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Behavioral inhibition refers to a temperament or style of reacting that some infants and young children exhibit when confronted with novel situations or unfamiliar adults or peers. Research on behavioral inhibition has examined the link between this set of behaviors to the neural systems involved in the experience and expression of fear. There are strong(More)
Gene-environment interactions are presumed to shape human behavior during early development. However, no human research has demonstrated that such interactions lead to stable individual differences in fear responses. We tested this possibility by focusing on a polymorphism in the promoter region of the gene for the serotonin transporter (5-HTT). This(More)
Li Fraumeni Syndrome (LFS) is a multicancer phenotype, most commonly associated with germ-line mutations in TP53. In a kindred with LFS without an inherited TP53 mutation, we have previously reported a truncating mutation (1100delC) in CHK2, encoding a kinase that phosphorylates p53 on Ser(20). Here, we describe a CHK2 missense mutation (R145W) in another(More)
In this comprehensive study of the early development of guilt, 106 children were observed in laboratory paradigms in which they were led to believe that they had damaged valuable objects, during two separate sessions at each of the assessments at 22, 33, and 45 months. The behavioral and affective components of guilt cohered significantly across the(More)
Parental power assertion is traditionally studied in the behavioral domain--discipline triggered by the child's immediate misbehavior--but rarely in the cognitive domain--parent-child discussions of the child's past misbehavior. Maternal power assertion was observed in "do" and 'don't" discipline contexts from 14 to 45 months and in the context of(More)
Recent studies have attempted to understand the processes involved in joint attention because of its relevance to both atypical and normal development. Data from a recent study of young children with autism suggests that performance on a delay nonmatch to sample (DNMS) task associated with ventromedial prefrontal functions, but not an A-not-B/delayed(More)
The hemophagocytic lymphohistiocytoses (HLH) comprise a heterogeneous group of disorders characterized by dysregulated activation of T cells and macrophages. Although some patients with HLH harbor perforin gene mutations, the cause of the remaining cases is not known. The phenotype of HLH bears a strong resemblance to X-linked lymphoproliferative disease(More)
Social communication is an important skill that emerges during infancy. We examined individual differences in this skill as a function of temperament and neural activity in nine-month-old infants. We found that maternal ratings of temperament were associated with joint attention, an important index of early social communication. More specifically, maternal(More)
The Wilms' tumor suppressor gene WT1 encodes a zinc finger transcription factor, whose expression inhibits the growth of the RM1 Wilms' tumor cell line. Transient transfection of WT1 constructs into 3T3 or 293 cells results in transcriptional repression of a number of cotransfected promoters containing the early growth response gene 1 consensus sequence. We(More)
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