Ask about this productRelated genes to: SFTPB antibody
- Gene:
- SFTPB NIH gene
- Name:
- surfactant protein B
- Previous symbol:
- SFTP3
- Synonyms:
- SP-B
- Chromosome:
- 2p11.2
- Locus Type:
- gene with protein product
- Date approved:
- 1988-07-06
- Date modifiied:
- 2016-10-05
Related products to: SFTPB antibody
Related articles to: SFTPB antibody
- Surfactant-related gene (SRG) variants are an emerging cause of interstitial lung disease/pulmonary fibrosis (ILD/PF). Accurate recognition of these disorders is relevant for diagnosis, genetic counselling, and clinical management. How do SRG-associated ILD/PF present in adults, and what clinical, radiological, and histopathological features characterize their phenotype? We performed a systematic review (PROSPERO CRD42024517610) to describe the clinical features, management, and outcomes of adults with SRG-related ILD/PF. PubMed, Embase, and Web of Science were searched from January 2000 to December 2024 for studies reporting adults (≥18 years) with genetically confirmed SRG variants and ILD/PF. Demographic, clinical, radiological, histological, functional, treatment, and lung transplantation (LT) data were extracted. Thirty-eight studies included 162 adults carrying class 3-5 variants in 2 (61), (52), (31), (16), and (2), including 35 novel variants. Age at diagnosis ranged from 19 to 79 years. Familial ILD predominated in SFTPA1/2, frequently associated with personal or familial lung cancer, whereas variants showed thyroid and neurological involvement. Lung function typically demonstrated mild-to-moderate restriction with impaired diffusing capacity. Chest CT findings were heterogeneous and often indeterminate, while usual interstitial pneumonia was the predominant histological pattern except in -related disease. Treatment approaches varied considerably; LT provided favourable outcomes in selected patients. Adult SRG-related ILD/PF is likely under-recognised because of variable penetrance, wide age at onset, and non-specific imaging features. International registries are needed to improve genotype-phenotype correlations and guide personalised management. - Source: PubMed
Publication date: 2026/09/24
Tirelli ClaudioSanduzzi Zamparelli StefanoLouvrier CamilleMira SabrinaItalia MartaIovine Paola RebeccaAlfano FaustaCentanni StefanoMondoni MicheleNathan NadiaBocchino Marialuisa - - Source: PubMed
Publication date: 2026/09/16
Tirelli ClaudioLogan NiamhMahida KrunalChua FelixDesai Sujal RMorris-Rosendahl Deborah JRenzoni Elisabetta - Neonatal lung biopsy guides management of unusually severe, diffuse lung disease with an uncertain diagnosis. Childhood interstitial lung disease (chILD) constitutes a diverse group of uncommon respiratory diseases which are associated with major morbidity and mortality. The incidence, outcome and mortality of chILD and other severe respiratory diseases in resource-limited settings (RLS) are unclear. - Source: PubMed
Goussard PierreSchubert Pawelvan Wyk LizelleBooysen LynnGie AndréUrban MichaelJanson JacquesAndronikou SavvasEber Ernst - H9N2 avian influenza virus (AIV) poses significant threats to poultry and public health, with the potential to contribute to the emergence of more virulent influenza strains. Hypoxia-inducible factor 1-alpha (HIF-1α) plays a crucial role in viral pathogenesis, immune regulation, and inflammation, making it a promising target for therapeutic intervention. Here, we explored the involvement of HIF-1α in H9N2 AIV pathogenesis and associated inflammatory responses. Our data revealed that HIF-1α exacerbates inflammation induced by H9N2 AIV infection through its regulation of mitochondrial fitness. Additionally, we investigated Sotetsuflavone (Seo), a naturally derived flavonoid, for its potential to modulate inflammatory responses following H9N2 infection. Molecular docking coupled with surface plasmon resonance assays indicated that Seo exhibits robust binding affinity to HIF-1α, implying a direct inhibitory mechanism. Subsequent studies demonstrated that Seo greatly reduced the production of inflammatory cytokines, including IL-1β, IL-6, and TNF-α, through suppressing the HIF-1α signaling pathway in HD11 cells infected with H9N2 AIV. Correspondingly, experiments established that Seo administration significantly ameliorated pulmonary inflammation in H9N2-infected mice, while also enhancing alveolar epithelial repair, as evidenced by increased expression of type II alveolar epithelial cell markers Abca3 and Sftpb. Collectively, these findings identify Seo as a potent inhibitor of HIF-1α, highlighting its therapeutic potential in mitigating inflammation and lung damage associated with influenza virus infection.IMPORTANCEH9N2 avian influenza virus triggers inflammatory lung injury via hypoxia-inducible factor-1 alpha (HIF-1α)-mediated dysregulation of mitochondrial function. The natural flavonoid Sotetsuflavone (Seo) directly binds and inhibits HIF-1α, as confirmed by molecular docking and surface plasmon resonance assays. In H9N2-infected HD11 cells, Seo suppresses HIF-1α signaling, reducing IL-1β, IL-6, and TNF-α production. , Seo treatment attenuates pulmonary inflammation and enhances alveolar epithelial repair in mice, upregulating type II pneumocyte markers (Abca3 and Sftpb). These findings identify Seo as a promising HIF-1α-targeted therapeutic for influenza-associated immunopathology. - Source: PubMed
Publication date: 2026/08/25
Zhu SuyingLi BeibeiWang YaqiTian YuhanZhang JiayueWang Hongshan - Immune checkpoint inhibitors (ICIs) have transformed the therapeutic landscape of advanced non-small cell lung cancer (NSCLC), yet ~50% of lung adenocarcinoma (LUAD) patients exhibit primary or acquired resistance, underscoring the urgent need for predictive biomarkers. Hypoxia and metabolic reprogramming (HMR) are intertwined oncogenic hallmarks that reshape the tumor microenvironment (TME) and drive immune evasion, representing promising targets for signature development. Herein, we constructed an integrated HMR gene signature to predict clinical outcomes and therapeutic vulnerabilities in LUAD. - Source: PubMed
Publication date: 2026/06/25
Ma YuquanYang MengmengSun ZhitaoLiu FeiZheng BoyingWang YanweiLiu Junfeng