology","Gene","Medicine"],"sdg_mappings":[],"linked_datasets":[],"clinical_trials":[],"software_tools":[],"database_accessions":[{"name":"omim"}],"source":"live","citation_network_status":"fetched"},"created_at":"2026-08-10T11:11:19.743429Z","pmid":null,"pmcid":null,"fwci":null,"citation_percentile":null,"influential_citations":0,"oa_status":null,"license":null,"views":0,"total_file_size_bytes":0,"version_count":0,"fair_f":null,"fair_a":null,"fair_i":null,"fair_r":null,"fair_zscore":null,"fair_rationale":null,"fair_model":null,"fair_agent_version":null,"fair_fulltext_source":null,"fair_has_llm":null,"fair_computed_at":null,"clinical_trials":[],"software_tools":[],"db_accessions":[],"linked_datasets":[],"topics":[]}