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Biomedical subjects

A B Fulton

Publications and source records attributed to A B Fulton.

At least 19 recordsLinked to original sources

Association of titin and myosin heavy chain in developing skeletal muscle.

To understand molecular interactions that organize developing myofibrils, we examined the biosynthesis and interaction of titin and myosin heavy chain in cultures of developing muscle. Use of pulse-labeling, immunoprecipitation, and a reversible cross-linking procedure demonstrates that within minutes of synthesis, titin and myosin heavy chain can be chemically cross-linked into very large, detergent-resistant complexes retaining many features of intact myotubes. These complexes, predominantly of titin and myosin, occur very early in myofibrillogenesis as well as later. These data suggest that synthesis and assembly of titin and myosin are temporally and spatially coordinated in nascent myofibrils and support the hypothesis that titin molecules help to organize sarcomere formation.

Animals

The effect of light adaptation on scotopic spatial summation in 10-week-old infants.

Psychophysical area-intensity functions of individual 10-week-old human infants and adults were obtained in the dark adapted state, and in the presence of a steady background that elevated threshold 1 log unit above the dark adapted level. For dark adapted infants, the mean diameter for complete spatial summation (4.42 degrees; SD: 1.67 degrees) was significantly larger than that of adults (2.32 degrees; SD: 0.09 degrees). The background reduced the mean critical diameter to 2.67 degrees for infants (SD: 0.64 degrees) and to 1.16 degrees for adults (SD: 0.08 degrees). Spatial probability summation has similar effects on infant and adult thresholds, and, therefore, does not appear to account for the developmental decrease in critical diameters. Rather, decreases in receptive field size are suspected.

Adaptation, Ocular

Effects of halothane on children's electroretinograms.

BACKGROUND: At times, anesthesia is necessary to test children's electroretinographic (ERG) responses. Halothane, an anesthetic commonly used for pediatric patients, affects some aspects of ERG responses, but it is unknown if halothane affects ERG parameters evaluated by contemporary clinical protocols. METHODS: Scotopic and photopic ERG responses were recorded from children when awake and then under halothane. RESULTS: Halothane has no effect on scotopic b-wave stimulus/response parameters, including amplitude, sensitivity, and implicit time. Scotopic a-wave amplitudes, implicit times, model parameters, and ratio of a- to b-wave amplitudes are unaffected by halothane. The amplitudes and implicit times of photopic responses to red flashes and 30 Hz flickering white light are not altered by halothane. Halothane causes no significant change in amplitudes and implicit times of the oscillatory potential wavelets. CONCLUSION: These results suggest that significant departures of ERG responses (studied with the protocol described herein) from a laboratory's normal values cannot be attributed to halothane.

Adolescent

The rod sensitivity of dark adapted human infants.

The determinants of infants' low scotopic visual sensitivity are controversial. Some interpret infants' scotopic vision as indicative of immature rod photoreceptor function while others attribute infantile sensitivities mainly to post receptoral immaturities. To date the rod photoreceptor sensitivity of human infants has not actually been measured. In the work reported herein, electroretinographic a-wave responses, which represent the rod photoresponse, were recorded from dark adapted 10-week old infants and adult control subjects. Rod isolated a-waves indicate that 10-week-old infants' rods are less sensitive than adults'. Thus, any explanation of infants' scotopic visual sensitivity must take into account this fundamental property of infants' rods, low sensitivity.

Adult

Titin, a huge, elastic sarcomeric protein with a probable role in morphogenesis.

Titin, the largest protein identified to date (over 1 micron long, almost 3 million daltons in mass) is the third most abundant component of the sarcomere. In the mature myofibril, titin molecules span from M line to Z line, forming a third filament system which provides sarcomeric alignment and elastic recoil. In the developing sarcomere, accumulating evidence from studies both in vivo and in vitro implicates titin as part of a morphogenetic scaffolding, upon which critical events in myofibrillogenesis are coordinated in a time- and space-dependent manner.

Animals

Dark adapted thresholds in young RCS rats.

The dark adapted thresholds of black-eyed RCS rats and cogenic controls were measured using a new psychophysical procedure that permits determination of thresholds at earlier ages than previously possible. Rats were tested longitudinally between about ages 1 and 3 months. At all ages, thresholds of RCS rats were higher than those of controls. Thresholds did not change systematically with age for either RCS rats or controls. At age 3 months, when the RCS rats' b-wave is no longer recordable, RCS thresholds are about 2.2 log units higher than those of controls.

Aging

Temporal summation in dark-adapted 10-week old infants.

The effect of stimulus duration on the b-wave and psychophysical responses of dark-adapted 10-week-old infants and adult control subjects is reported. Both infant and adult b-wave sensitivities vary with stimulus duration, show summation for brief duration stimuli, critical durations estimated at 88-155 msec, and little variation in sensitivity for longer durations. There are however, substantial differences between the infant and adult psychophysical temporal summation functions. The infant function is described by a straight line, slope about -0.5, across all flash durations while adults show summation at durations less than 100 msec and critical durations of 136 to 151 msec. Adult, but not infant, b-wave integration times and b-wave rise and fall times show duration-dependent changes. Thus, both ERG and psychophysical measures demonstrate immaturities in the rod mediated function of the infant retina.

Adult

Electroretinographic assessment of background adaptation in 10-week-old human infants.

Full field, scotopic b-wave stimulus/response functions of 10-week-old infants and adults were measured in the dark-adapted condition, and in the presence of steady backgrounds. Dark adapted b-wave sensitivity (log sigma) differed significantly between infants and adults; the median dark adapted sensitivity of infants was 0.50 log unit less than that of adults. The median eigengraus of infants (-1.32 log scot. td) and adults (-1.55 log scot. td) did not differ significantly. The median slope of the linear portion of the background adaptation function was about 0.9 for infants and adults. These results argue for post-receptoral immaturities, but do not rule out receptoral immaturities.

Adaptation, Ocular

Visual development of infants with severe ocular disorders.

Among 11 patients who presented as blind in early infancy, with Leber's congenital amaurosis (5 patients), optic nerve hypoplasia (4 patients), or macular colobomata (2 patients), 8 developed visually guided behavior and measurable grating acuity by age 5 to 46 months. All children with measurable grating acuity demonstrated visually guided mobility. Grating acuity was predictive of later visual performance in 10 of 11 patients by age 12 to 16 months. The best grating acuity attained by 7 months was 1.3 to 3.0 cycles/degrees (20/460 to 20/200) and 0.13 cycles/degrees (20/4700) by month 8. Two patients with Leber's congenital amaurosis and one with optic nerve hypoplasia remained blind. No clinical features existed to differentiate these three patients from the eight whose visual status improved. Posterior visual pathway maturation may underlie the improvement.

Child

The quantity of rhodopsin in young human eyes.

The rhodopsin content of 20 eyes of infants and children ages 27 weeks gestation to 8 years (11 donors) was assayed and compared to the rhodopsin content of adults (36 eyes; 19 donors). Infants have significantly lower rhodopsin contents than adults. On average the rhodopsin content of young infants is about a third of adults. Previously reported full-field b-wave sensitivity of young infants is about 0.5 log units, that is about a third, less than adults. Thus, as previously found in infant rats, photon capture by rhodopsin appears to limit the dark adapted sensitivity of young human infants.

Adolescent

The quantity of rhodopsin in human eyes.

The content of rhodopsin in the eyes of 15 donors (30 eyes) was determined. Both retinal and pigment epithelial fractions were collected from each globe, extracted using 1% CTAB, and the rhodopsin difference spectrum of each fraction was obtained separately. The total amount of rhodopsin, obtained by summing the amounts recovered from the retinal and PE fractions, ranged from 2.00 to 11.94 (median: 6.40) nmoles/eye. Previously reported mean values of about 3.5 to 4.0 nmoles per retina have been obtained using a variety of methods. The present higher values, perhaps largely dependent on procedural details described herein, appear plausible given the known concentrations of rhodopsin in rod outer segments, rod outer segment volumes, and number of rods in the human retina.

Adult

Infant vision testing by a behavioral method.

A behavioral method of screening binocular infant vision called forced choice preferential looking (FPL) has been developed. Clinical trials of the FPL test for young infants are reported here. The test aids nonspecialized personnel in early identification of bilateral ocular abnormalities and anomalies of binocular cooperation (strabismus). It is not possible to screen for monocular eye disease or amblyopia with this test. Modifications of the FPL test may offer new ways of assessing other aspects of visual function early in life.

Age Factors

Human albinism. Light and electron microscopy study.

The eyes of a 13-year-old leukemic boy with the attributes of tyrosinase-negative oculocutaneous albinism were obtained for light and electron microscopic study. Repeated examinations had failed to reveal WBCs with giant oxidase-positive granules, and leukemic involvement of the fundus never occurred. Light microscopic examination of horizontal and vertical sections through the retina confirms earlier reports that the fovea is absent in albinos. The synaptic apparatus of the photoreceptor terminals appears abnormal. The rough endoplasmic reticulum of the retinal pigment epithelial cells is sparse though the presence of phagosomes suggests that phagocytic function is intact. Suggestions as to the importance that the morphological findings may have on albino visual function are made.

Acute Disease