Rural land mobile radio market assessment and satellite and terrestrial system concepts

Market potential exists; the nature of the market in terms of service needs, usage characteristics, service requirements, and forecasting the demand to the year 2000; alternative system cncepts that show promise in addressing the identified needs, in a cost effective manner; and advanced technology requirements associated with these concepts are considered

Project for the analysis of technology transfer Quarterly report, 1 Jul. - 30 Sep. 1969

Research activities in technology transfer progra

Golabi-Ito-Hall syndrome results from a missense mutation in the WW domain of the PQBP1 gene

Background: Golabi, Ito, and Hall reported a family with X linked mental retardation (XLMR), microcephaly, postnatal growth deficiency, and other anomalies, including atrial septal defect, in 1984.Methods: This family was restudied as part of our ongoing study of XLMR, but significant linkage to X chromosome markers could not be found. Extreme short stature and microcephaly as well as other new clinical findings were observed. Mutations in the polyglutamine tract binding protein 1 gene (PQBP1) have recently been reported in four XLMR disorders (Renpenning, Hamel cerebro-palato-cardiac, Sutherland-Haan, and Porteous syndromes) as well as in several other families. The clinical similarity of our family to these patients with mutations in PQBP1, particularly the presence of microcephaly, short stature, and atrial septal defect, prompted examination of this gene.Results: A missense mutation in PQBP1 was identified which changed the conserved tyrosine residue in the WW domain at position 65 to a cysteine (p.Y65C).Conclusions: This is the first missense mutation identified in PQBP1 and the first mutation in the WW domain of the gene. The WW domain has been shown to play an important role in the regulation of transcription by interacting with the PPxY motif found in transcription factors. The p.Y65C mutation may affect the proper functioning of the PQBP1 protein as a transcriptional co-activator

Simulation studies of air transport operational problems

An experimental evaluation of the monitored approach procedure for conducting low visibility instrument approaches is described. Four airline crews each flew 16 approaches using the monitored procedure and 16 using a modified standard procedure in a DC-10 simulator under various conditions of visibility, wind shear and turbulence, and radar vectoring scenarios. In terms of system measures of aircrew performance, no major differences were found. Pilot opinion data indicate that there are some desirable characteristics of the monitored procedure, particularly with reference to the increased role of the flight engineer in conducting low visibility approaches. Rationale for developing approach procedures is discussed