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Biomedical subjects

J A Pierce

Publications and source records attributed to J A Pierce.

15 recordsLinked to original sources
PubMed1992

Family study of alpha 1-antitrypsin deficiency: effects of cigarette smoking, measured genotype, and their interaction on pulmonary function and biochemical traits.

To gain insight into the variable expression of lung disease in alpha 1-antitrypsin (alpha 1AT) deficiency, five quantitative variables including forced expiratory volume at 1 sec (FEV1), forced expiratory flow rate between 25 and 75% of forced vital capacity (FEF25-75), total serum alpha 1AT, oxidized serum alpha 1AT, and total serum immunoglobulin E (IgE) were measured in alpha 1AT deficient individuals and their families. The effect of a known, measured genotype (the Pi type) was estimated for each quantitative trait; the influence of mode of case ascertainment on the measured genotype effect was also assessed. These analyses showed that total alpha 1AT levels are strongly influenced by Pi type; IgE levels are unaffected by Pi type; and FEV1, FEF25-75, and oxidized alpha 1AT are moderately influenced by Pi type. The effect of genotype-by-environment interaction between Pi type and pack-years of cigarette smoking on the five quantitative phenotypes was studied using an analysis of covariance. Significant Pi x pack-years interaction was evident for FEV1, but this effect is confounded in this data set with the Pi x age interaction. Probands who were ascertained because they had chronic obstructive pulmonary disease (COPD) do not demonstrate the significant Pi x pack-years interaction effect of the Pi x pack-years subjects ascertained for other reasons demonstrate. The effect of the Pi x pack-years interaction on FEV1 was no longer significant on a transformed scale, (FEVf12,) thus providing an additive scale for future data analysis. The increased sensitivity of Pi MZ individuals in our sample to cigarette smoking reduced the Pi x packs-years interaction effect on FEF25-75 to borderline significance. This investigation has provided an opportunity to incorporate both measured genotype and genotype-by-environment interaction analyses into the study of the variable expression of lung disease in Pi Z individuals.

Family↗
PubMed1979

Improved identification of antitrypsin phenotypes through isoelectric focusing with dithioerythritol.

Isoelectric focusing has replaced acid starch-gel electrophoresis for the routine determination of antitrypsin phenotypes in recent years. We observed increased sharpness of antitrypsin bands and decreased background stain following the addition of DTE to serum before isoelectric ofcusing in polyacrylamide gels. DTE and DTT were the only sulfhydryl reducing agents which produced this effect. Electrophoretic mobility of antitrypsin bands was increased very slightly. Decreased background stain resulted from the precipitation of albumin. Precipitation (coagulation) of albumin was complete in serum after 60 min incubation at 37 degrees C at concentrations of 30 mM DTE or DTT. Optimal pH for denaturation was 7.6 to 8.8. Ionic concentrations reduced the strength of the coagulum at 2.5M sodium chloride but had little effect at lower concentrations. Marked temperature effects were noted. As a result of these studies, we recommend examination of native and reduced (30 mM DTE) serum on isoelectric focusing in polyacrylamide gel for all samples submitted for routine antitrypsin phenotype determinations. It also seems possible that the nontoxic DTE (DTT) precipitation of albumin could prove useful for studies of serum proteins other than alpha 1-antitrypsin.

Animals↗
PubMed1977

Cervical ankylosis with acute spinal cord injury.

Twenty-three cases of acute spinal cord injury in persons with cervical ankylosis are presented. Certain characteristics of major sub-groups are described: ankylosing spondylitis (N = 8), degenerative spondylosis (N = 9) and congenital fusion (congenital non-segmentation) (N = 6). The ankylosing spondylitic group presented a grim prognosis for survival (death rate 50 per cent within 60 days) and for loss of neurological function. Five out of eight cases had permanent neurological loss subsequent to their injuries. Both the ankylosing spondylitic and degenerative spondylotic groups presented problems in diagnosis and medical management. The basic principle is immobilisation of the fracture and mobilisation of the patient. The halo is the technique of choice for fracture immobilisation. An integrated intensive respiratory management programme is essential. Patients with ankylosed spines, particularly those with ankylosing spondylitis, should be educated in simple measures to prevent fracture of their spines.

Adult↗
PubMed1977

The induction of pulmonary emphysema with human leukocyte elastase.

Purified human leukocyte elastase was injected into the tracheas of 46 hamsters. Thirteen animals died spontaneously within 1 week, with extensive lung hemorrhage. The elastin content of the lungs was only slightly less than control values 3 hours after injection. At 2 months, the lungs of the remaining animals showed mild, patchy emphysema and morphometric changes consistent with emphysema. These results contrasted with the effects of a similar elastolytic dose of pancreatic elastase administered to 26 other hamsters in that only one animal died spontaneously, the lung elastin content 3 hours after injection was substantially decreased, and severe emphysema was present 2 months later. Leukocyte elastase appears to be capable of causing emphysema; but unlike pancreatic elastase, leukocyte elastase produces emphysema that is mild, even at a dose sufficient to produce intense lung hemorrhage and a high mortality.

Animals↗
PubMed1977

Antitrypsin and chronic obstructive pulmonary disease among Japanese-American men.

A total of 161 patients with chronic obstructive pulmonary disease (COPD) plus 100 control subjects (identified during a study of heart disease in 6,860 Japanese-American men aged 52 to 75 years who were residing in Hawaii) were analyzed for phenotype in search of the antitrypsin gene Z, which has been shown to be associated with pulmonary emphysema in other racial groups. No carriers of the Z gene were found, and the question of whether the rarity or absence of this gene relates to a low frequency of COPD among Japanese-Americans is reviewed.

Chronic Disease↗
PubMed1976

Vesical neck suspension under vision with cystotomy enhances treatment of female incontinence.

Failure to surgically correct incontinence usually results from inadequate screening of patients with hyperreflexic incontinence, improper fixation of the urethra or yielding of tissues that have supported the repair. A surgical approach is described which fixes the bladder neck to the rectus tendon and adjacent pubic periosteum, under direct vision, through a cystotomy incision. The procedure achieves good continence in more than 90 per cent of the cases. Temporary postoperative voiding dysfunction occurred in about half of the patients.

Adult↗
PubMed1975

Antitrypsin phenotypes in St. Louis.

Antitrypsin phenotypes were determined from 2,285 donors at the Barnes Hospital Blood Bank in St. Louis . Results resembled those obtained from Central Europeans: there were fewer proteinase inhibitor (Pi) Z alleles in St. Louis than in Scandinavian test groups. Interestingly, major antifrypsin variants occurred only 40% as frequently in black as in the balance of the study group.

Adult↗
PubMed1975

Improved estimate of the Gm-Pi linkage.

The Gm-Piota linkage group is firmly established. A heterogeneity of recombination fraction amongst males of different Piota types has now become very likely. The major differences seem to be between the Piota (z) and other alleles of the Pi system. A chromosomal deletion, inversion or a regulatory (rec) locus in linkage disequilibrium with the Piota locus offer possible explanations.

Alleles↗
PubMedDate not supplied

Physiological and genetic strategies for enhanced subtilisin production by Bacillus subtilis.

Defined minimal media conditions were used to assess and subsequently enhance the production of subtilisin by genetically characterized Bacillus subtilis strains. Subtilisin production was initiated by the exhaustion or limitation of ammonium in batch and fed-batch cultures. Expression of the subtilisin gene (aprE) was monitored with a chromosomal aprE::lacZ gene fusion. The beta-galactosidase production driven by this fusion reflected subtilisin accumulation in the culture medium. Subtilisin gene expression was temporally extended in sporulation-deficient strains (spoIIG), relative to co-genic sporogenous strains, resulting in enhanced subtilisin production. Ammonium exhaustion not only triggered subtilisin production in asporogenous spoIIG mutants but also shifted carbon metabolism from acetate production to acetate uptake and resulted in the formation of multiple septa in a significant fraction of the cell population. Fed-batch culture techniques, employing the spoIIG strain, were investigated as a means to further extend subtilisin production. The constant provision of ammonium resulted in linear growth, with doubling times of 11 and 36 h in each of two independent experiments. At the lower growth rate, the responses elicited (subtilisin production, glucose metabolism, and morphological changes) during the feeding regime closely approximated the ammonium starvation response, while at the higher growth rate a partial starvation response was observed.

Bacillus subtilis↗