Endocrine Abstracts

Von-Hippel-Lindau syndrome (VHL) is an autosomal-dominant disorder associated with CNS haemangioblastomas, phaeochromocytomas, renal carcinomas and retinal haemangiomas. We present the case of a 33-year-old woman with VHL who developed clinical progression of a CNS lesion during pregnancy. The patient had undergone removal of a cerebellar haemangioblastoma at age 18, and bilateral phaeochromocytomas were removed at age 27. The only other abnormality on regular review was a mix...

Neuronal glucose and insulin signals are integrated by hypothalamic neurons to regulate energy balance. These signalling mechanisms are not clearly defined in the hypothalamus, and may be altered in obesity and diabetes. This study aimed to analyse quantitatively hypothalamic expression of candidate genes in signal transduction pathways and investigate dysregulation using Zucker and Zucker Diabetic Fatty (ZDF) models.Taqman RT-PCR was used to compare exp...

Peripheral metabolic signals such as leptin, glucose and insulin are integrated by neurons in the hypothalamus to regulate both short and long term energy balance. However, the signal transduction mechanisms by which neurons sense glucose and insulin levels are unclear and specific parts of the pathways may be distinct from those in the pancreas. The aim of this study was to quantitatively analyse hypothalamic expression of genes known to be involved in pancreatic glucose/insu...

Agouti-related protein (AGRP) plays a key role in energy homeostasis with the carboxy-terminal domain acting as an endogenous antagonist of the melanocortin-4 receptor (MC4-R). It has been suggested that the amino-terminal domain of AGRP binds to syndecan-3, allowing it to regulate local concentrations of AGRP at the MC4-R. This model assumes that AGRP is secreted as a full-length peptide. However, AGRP contains several consensus prohormone convertase cleavage sites: Lys52-Lys...