A novel VCP mutation underlies scapuloperoneal muscular dystrophy and dropped head syndrome featuring lobulated fibers.

Introduction: Valosin-containing protein (VCP) is a ubiquitously expressed, multifunctional AAA-ATPase protein. Its dominant mutations cause hereditary inclusion body myopathy associated with Paget disease of bone and frontotemporal dementia (IBMPFD) or amyotrophic lateral sclerosis. The pattern of muscle weakness in IBMPFD patients is variable and includes limb-girdle, scapuloperoneal, distal, or axial distributions.

Correlations between psychological tests and physiological responses during fear conditioning and renewal.

Background: Anxiety disorders are characterized by specific emotions, thoughts and physiological responses. Little is known, however, about the relationship between psychological/personality indices of anxiety responses to fear stimuli.

Methods: We studied this relationship in healthy subjects by comparing scores on psychological and personality questionnaires with results of an experimental fear conditioning paradigm using a visual conditioned stimulus (CS).

Inhibitory and disinhibitory effects of psychomotor stimulants and depressants on the sexual behavior of male and female rats.

Drugs of abuse comprise several pharmacological classes, including psychomotor stimulants, such as amphetamine and cocaine, and CNS depressants, such as morphine and alcohol. Few studies have examined the effects of those drugs systematically on human sexual behavior, although substantial clinical and epidemiological literatures suggest that drugs in both classes either inhibit sexual responding or can be "prosexual" in certain situations, thereby increasing the potential of risky sexual activity and the spread of sexually transmitted diseases. This paper reviews original data in rats showing that both classes of drug inhibit or disinhibit sexual behavior depending on the animal's baseline level of sexual responding, hormonal status, whether the drug is given acutely or chronically, and whether the animal has learned to inhibit sexual responding toward nonreceptive partners or in the presence of conditioned olfactory cues that predict sexual nonreward.