Ask about this productRelated genes to: SFTPC antibody
- Gene:
- SFTPC NIH gene
- Name:
- surfactant protein C
- Previous symbol:
- SFTP2
- Synonyms:
- SP-C, PSP-C, SMDP2, BRICD6
- Chromosome:
- 8p21.3
- Locus Type:
- gene with protein product
- Date approved:
- 1988-05-11
- Date modifiied:
- 2016-10-05
Related products to: SFTPC antibody
Related articles to: SFTPC antibody
- Alveolar type 2 (AT2) cell dysfunction is key to the development of many lung diseases including pulmonary fibrosis (PF). The underlying mechanisms often remain poorly understood due to a paucity of manipulable primary human models and lack of defined triggers. Monogenic forms of familial pulmonary fibrosis combined with recent advances in in vitro culture techniques offer a unique opportunity to interrogate early pathogenic events in PF. To model toxic gain-of-function disease caused by the SFTPC variant I73T, we employed a base editing strategy in human lung-derived AT2 (fdAT2) organoids to edit the endogenous SFTPC locus and generate a heterozygous SFTPC-I73T-expressing disease model which we interrogated when grown in standard 3D culture, monolayer culture, and at air-liquid interface. SFTPC-I73T expressing organoids failed to form lumens and displayed disrupted epithelial polarity. This was due to SFTPC accumulation in enlarged early endosomes resulting in impaired apico-basal trafficking of polarity and adhesion proteins. Air exposure exacerbated these defects, causing epithelial barrier breakdown and impaired wound healing. Together, we demonstrate the ability to edit endogenous loci in differentiated alveolar organoids to generate disease models that provide mechanistic insights into disease. We establish endosomal dysfunction and polarity loss as drivers of SFTPC-I73T-mediated epithelial injury and highlight mechanisms that may underlie AT2 dysfunction in disease more broadly. - Source: PubMed
Publication date: 2026/08/27
Rutherford Eimear NSun DaweiLim KyungtaeGodbehere Christopher SEdgar James RMatesic Lydia EMarciniak Stefan JRawlins Emma LDickens Jennifer A - This study retrospectively analyzed the clinical data of a female child with primary ciliary dyskinesia (PCD) caused by variants in the CFTR (NM_000492.3) and SFTPC (NM_003018.3). All variant descriptions in this report follow the HGVS standardized nomenclature guidelines. These two variants are considered to be potentially associated with pediatric PCD. - Source: PubMed
Publication date: 2026/08/24
Yuan XinhuiShao DanLi YumeiLing JizuGong Yigu - The progressive lung function decline in severe asthma is poorly explained by traditional inflammation‑centered paradigms. Calcium (Ca²⁺) dysregulation and transforming growth factor‑β (TGF‑β) signaling have been implicated, but the molecular link between them and their upstream regulators remain elusive. - Source: PubMed
Publication date: 2026/08/14
Zhu ZhiqinXiao QingaoCai RunjinQi EnyuGong XiaoxiaoTang JialeLi XiaozhaoFeng Juntao - To determine whether radiation directly compromises AT2 cell stemness through oxidative and DNA damage, and to evaluate whether Compound Kushen Injection (CKI) protects AT2 cells, restores alveolar regeneration, and mitigates radiation-induced pulmonary fibrosis. - Source: PubMed
Publication date: 2026/08/15
Liu Yan-LiXu Cheng-YanWang YongLiang Xiao-YanChen Yun - Lung cancer is the second most commonly diagnosed malignancy worldwide and remains the leading cause of cancer-related death. Non-small cell lung cancer (NSCLC) accounts for approximately 80-85% of lung cancers. Given the low rate of early detection and substantial interpatient heterogeneity, management of NSCLC, particularly locally advanced disease, remains challenging. This study aimed to characterize clinical heterogeneity and progression-associated molecular features of locally advanced NSCLC and to identify candidate biomarkers and therapeutic targets. - Source: PubMed
Publication date: 2026/06/24
Tian YuanhuiDeng YanLiao XiaoliYuan ShutaoLi Ke