Anti-GHR monoclonal antibody, clone C878N

Marjolin's ulcer is an atypical malignancy that develops from deep scars of chronically traumatised skin. Laron syndrome (LS) is a rare autosomal recessive growth retardation from a mutation in the growth hormone receptor (GHR) gene leading to defective GHR, growth hormone insensitivity and eventual low levels of insulin-like growth factor type 1 (IGF-1). Affected individuals present with proportionate dwarfism and other characteristic physical defects, but at the same time are conferred protection against cancer due to low serum levels of IGF-1. We report an exceptional case of Marjolin's ulcer in the foot of a female LS patient 30 years after she sustained flame burns as a 6-month-old baby. Three months before coming to us, she had a 2x3cm ulcer that turned into a rapidly enlarging fungating mass involving the leg, ankle, and foot. Histopathologic analysis of an incision biopsy showed well-differentiated squamous cell carcinoma. The extent of her lesion precluded wide excision. Below knee amputation was done. A second biopsy confirmed the histopathologic diagnosis. This is the first reported case in the literature of Marjolin's ulcer in LS which raises the possibility that IGF-1 deficiency does not completely protect against squamous cell cancer.

Growth hormone (GH) is pituitary derived hormone which acts on most tissues of the body either directly or indirectly and affects many metabolic processes throughout life. Genetically engineered mouse lines have become vital tools for uncovering the various in vivo activities of a GH. A particularly useful mouse line has been the GH receptor (GHR) gene disrupted or knockout (KO) mouse which has been used world-wide in many studies. Recent advances in biotechnology have allowed the development of tissue-specific knockout mouse lines which allows for more direct enquiries on the activities of a given protein in specific tissues or cell types. Accordingly, twenty-two novel tissue-specific GHRKO mouse lines have been developed in the last eleven years. In this paper we provide a detailed list and review the phenotypic changes that occur in each of these tissue-specific GHRKO mouse lines.