Murine MPS I: insights into the pathogenesis of Hurler syndrome.

Mucopolysaccharidosis type I (MPS I) is an autosomal recessive disease resulting from deficiency of the lysosomal enzyme alpha-L-iduronidase. A murine model which shows complete deficiency in alpha-L-iduronidase activity has been developed and shows phenotypic features similar to severe MPS I in humans. Here we report on the long-term clinical, biochemical, and pathological course of MPS I in mice with emphasis on the skeletal and central nervous system (CNS) manifestations. Affected mice show a progressive clinical course with the development of coarse features, altered growth characteristics and a shortened life span. Progressive lysosomal accumulation is seen in all tissues. Skeletal manifestations represent the earliest clinical finding in MPS I mice with histologic analysis of growth plate and cortical bone revealing evidence that significant early pathology is present. Analysis of the CNS has revealed the novel finding of progressive neuronal loss within the cerebellum. In addition, brain tissue from MPS I mice shows increased levels of GM2 and GM3 gangliosides. This murine model clearly shows phenotypic and pathologic features which mimic those seen in severe human MPS I and should be an invaluable tool for the study of the pathogenesis of generalized storage disorders.

Familial variables and domestic violence.

We investigated variables predictive of domestic violence from 64 intake forms at a battered women's shelter. The batterer, usually the financial provider, used drugs/alcohol. Clients' physical abuse as children was correlated with whether their siblings were abused, and to emotional and sexual abuse. Clients' parents being alcoholic was correlated with spouse abuse between clients' parents and with clients' sexual abuse as a child. Findings support theories viewing domestic violence as a familial pattern. Domestic violence is, unfortunately, a common place occurrence. Statistics compiled by the Arkansas Coalition Against Violence to Women and Children indicate that domestic violence is the major cause of injuries to women, exceeding automobile accidents, muggings, and rapes combined. National statistics (Healthy People 2000, 1990) reveal that "between 2 and 4 million women are physically battered each year by partners" (p. 61), with some 21 to 30% of women having been beaten by a partner at least once (Healthy People 2000, 1990). Moreover, domestic violence occurs at least once in up to half of all marriages. In fact, domestic violence is the most frequently experienced type of violent crime (Northeast Arkansas Council..., 1994). This violence also extends to children. For example, Healthy People 2000 indicated that "In 1986 an estimated 1.6 million children nationwide experienced some form of abuse or neglect" (p. 61), with physical abuse being the most common type of abuse. From battered women seeking assistance, data have been gathered on both victims and abusers. Although battered women can come from different backgrounds, commonalities exist.(ABSTRACT TRUNCATED AT 250 WORDS)

Cognitive strategies of university athletes.

Thirty-six college basketball players and skiers, both men and women, were interviewed about their use of cognitive processes to prepare themselves for competition. The interviews examined cognitive techniques for enhancing motor skills and developing competitive strategies. During the interviews the subjects engaged in some of their cognitive preparation strategies and then described the details of these experiences. All athletes reported that the use of cognitive strategies enhanced their performance. Their imagery was rich, detailed, and multisensory. An average of three sensory modalities were present in the imagery. Vision, kinesthesis, and touch were the most common sensory experiences reported, but audition, taste, and smell were also experienced by some athletes. Strong affective states, especially confidence and satisfaction, accompanied the imagery. Individual differences in the imagery used by the athletes were also found and may be related to individual differences in the athletes' cognitive styles.