{"doi":"10.1152/ajplung.00032.2023","title":"GAA deficiency disrupts distal airway cells in Pompe disease","abstract":"This research highlights the impact of Pompe disease on distal airway cells. Prior to this work, respiratory insufficiency in Pompe disease was classically attributed to pathology in respiratory muscles and motor neurons. Using the Pompe mouse model, we note significant pathology in alveolar type 1 and 2 cells with reductions in surfactant protein D and disrupted surfactant homeostasis. These novel findings highlight the potential contributions of alveolar pathology to respiratory insufficiency in Pompe disease.","journal":"American Journal of Physiology-Lung Cellular and Molecular Physiology","year":2023,"id":372987,"datarank":0.26876392038420827,"base_score":1.791759469228055,"endowment":1.791759469228055,"self_citation_contribution":0.26876392038420827,"citation_network_contribution":0.0,"self_endowment_contribution":0.26876392038420827,"citer_contribution":0.0,"corpus_percentile":null,"corpus_rank":null,"citation_count":5,"citer_count":0,"citers_with_citation_signal":0,"citers_with_endowment":0,"datacite_reuse_total":0,"is_dataset":false,"is_dataset_confidence":0.9409,"is_data_producer":false,"deposit_databanks":null,"is_oa":true,"file_count":0,"downloads":0,"has_version_chain":false,"published_date":"2023-01-01","fair_score":null,"fair_percentile":null,"algorithm_id":"datarank_citation_only_1hop_v6","ranking_scope":"data_only","authors":[{"id":922758,"name":"Elias Lai","orcid":"0009-0004-2116-1812","position":1,"is_corresponding":false},{"id":12144,"name":"Preetish Kadur Lakshminarasimha Murthy","orcid":"0000-0002-9762-8376","position":2,"is_corresponding":false},{"id":373518,"name":"Debolina D. Biswas","orcid":"0000-0003-1305-0149","position":3,"is_corresponding":false},{"id":1133882,"name":"Rania Soufny","orcid":null,"position":4,"is_corresponding":false},{"id":852973,"name":"Angela L. Roger","orcid":"0000-0001-5249-7176","position":5,"is_corresponding":false},{"id":12178,"name":"Purushothama Rao Tata","orcid":"0000-0003-4837-0337","position":6,"is_corresponding":false},{"id":373519,"name":"Mai K. ElMallah","orcid":"0000-0002-1775-1156","position":7,"is_corresponding":false},{"id":923250,"name":"Léa El Haddad","orcid":null,"position":0,"is_corresponding":true}],"reference_count":39,"raw_metadata":{"citation_network_status":"fetched"},"created_at":"2026-07-19T01:15:58.101083Z","pmid":"37366541","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":[]}