Introduction The steroid hormones constitute a very important class of compounds and are
present in most species. The hormones that are synthesized in the adrenal gland, namely …

The rate-limiting, hormone-regulated, enzymatic step in Steroidogenesis is the conversion of
cholesterol to pregnenolone by the cholesterol side-chain cleavage enzyme system …

The gonadotropin FSH plays a key role in the control of Sertoli cell function. The FSH
molecular mechanism of action is best recognized for its stimulation of the adenylyl …

The acute response of steroidogenic cells to trophic hormone stimulation is the mobilization
of cholesterol from cellular stores to the mitochondrial outer membrane and the transfer of …

Congenital lipoid adrenal hyperplasia is an autosomal recessive disorder that is
characterized by impaired synthesis of all adrenal and gonadal steroid hormones. In three …

Quantitative gene expression data are often normalized to the expression levels of control or
so-called “housekeeping” genes. An inherent assumption in the use of housekeeping genes …

Objectives. This study was designed to determine the frequency of 22q11 deletions in a
large, prospectively ascertained sample of patients with conotruncal defects and to evaluate …

Background—There are limited data regarding the long-term survival of patients who have
undergone reconstructive surgery for hypoplastic left heart syndrome (HLHS). We reviewed …

The outcome of the first pilot study of liver-directed gene therapy is reported here. Five
patients with homozygous familial hypercholesterolaemia (FH) ranging in age from 7 to 41 …

An essential component of regulated steroidogenesis is the translocation of cholesterol from
the cytoplasm to the inner mitochondrial membrane where the cholesterol side-chain …