GLI1 Antibody
- Known as:
- GLI1 Antibody
- Catalog number:
- GWB-90B2E7
- Product Quantity:
- 0.1 mg
- Category:
- -
- Supplier:
- GenWay
- Gene target:
- GLI1 Antibody
Related genes to: GLI1 Antibody
- Gene:
- GLI1 NIH gene
- Name:
- GLI family zinc finger 1
- Previous symbol:
- GLI
- Synonyms:
- -
- Chromosome:
- 12q13.3
- Locus Type:
- gene with protein product
- Date approved:
- 1986-01-01
- Date modifiied:
- 2016-01-15
Related products to: GLI1 Antibody
Related articles to: GLI1 Antibody
- Carpenter syndrome, caused by biallelic mutations in MEGF8 or RAB23, manifests with craniosynostosis through incompletely defined mechanisms. While both genes encode negative regulators of Hedgehog (Hh) signaling, we demonstrate that MEGF8 maintains cranial suture patency through a distinct, Hh-independent pathway. Loss of MEGF8 disrupts ubiquitination and lysosomal degradation of BMPR1A, leading to BMPR1A accumulation and hyperactivation of canonical BMP-SMAD1/5/9 signaling, which accelerates osteogenic differentiation of cranial mesenchyme. Using Megf8 mutant mice, we show tissue-specific specialization: limb defects are Hh-dependent and rescued by SMO inhibition, whereas craniosynostosis is BMP-driven and refractory to Hh blockade, with BMP type I receptor inhibition selectively rescuing the cranial phenotype. Comparative analyses reveal that MEGF8 and RAB23 promote osteogenic differentiation through distinct mechanisms-MEGF8 via ubiquitin-mediated BMPR1A turnover and BMP-SMAD activation, RAB23 through FGF-ERK signaling-despite both affecting GLI1-mediated transcription. Reintroduction of human MEGF8 in MEGF8-knockdown cells restores BMPR1A protein levels, validating the specificity of MEGF8-mediated BMPR1A regulation. These findings suggest that MEGF8 modulates BMP signaling post-transcriptionally, establishes tissue-specific regulatory mechanisms in syndromic disorders, and demonstrates how divergent pathways converge on shared phenotypes, with implications for pathway-specific therapeutic strategies. - Source: PubMed
Publication date: 2026/07/03
Hwangbo KoeunPark JihyunRho HyunjinWoo Dong-CheolSung Young HoonKim Soo-HyunSong JaewhanKo Hyuk Wan - The emphysematous phenotype is an important phenotype in chronic obstructive pulmonary disease (COPD), with substantial morbidity and mortality. The mechanisms underpinning the role of alveolar type II (AT2) cells in alveolar repair within this phenotype remain poorly understood. This study aimed to elucidate the role of PHLDA1, a potential stemness regulator in AT2 cells, on emphysema development. Utilizing mice model, we performed a targeted knockdown of PHLDA1 in AT2 cells and subsequently exposed these mice to tobacco smoke to assess the resultant severity of emphysema and related alveolar damage. We manipulated PHLDA1 expression in AT2 cells line or primary mouse AT2 cells to examine its influence on AT2 stemness-related processes- differentiation, proliferation, and wound closure ability. The specific pathway of PHLDA1 mediated in AT2 cells, as well as its interaction with the GLI1 protein, was further investigated. Mice with reduced PHLDA1 expression developed the emphysema independent of smoking exposure. PHLDA1 knockdown in AT2 cells attenuated their proliferation via the Hedgehog pathway, impairing wound closure ability in the emphysematous phenotype. We also discovered a binding relationship between PHLDA1 and GLI1, where PHLDA1 modulates the nuclear translocation of GLI1, thus regulating the Hedgehog pathway and influencing the stemness and proliferation of AT2 cells. Our study suggests that PHLDA1 is a critical factor in the proliferation process of AT2 cells via modulation of GLI1 nuclear translocation. This regulation is essential to the pathogenesis of the emphysematous phenotype in COPD, signifying potential therapeutic targets for intervention. - Source: PubMed
Publication date: 2026/07/04
Bai ShuangWang ShuaifuYe RuiWang DiMa MingxinZhao Li - - Source: PubMed
Zhang M NZhang X FJiang RZhao Z WZhao J JZhang HLi BDai Y F - Gastroblastoma is a malignant epithelial tumor of the stomach, which is primarily diagnosed based on its biphasic histomorphology and the demonstration of its characteristic molecular fusion gene, MALAT1-GLI1. Variability in the histomorphology has not been studied so far owing to its rarity. With very few cases reported in the literature, its long-term course is not entirely understood, with most of the cases treated based on its stage. - Source: PubMed
Publication date: 2026/06/30
Rajagopal LakshmiDathan Neetu SAbraham RachelShabeerali T U - Aberrant activation of the Sonic Hedgehog (SHH)-glioma-associated oncogene 1 (GLI1) signaling pathway is a determinant driver of basal cell carcinoma (BCC) and SHH-subgroup medulloblastoma (MB). Here, we demonstrate the role of S-palmitoylation and depalmitoylation of GLI1 in the regulation of incidence and progression of MB. High expression of acyltransferase ZDHHC13 in MB induces S-palmitoylation of human GLI1 at Cys1034, stabilizes GLI1 by preventing its ubiquitin-dependent proteasomal degradation, and in turn activates this signaling pathway to promote the progression of MB. Conversely, the deacylase ABHD17A depalmitoylates GLI1 at Cys1034, destabilizes GLI1, and in turn inactivates this signaling pathway. As a result, conditional knockout of Zdhhc13 in cerebellar granule neuron precursors (GNPs) significantly alleviates the severity of MB induced by constitutively active Smoothened (Smo-M2) overexpression and markedly extends overall survival, whereas knockout of Abhd17a increases the incidence of MB induced by Patched-1 ablation, rather than its progression. Together, these findings uncover the dynamic regulation of GLI1 palmitoylation and depalmitoylation by the ZDHHC13 and ABHD17A and subsequent GLI1 stabilization and destabilization as a hitherto uncharacterized mechanism controlling SHH/GLI1 signaling and may provide additional targets for therapeutic intervention of MB. - Source: PubMed
Publication date: 2026/06/29
Shen TingyuBao HangyangWang JirongJi XingGu WeizhongTang ChaoHe QiangqiangLuo JiahaoTan DanYao JialiXu ChengyunGong YingWu XimeiZhang Shi-HongZeng Ling-Hui