A5017663247- Lymphatic System and Diseases
- Genetic and Kidney Cyst Diseases
- Cell death mechanisms and regulation
- Renal and related cancers
- Hedgehog Signaling Pathway Studies
- Endoplasmic Reticulum Stress and Disease
- CRISPR and Genetic Engineering
- Lymphatic Disorders and Treatments
- Single-cell and spatial transcriptomics
- Renal Transplantation Outcomes and Treatments
- NF-κB Signaling Pathways
- Ethics and Legal Issues in Pediatric Healthcare
- Systemic Sclerosis and Related Diseases
- Neonatal Respiratory Health Research
- Renal Diseases and Glomerulopathies
- Epigenetics and DNA Methylation
- Genomics, phytochemicals, and oxidative stress
- Adenosine and Purinergic Signaling
- Pediatric Urology and Nephrology Studies
- Planarian Biology and Electrostimulation
University College London
2022-2025
Great Ormond Street Hospital
2022-2025
Newcastle University
2021-2022
Abstract Polycystic kidney diseases (PKD) are genetic disorders which disrupt architecture and function. Autosomal recessive PKD (ARPKD) is a rare form of PKD, caused by mutations in PKHD1 , clinically more severe than the common autosomal dominant (ADPKD). Prior studies have implicated Hedgehog (Hh) signaling ADPKD, with increased levels Hh components experimental ADPKD reduced cystogenesis following pharmacological inhibition. In contrast, role pathway ARPKD poorly understood. We...
Background and Aims: Hepatocellular carcinoma (HCC) is a leading cause of cancer‐related death. The NF‐κB transcription factor family subunit c‐Rel typically protumorigenic; however, it has recently been reported as tumor suppressor. Here, we investigated the role in HCC. Approach Results: Histological transcriptional studies confirmed expression human patients with HCC, but low correlated increased cell proliferation mutational burden was associated advanced disease. In vivo , global ( Rel...
ABSTRACT Studies of the structural and molecular features lymphatic vasculature, which clears fluid, macromolecules leukocytes from tissue microenvironment, have largely relied on animal models, with limited information in human organs beyond traditional immunohistochemical assessment. Here, we use three-dimensional imaging single-cell RNA-sequencing to study lymphatics kidney. We found a hierarchical arrangement vessels within kidneys, initiating along specialised nephron epithelium renal...
Lymphatic vessels are complex three-dimensional (3D) structures that facilitate tissue fluid clearance and regulate immune responses in health inflammatory contexts. Recent advances wholemount immunolabelling 3D imaging have provided insights into organ-specific heterogeneity of lymphatic structure function. However, the visualisation deep within an intact organ remains a challenge. We hypothesised nanobodies, single-domain antibodies raised camelid species, would result improved labelling...
Polycystic kidney diseases (PKD) are genetic disorders characterised by the formation of fluid-filled cysts, which disrupt architecture and function. Autosomal recessive PKD (ARPKD) is a rare form PKD, caused mutations in PKHD1, clinically more severe than common autosomal dominant (ADPKD). Prior studies have implicated ciliary-located Hedgehog (Hh) pathway ADPKD, with increased levels Hh components experimental ADPKD models, reduced cystogenesis following pharmacological inhibition. In...
Abstract The product encoded by the X-linked inhibitor of apoptosis (XIAP) gene is a multi-functional protein which not only controls caspase-dependent cell death, but also participates in inflammatory signalling, copper homeostasis, response to hypoxia and control migration. Deregulation XIAP, either elevated expression or inherited genetic deletion, associated with several human disease states. Reconciling XIAP-dependent signalling pathways its role progression essential understand how...
Abstract The product encoded by the X-linked inhibitor of apoptosis (XIAP) gene is a multi-functional protein which not only controls caspase-dependent cell death, but also participates in inflammatory signalling, copper homeostasis, response to hypoxia and control migration. Deregulation XIAP, either elevated expression or inherited genetic deletion, associated with several human disease states. Reconciling XIAP-dependent signalling pathways its role progression essential understand how...