P4HB protein
- Known as:
- P4HB protein
- Catalog number:
- 80R-4394
- Product Quantity:
- 50 ug
- Category:
- -
- Supplier:
- Fitzgerald
- Gene target:
- P4HB protein
Related genes to: P4HB protein
- Gene:
- P4HB NIH gene
- Name:
- prolyl 4-hydroxylase subunit beta
- Previous symbol:
- PO4DB, ERBA2L
- Synonyms:
- PDIA1, PROHB, DSI, GIT, PDI, PO4HB, P4Hbeta
- Chromosome:
- 17q25.3
- Locus Type:
- gene with protein product
- Date approved:
- 1986-01-01
- Date modifiied:
- 2016-10-05
Related products to: P4HB protein
Related articles to: P4HB protein
- Prions are misfolded, self-propagating versions of cellular proteins. In humans and animals, misfolding of the cellular prion protein (PrPC) causes invariably fatal transmissible neurodegenerative diseases. Little is known about how mammalian prions replicate in the brain, including whether other proteins participate in prion replication in vivo. Several members of the protein disulfide isomerase family have been shown to reside in close spatial proximity to PrPC in cells and mice, implying that they could be involved in prion biogenesis. Here, we show that stable knock-down of the protein disulfide isomerase P4HB (also called PDIA1) in prion-susceptible CAD5 cells reduces PrPC levels and hinders the generation of protease-resistant PrP (PrPres) following infection with two different prion strains. Moreover, transient knock-down of P4HB decreases PrPres levels in cells with established prion infection. Partial reduction of P4HB activity using the P4HB-selective inhibitor KSC-34 also decreases PrPC levels in uninfected CAD5 cells whereas treatment of prion-infected CAD5 cells with KSC-34 results in higher levels of PrPres. A proportion of P4HB reaches the cell surface where PrPC is located, and a secreted P4HB variant increases PrPres levels in cells. Collectively, these results suggest that P4HB influences PrPC homeostasis and modulates the conversion of PrPC into misfolded species. Thus, targeting P4HB during prion disease may have therapeutic benefit. - Source: PubMed
Publication date: 2026/08/18
Amano GenkiArshad HamzaPatel ZeelSchmitt-Ulms GeroldWatts Joel C - Regulatory T cells (Tregs) may influence prognosis in leukemia. This study aimed to explore the effects and mechanisms of Treg subclusters on the prognosis in B-cell acute lymphoblastic leukemia (B-ALL) using single-cell RNA sequencing (scRNA-Seq). - Source: PubMed
Publication date: 2026/07/28
Songnan SuiZheng JiamianLai JingZhang YupeiJiang XuXu LingZhong LiyeChen JieWu JieZhu XunLi YangqiuWu GuocaiChen Zhanghui - Fibrotic scar formation constitutes a significant pathological obstacle that impedes neural regeneration and long-term functional recovery following spinal cord injury (SCI). However, the spatial distribution of key pro-fibrotic mediators within lesion scars and the upstream regulatory mechanisms driving fibroblast activation remain inadequately defined. This study aims to characterize CD36-associated fibrotic remodeling after SCI and to determine whether targeting the c-Jun-Irf8-CD36 axis could attenuate scar formation, improve the regenerative microenvironment, and promote functional recovery. - Source: PubMed
Publication date: 2026/03/12
Feng MinLi JiYuZhang JieyingZhang HangLi WanhuaLiu YiliGao XiangYang HanyueLiu ZeyuGao ChongXiao RuiyangXiao JianruZhou XiaozhongGong LeileiZhou SonglinZhao JianCheng Caiqi - Cole-Carpenter syndrome (CCS) is a rare autosomal dominant skeletal disorder characterized by severe bone fragility, recurrent fractures, craniosynostosis, and distinctive craniofacial abnormalities. Pathogenic variants in P4HB, encoding protein disulfide isomerase A1 (PDIA1), represent the most frequent cause of CCS. The recurrent missense mutation p.Tyr393Cys (Y393C) has been identified in unrelated patients, yet the in vivo consequences of this mutation on skeletal biology and its therapeutic implications remain poorly understood. Here, we generated a mouse model carrying the mouse homolog P4hb mutation. Comprehensive skeletal phenotyping revealed pronounced osteopenia and reduced local bone quality in mutant mice across multiple ages. Consistently, CCS mice exhibited reduced circulating levels of procollagen type I N-terminal propeptide (PINP I), indicating decreased type I collagen biosynthesis. At the cellular level, primary osteoblasts isolated from CCS mice showed lower expression and secretion of type I collagen, along with decreased expression of osteocalcin and higher mineralization. On the other hand, osteoclast differentiation was increased. Given the lack of disease-modifying therapies for CCS, we explored different therapeutic strategies. We performed a functional screen of an FDA-approved drug library identifying four compounds that significantly enhanced type I collagen secretion. Finally, we developed an allele-specific RNA interference (siRNAs) approach and identified siRNA sequences capable of selectively silencing the mutant P4hb allele in vitro and ex vivo without cytotoxic effects. Collectively, our study establishes a robust preclinical model for P4hb-related CCS, defines defective type I collagen biosynthesis as a central pathogenic mechanism, and identifies promising therapeutic strategies with translational potential for this currently untreatable skeletal disorder. - Source: PubMed
Publication date: 2026/07/09
Maurizi AntonioPucci ElisaPatrizii PiergiorgioSilvestri LucianaRucci NadiaCapulli Mattia - Lung cancer, with high death rate and incidence rate, is one of the top three lethal cancers worldwide. Present therapies can bring little benefit for patients with recurrence and metastasis, which is due to the heterogeneity and complicated mechanism underlying lung cancer. PDIA2 is reported to promote cancer progression. However, its role in lung cancer is not clear. According to the TCGA, PDIA2 was expressed much higher in lung cancer tissues (n = 515) than that in normal tissues (n = 59). And the survival probability of patients with high PDIA2 expression was much worse than that with low PDIA2 expression (p = 0.022). The median survival time of patients with high PDIA2 expression was 65.1 months but it was 75.43 months in patients with low PDIA2 expression. In lung cancer cells, PDIA2 also demonstrated higher expression than that in BEAS-2B cells. When PDIA2 was decreased in lung cancer cells, the ability of cell proliferation and migration was inhibited. In contrast, cell apoptotic rate was increased. However, overexpression of PDIA2 promoted cell proliferation and migration. In addition, the level of the MT membrane potential was decreased significantly while the ROS level was increased. Moreover, PDIA2 knockdown caused significant decrease of critical members in JNK signaling pathway such as JNK, c-Jun, and smad4 while PDIA2 overexpression exhibited reversed effects. And the expression levels of cleaved caspase-3, bax, and bcl-2 were affected by PDIA2. These data suggest that PDIA2 regulated both the JNK signaling and caspase-3 cascade response in lung cancer. In addition, the IC50 of Carboplatin in lung cancer cells was declined when PDIA2 was knocked down, which suggests the increased efficacy of Carboplatin. In summary, PDIA2 contributes to the progression of lung cancer and JNK and caspase-3 signaling pathway play important roles, which proposes PDIA2 as a potential target for therapy of lung cancer. - Source: PubMed
Publication date: 2026/07/07
Fang HuilongPeng ZhongluLi PingHou ChenxiTian JiaxinLuo XingWang JunjieYang ZhiyingHe Dongyang