The MA-10 mouse Leydig tumor cell line produces large amounts of steroids only in response to hormonal stimulation while the R2C rat Leydig tumor cell line is constitutively steroidogenic in nature. In an effort to uncover the potential reasons for constitutive steroidogenesis in R2C cells, we have recently shown that compared to MA-10 cells, R2C cells express much higher levels of the Scavenger Receptor Class B type 1 which results in a higher capacity for cholesteryl ester uptake through the selective uptake pathway. We also found an enhanced expression of Hormone Sensitive Lipase and the Steroidogenic Acute Regulatory protein in these cells and reasoned that they may further facilitate the conversion of cholesteryl esters to free cholesterol and its mobilization to the inner mitochondrial membrane, thus rendering them constitutively steroidogenic. Given the proposed role of the peripheral-type benzodiazepine receptor (PBR) in conferring a constitutively steroidogenic phenotype to the R2C cells, the current study was conducted to investigate the relationship between its expression in MA-10 and R2C cells and correlate it with the constitutive nature of R2C cell steroidogenesis. Our studies show that PBR expression as measured by PK 11195 ligand binding and Western analysis is much higher in MA-10 cells than R2C cells. We also determined that the affinity of ligand binding to the PBR is comparable in the two cell lines, suggesting that PBR is unlikely to be solely responsible for the constitutive nature of R2C cell steroidogenesis.
Seckel syndrome is a very rare form of primordial dwarfism characterized by antenatal and postnatal growth delay, proportionate extreme short stature, a prominent beak-like nose, hypoplasia of the malar area, small chin, microcephaly, deformed ears lacking lobules, skeletal malformations, mental retardation, and developmental delay. This syndrome has been described with associated disorders of orthopedic, neurologic, hematologic, cardiac, and ocular systems; however, only a few reports mention dermatologic involvement. We describe a 5-year-old girl with classic Seckel syndrome who presented with moderately severe atopic dermatitis and diffuse hypopigmented macules and papules.
Oral valacyclovir 1.5 g bid is safe and effective for the treatment of uncomplicated herpes zoster in immunocompetent patients over 18 years of age. Twice-daily dosing may help increase patient compliance and therefore increase the effectiveness of treatment of the acute herpes zoster rash and the prevention of ZAP.
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