AEBP1 antibody
- Known as:
- AEBP1 (anti-)
- Catalog number:
- orb10059
- Product Quantity:
- EUR
- Category:
- -
- Supplier:
- Biorbyt biorb
- Gene target:
- AEBP1 antibody
Related genes to: AEBP1 antibody
- Gene:
- AEBP1 NIH gene
- Name:
- AE binding protein 1
- Previous symbol:
- -
- Synonyms:
- ACLP
- Chromosome:
- 7p13
- Locus Type:
- gene with protein product
- Date approved:
- 1998-03-06
- Date modifiied:
- 2016-10-05
Related products to: AEBP1 antibody
Related articles to: AEBP1 antibody
- Persistent activation of cardiac fibroblasts into myofibroblasts drives excessive extracellular matrix deposition, leading to maladaptive myocardial fibrosis, adverse remodeling and heart failure (HF) progression. Adipocyte enhancer binding protein 1 (AEBP1) and Aortic carboxypeptidase-like protein (ACLP, full-length AEBP1 protein coded by ) has been implicated in pathological fibrosis across multiple organs, with tissue-specific knockdown attenuating fibrosis in preclinical models. Although elevated expression has been associated with human HF, its role in myocardial fibrosis progression in vivo remains undefined. Here, we demonstrated that is a critical mediator of myocardial fibrosis and adverse cardiac remodeling. Fibroblast-specific knockout and cardiac-specific knockdown of si gnificantly improved cardiac function and prevented pathological remodeling in murine models of myocardial ischemia and pressure-overload induced injury. In ex vivo human myocardial tissue culture studies, ACLP overexpression in non-failing hearts induced pathological remodeling, whereas knockdown in failing human hearts induced structural reverse remodeling. Mechanistically, we also showed that ACLP regulates key pro-fibrotic transcription factors and genes, including MRTFB, RUNX2, SM22 and COL1A1, thereby orchestrating fibroblast activation. Collectively, these findings establish as a central driver of myocardial fi brosis and highlights its inhibition as a promising therapeutic strategy to mitigate both acute and chronic HF. - Source: PubMed
Publication date: 2026/05/26
Drakos StavrosShankar Thirupura SundariVisker JosephAmrute JunedhPolishchuk GeorgiyLunde TyLing JingFerrin PeterHamouche RanaFeigle DominikCalder DallenSachse FrankKyriakopoulos ChristosManeta EleniTseliou EleniNavankasattusas SutipSelzman CraigSeidel ThomasLavine Kory - Ehlers-Danlos syndrome classical-like type 2 (clEDS2) is a rare autosomal recessive connective tissue disorder caused by biallelic loss-of-function variants in the gene encoding adipocyte enhancer-binding protein 1 (AEBP1). While cutaneous and skeletal manifestations are commonly observed, gastrointestinal complications, including bowel rupture, have been reported only rarely, and their histopathological basis remains poorly characterized. Here, we report findings of molecular investigations, gastrointestinal histopathological evaluation, and long-term clinical follow-up in the 16th reported patient with AEBP1-related clEDS2, complicated by spontaneous bowel perforation. A homozygous AEBP1 splice-site variant (NM_001129.5:c.1401-2 A > G) was identified by a custom next-generation sequencing-based panel analysis for hereditary connective tissue disorders. Transcript-level analysis by reverse transcription-polymerase chain reaction and Sanger sequencing demonstrated complete skipping of exon 12, resulting in a frameshift and predicted loss of function. Clinically, the patient experienced postoperative perforation in the sigmoid colon shortly after rectal cancer surgery, followed nearly 20 years later by spontaneous small intestinal perforation. Histopathological examination of the affected colonic tissue demonstrated mildly widened intermuscular spaces without other overt structural abnormalities. Notably, repeated upper and lower gastrointestinal endoscopic procedures were performed during long-term oncological surveillance without procedure-related bowel perforation. This observation could offer an opportunity to learn about gastrointestinal involvement in AEBP1-related clEDS2 and suggests that its gastrointestinal manifestations may differ in clinical context from those typically associated with vascular Ehlers-Danlos syndrome, warranting further investigation as additional cases are accumulated. - Source: PubMed
Publication date: 2026/05/26
Nakahara HikaruYamaguchi TomomiNiitsu HiroakiAbe AkikoHayashi RyoheiOka ShiroArihiro KojiKosho TomokiHinoi Takao - Endometriosis is characterized by progressive fibrosis and limited therapeutic options. Cuproptosis, a copper-dependent form of regulated cell death, has been implicated in multiple pathological conditions, but its relevance to fibroblast-mediated fibrotic progression in endometriosis remains unclear. Single-cell RNA sequencing data from normal, eutopic, and ectopic endometrial tissues were analyzed to assess cuproptosis-related gene (CRG) activity and fibroblast heterogeneity. Pseudotime analysis, cell-cell communication analysis and high-dimensional weighted gene co-expression network analysis were performed to identify disease-associated fibroblast states and candidate fibrosis-related genes. Machine learning approaches were applied to prioritize candidate hub genes. Functional validation was conducted in endometrial stromal cells, and a mouse model of endometriosis was used to assess the effects of tetrathiomolybdate (TTM), a copper chelator. Elevated CRG activity was enriched in a distinct fibroblast subpopulation with profibrotic transcriptional features. Network and machine learning analyses consistently prioritized AEBP1 as a candidate fibroblast-associated hub gene linked to cuproptosis-related signatures. In vitro, CuCl plus elesclomol treatment was associated with increased AEBP1 and fibrosis-related marker expression, accompanied by changes in β-catenin pathway-related proteins, whereas FDX1 or AEBP1 knockdown attenuated these effects. In vivo, TTM treatment reduced lesion burden, fibrotic marker expression and collagen deposition in ectopic lesions. Cuproptosis-related molecular alterations are associated with fibroblast activation and fibrotic progression in endometriosis. Targeting copper metabolism may have therapeutic potential in limiting lesion fibrosis. - Source: PubMed
Publication date: 2026/05/18
Huang ErqingLi Jiang-TianZuo DanhuiLi RuijieWu QingyueLin NaZhao JinruWang HuajingLiu YiZhang Ling - Proliferative vitreoretinal diseases (PVDs) encompass severe ocular disorders such as proliferative vitreoretinopathy (PVR), proliferative diabetic retinopathy (PDR), and epiretinal membranes (ERM), characterized by the formation of fibrovascular membranes that often lead to retinal detachment and vision loss. A central mechanism driving these conditions is the epithelial-to-mesenchymal transition (EMT) of retinal pigment epithelial (RPE) cells, orchestrated by a network of transcription factors (TFs). Among these, zinc finger E-box binding homeobox 1 (ZEB1) emerges as a pivotal regulator by repressing epithelial markers like E-cadherin and inducing mesenchymal markers such as N-cadherin and vimentin, thereby promoting cell migration and fibrotic membrane formation. nuclear factor of activated T cells 5 (NFAT5) contributes to this process by mediating osmotic stress responses and upregulating inflammatory cytokines, which further act upon EMT and fibrosis. activator protein 1 (AP-1) and hypoxia inducible factor 1 subunit alpha (HIF-1α) participate in driving inflammation, extracellular matrix (ECM) remodeling, and angiogenesis. While HIF-1α triggers vascular endothelial growth factor (VEGF) expression under hypoxic conditions, AP-1 modulates matrix metalloproteinases (MMPs) essential for ECM degradation and remodeling. Additional TFs, including Kruppel-like factor 4 (KLF4) and microphthalmia-associated transcription factor (MITF), are vital in maintaining RPE cell identity. Their downregulation under pathological conditions disrupts epithelial integrity and predisposes cells to undergo EMT. Moreover, β-catenin, through its role in the wingless-related integration site (Wnt) signaling pathway, reinforces EMT and ECM remodeling, further enhancing fibrotic progression. Adipocyte enhancer-binding protein 1 (AEBP1) and ZFP36 ring finger protein like 1 (ZFP36L1) also regulate inflammatory responses and ECM dynamics, providing novel post-transcriptional targets for therapeutic intervention. Overall, the synergistic interactions among these TFs create complex feedback loops that amplify pathological changes in PVDs. Targeting these molecular pathways offers promising avenues for developing multi-targeted therapies aimed at saving vision-threatening disease while reducing invasive surgical interventions. - Source: PubMed
Publication date: 2026/05/08
Duveau ClémentRaiss El Harrak YosraDatlibagi AzinePerret JasonWillermain FrançoisDelporte ChristineMotulsky Elie - Endometriosis (EMs) is a chronic gynaecological condition characterised by the ectopic growth of endometrial tissue; however, its molecular mechanisms remain insufficiently understood. Ferroptosis, an iron-dependent form of regulated cell death, has been suggested as a potential contributor to its pathogenesis. This study aimed to identify differentially expressed ferroptosis-related genes (DE-FRGs) in EMs through bioinformatics analysis and to explore their underlying molecular mechanisms. - Source: PubMed
Publication date: 2026/04/30
Xu Jia-YanLiu Qin