Anti-RAF1 monoclonal antibody, clone pS338 (DPR/2/2A/3A)

Progestin and adipoQ receptor family member III (PAQR3), a member of the PAQR family, is frequently downregulated in different types of human cancer. However, its expression and functions in esophageal cancer are still unknown. This study aimed to explore the expression of PAQR3 in esophageal cancer cell lines and to investigate the role of PAQR3 in the development of esophageal cancer. Our data showed that PAQR3 is expressed in low amounts in human esophageal cancer cell lines. Overexpression of PAQR3 significantly suppressed the proliferation, migration, and invasion of esophageal cancer cells. In addition, overexpression of PAQR3 downregulated the protein expression levels of RAF1, p-MEK1, and p-ERK1/2 in esophageal cancer cells. Furthermore, overexpression of PAQR3 attenuated the tumor growth in a tumor xenograft model. In conclusion, we demonstrated that overexpression of PAQR3 suppresses cell proliferation, migration, and invasion in esophageal cancer in vitro and in vivo. Therefore, PAQR3 may act as a therapeutic target for human esophageal cancer.

Noonan syndrome (NS) is an autosomal dominant disorder, characterized by variable expressivity of clinical features such as: postnatal growth reduction, congenital heart disease, characteristic facial dysmorphisms and development delay. In similar to 75% of all NS cases, germline mutations involving RAS-MAPK signaling pathway genes (PTPN11, SOS1, RAF1, KRAS, NRAS, BRAF, SHOC2, MEK1, CBL) are causative. We reported a case of 13-year-old girl [born at 36w by CS (BW 3250 g (similar to 95 degrees), BL 48 cm (similar to 75 degrees)] referred for genetic counseling due to growth retardation, facial dysmorphisms, development delay and learning disability. After birth she presented frequent vomiting, with failure to thrive and at 5 months of age underwent surgery for intestinal malrotation. Because of short stature, Growth Hormone (GH) therapy have been introduced at age of 3yrs up to 11yrs. Negative molecular testing for PTPN11 and SOS1 genes, normal female karyotype and aCGH analysis were observed. Objective examination: H 138 cm, (<3 degrees); W 33 kg, (<3 degrees), no menarche, hypertelorism, eyelids ptosis with down slanting palpebral fissures, low-set and posteriorly rotated ears, high-arched palate, micrognathia, short and webbed neck, low hairline at the back of the neck, pectus excavatum, prominent scoliosis, joint hyperextensibility, bilateral pes planus and mitral valve prolapse disclosed by US. Phenotype of our patient was suggestive to NS, thus further mutational screening has been requested. Missense mutation in exon 2 of KRAS gene (c.40G>A; p.Val14Ile) has been identified. Even though KRAS mutations are usually associated with NS severe phenotype with cardiac involvement (hypertrophic cardiomyopathy), this finding is not present in our patient.