Tbx4 Antibody - N-terminal region (ARP33740_P050)
- Known as:
- Tbx4 Antibody - N-terminal region (ARP33740_P050)
- Catalog number:
- arp33740_p050
- Product Quantity:
- USD
- Category:
- -
- Supplier:
- Aviva Systems Biology
- Gene target:
- Tbx4 Antibody - N-terminal region (ARP33740_P050)
Related genes to: Tbx4 Antibody - N-terminal region (ARP33740_P050)
- Gene:
- TBX4 NIH gene
- Name:
- T-box 4
- Previous symbol:
- -
- Synonyms:
- -
- Chromosome:
- 17q23.2
- Locus Type:
- gene with protein product
- Date approved:
- 1999-04-16
- Date modifiied:
- 2019-04-23
Related products to: Tbx4 Antibody - N-terminal region (ARP33740_P050)
Related articles to: Tbx4 Antibody - N-terminal region (ARP33740_P050)
- Fibroblasts in the lung mesenchyme produce growth factors and extracellular matrix components that guide formation of distal airspaces during the saccular stage of lung development. Inflammation in preterm infants disrupts this process, leading to bronchopulmonary dysplasia (BPD). To examine how mesenchymal inflammation contributes to BPD pathogenesis, we developed a transgenic mouse model ("IKKβTbx4") in which expression of activated human IκB kinase beta (IKKβ), an upstream activator of NF-κB, was induced in Tbx4 lung enhancer-positive mesenchymal cells during the saccular stage of lung development (postnatal day 0 [PN0] - PN5). Saccular stage IKKβTbx4 mice exhibited a BPD-like phenotype with interstitial thickening and reduced distal airspaces at PN5, progressing to emphysematous enlargement of the distal lung at 2 mo of age. Mesenchymal NF-κB activity upregulated the chemokines CCL2 and CCL7, recruiting CCR2pos monocyte-derived macrophages to the lung. Recruited macrophages disrupted the elastin scaffold and impaired microvascular organization with reductions in CAP2 endothelial cells (aCaps) and pericytes. Blocking CCR2-dependent monocyte recruitment with a small molecule CCR2 antagonist rescued the abnormal lung phenotype. These findings identify mesenchyme-macrophage crosstalk as a mechanism by which inflammation disrupts saccular stage lung development, suggesting a role for this signaling axis in BPD pathogenesis. - Source: PubMed
Publication date: 2026/06/02
Crawford Benjamin CLee Jessica ChauviereElias Bertha CDave Shivangivan der Meer RietHan WeiSharkey Alexandria LNichols David SShissias CharlesPate LaurenTan HaydenNewcomb Dawn CShi WeiPrince Lawrence SPlosa Erin JRichmond Bradley WBlackwell Timothy SGuttentag Susan HBenjamin John T - Pulmonary arterial hypertension (PAH) is a life-threatening disease of the pulmonary arteries. Progressively increasing pulmonary artery pressure may lead to fatal failure of the right ventricle, necessitating aggressive drug treatment. Sotatercept, a novel drug for PAH-targeted therapy, shows promising results in clinical trials. Nevertheless, efficacy in the heterogeneous PAH population and long-term outcomes have yet to be investigated. This case report illustrates the life-changing effects of sotatercept in a patient with heritable PAH associated with a TBX4 mutation. Our patient faced near immobility, oxygen dependency, and was listed for lung transplantation in November 2023. Following sotatercept administration from July 2024 onward, she started sporting activities and has been removed from the transplantation waiting list. Thereby, this case illustrates an exceptional response to sotatercept in a patient with TBX4-associated heritable PAH. Positive effects persist 1 year after initiation without significant side effects. - Source: PubMed
Publication date: 2026/05/15
Schneijdenberg Jop Wvan den Bosch Annemien EKap Miriam EKauling Robert MChandoesing Prewesh Pvan den Toorn Leon MMathot Bas JKoudstaal ThomasBoomars Karin A - To determine the prevalence and phenotypes of children with heritable pulmonary arterial hypertension (PAH) and those with pulmonary hypertension (PH) associated with genetic disorders. - Source: PubMed
Publication date: 2026/04/21
Grynblat JulienEyries MélanieAmbar-Akkaoui MarineLevy MarilyneMeot MathildeSzezepanski IsabelleRanchoup JulienCallegari AlessiaKarila-Cohen JulieBonnet CarolineMarijon PierreChamp JeromeCoulet FlorenceOvaert CarolinePerros FrédéricAntigny FabriceMaragnes PascaleVaksmann GuyHumbert MarcMalekzadeh Milani Sophie GuitiMontani DavidBonnet Damien - Pathogenic variants involving the transcription factor TBX4 gene have been associated with various skeletal and pulmonary abnormalities, including lethal lung developmental disorders (LLDD). - Source: PubMed
Publication date: 2026/04/30
Pande Shruti AChan Joiner HiulingSzafranski PrzemyslawGambin TomaszWright MichelleWang QianWalters MaiahFriedman Jan MSaunders JessicaAvdimiretz NicholasBoerkoel Cornelius FCortes-Santiago NahirDeutsch GailStankiewicz Pawel - Heterozygous TBX4 variants are the second most common genetic cause of pediatric pulmonary hypertension (PH), yet mechanisms underlying TBX4-related lung disease remain poorly understood. This study developed a lung mesenchyme-specific Tbx4 loss-of-function (Tbx4cKO) mouse model that bypasses embryonic lethality to investigate this condition. Adult Tbx4cKO mice demonstrated significantly impaired pulmonary flow acceleration consistent with PH. Three-dimensional analysis of embryonic lungs revealed reduced lobe volumes and decreased distance between pleural edges and muscularized vessels. In adult Tbx4cKO lungs, we identified extensive vascular remodeling characterized by medial thickening and the extension of muscularized arteries into normally non-muscularized subpleural parenchymal zones. Contrary to previous reports suggesting vascular simplification, three-dimensional analysis demonstrated an elaborated pulmonary artery (PA) tree in addition to pathologic wall muscularization. Depletion of a single Tbx5 allele in addition to both Tbx4 alleles exacerbated histologic phenotypes with worsened right ventricular dilation. This model also demonstrated dysregulated airway smooth muscle patterning and prominent subpleural smooth muscle bands, similar to those in human TBX4 syndrome. We identify TBX4 as a critical regulator of smooth muscle differentiation and patterning across multiple lung compartments. Our model recapitulates key features of human TBX4 syndrome and identifies dysregulated smooth muscle differentiation as a potential future therapeutic target. - Source: PubMed
Publication date: 2026/04/23
Steffes Lea CChiles Kaylie AMasud Sehar RRahman AleenDawson MadelineGalambos CsabaKumar Maya EArora Ripla