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Biomedical subjects

J B Wilson

Publications and source records attributed to J B Wilson.

At least 289 records · Page 16Linked to original sources
PubMed1977

Some properties of hemoglobin Gun Hill.

Hb Gun Hill has been found in an asymptomatic black female living in Alabama; neither her parents nor her two siblings carry the variant. The deletion of five amino acid residues in the beta chain of Hb Gun Hill (alpha2beta2 91-95 deleted) causes this variant to have a high oxygen affinity, an interaction coefficient of unity and an absence of the Bohr effect. The rate of oxidation of liganded Hb Gun Hill by the ferricyanide ion follows first-order kinetics. Hb Gun Hill is to a great extent dissociated into dimers over a wide range of pH values.

Adolescent↗
PubMed1977

The T gamma chain of human fetal hemoglobin at birth and in several abnormal hematologic conditions.

The T gamma chain of human fetal hemoglobin has a threonyl in stead of an isoleucyl residue in position 75. When the cord bloods from infants from varied ethnic backgrouds and geographic areas were tested for the presence of the T gamma chain, it was present in 28 or 98 samples. In some groups as many as 40% had the T gamma chain whereas none was detected in other. When the T gamma chain was present, its quantity was about 20% of the total gamma chains, but one case had 35%. Among beta-thalassemia homozygotes of the Mediterranean region, 70% and the T gamma chain in the amount of 20-50% of the total gamma chains, but seven Black beta-thalassemia homozygotes were negative for the T gamma chain. The fetal hemoglobin of 16 adult patients with sickle cell anemia had no T gamma chains, but 2 of 9 newborn children with sickle cell anemia had the T gamma chain. The frequency of the T gamma gene (16), the relationship of the T gamma gene to the G gamma and A gamma genes, and the significance of the T gamma gene are discussed.

Adult↗
PubMed1976

Thalassemia intermedia caused by heterozygosity for both beta-thalassemia and hemoglobin Saki [beta 14 (A11) Leu replaced by Pro].

The syndrome thalassemia intermedia can be the clinical expression of heterozygosity for different tyes of thalassemia, beta-thalassemia and hereditary persistence of fetal hemoglobin, beta-thalassemia and Hb-Lepore, and in blacks it may even represent a true beta-thalassemia homozygote. This report describes thalassemia intermedia in a white male due to beta-thalassemia and an unstable hemoglobin. Chain-synthesis studies showed an excess of alpha-chain production over beta-chain production in the propositus and his mother but balanced chain synthesis in the clinically normal father, who is heterozygous for the unstable hemoglobin. The unstable hemoglobin was found to be beta14 (A11) Leu leads to Pro, which has previously been described in a clinically normal African woman, and named Hb-Saki. This hemoglobin is not distinguishable from Hb-A on routine electrophoresis at alkaline or acid pH and tests for unstable hemoglobins are necessary for its detection. The increasing list of such hemoglobin variants and previous cases of heterozygosity for beta-thalassemia and unstable hemoglobins are reviewed.

Adolescent↗
PubMed1976

Hb Leslie, an unstable hemoglobin due to deletion of glutaminyl residue beta 131 (H9) occurring in association with beta0-thalassemia, HbC, and HbS.

A new unstable hemoglobin, Hb Leslie, has been observed in three generations of a Georgia family. The propositus, a 42-yr-old black veteran with hemolytic anemia and splenomegaly, has a hemoglobin variant with an electrophoretic mobility similar to that of hemoglobin F. The variant comprises about 85% of the total hemoglobin and was isolated by chromatography. Chemical analysis has identified the abnormality as a deletion of the glutaminyl residue in position 131 (H9) of the beta-chain. Deletion of this critical residue which participates in the alpha1beta1 contact causes decreased stability of the hemoglobin without significant changes in functional properties or morphologic abnormalities in the erythrocyte. Family studies revealed hemoglobin Leslie occurring in combination with beta0-thalassemia, HbS, and HbC. All persons with the various Hb Leslie combinations, including the propositus, have no clinical manifestations other than anemia. In some the anemia is fully compensated. There is no history of drug-associated hemolysis.

Adolescent↗
PubMed1975

Hemoglobin Fort Gordon or alpha2beta2145 Tyr replaced by Asp, a new high-oxygen-affinity hemoglobin variant.

Hemoglobin Fort Gordon, alpha2beta2145 Tyr replaced by Asp (HC2), has been observed in a 20-year-old black male with compensatory erythrocytosis. The variant was readily identified by electrophoresis and chromatography, and comprised about 30% of the red cell hemoglobin. The substitution was identified through analyses of tryptic peptides of various digests of the isolated beta chain. The oxygen affinity of whole blood was increased; two components were observed one of which had a greatly increased affinity for oxygen and a markedly reduced subunit cooperativity. It appears that the Tyr replaced by Asp substitution resembles the Tyr replaced by His substitution in hemoglobin Bethesda (Bunn, H. F. et al. (1972) J. Clin. Invest. 51, 2299-2309; Olson, J. S. and Gibson, G. H. (1972) J Biol. Chem. 247, 3662-3670; Adamson et al. (1972) J. Clin. Invest. 51, 2883-2888) in that both inhibit the quarternary change of the oxy to the deoxy conformation, resulting in greatly altered functional properties. Studies of a few members of the family were negative.

Adult↗
PubMed1975

Hemoglobin-A2-Coburg or alpha2delta2116Arg leads to His (G18).

Hemoglobin-A2-Coburg or alpha2delta2-116 Arg leads to His (G18) has been found in members of a family of Sicilian origin. The propositus is heterozygous for hemoglobin-A2-Coburg as well as for beta-thalassemia, and family data indicate that the gene for the delta-Coburg chain is in trans of the beta-thalassemia determinant.

Adolescent↗
PubMed1975

Hemoglobin Atlanta or alpha 2 beta 2 75 Leu-Pro (E19): an unstable variant found in several members of a Caucasian family.

Hemoglobin Atlanta, alpha 2 beta 2 75 Leu-Pro (E19), has been found in several members of three generations of a Caucasian family living in metropolitan Atlanta. The abnormal hemoglobin is one of the nine unstable variants in which either a leucyl or an alanyl residue is replaced by a prolyl residue. These substitutions have been observed in the B, E, F, and G helixes of the beta chain and in the H helix of the alpha-chain. Hemoglobin Atlanta heterozygotes are mildly affected by the presence of this unstable hemoglobin.

Alanine↗
PubMed1975

Hb Camden and Hb Hope found during routine testing.

The electrophoretically fast-moving hemoglobin variant Hb Camden [beta131 (H9) gln leads to glu] has been found in a middle-aged female suffering from pulmonary disease, and Hb Hope [beta136 (H14) gly leads to asp] which migrates just ahead and very close to Hb A was present in a young obstetrical patient with a mild hemolytic anemia.

Adult↗
PubMed1975

The work of a rural practice.

Study of the work load in the month of November over some years in a single handed rural practice has revealed several interesting trends, one being the way in which the work is gradually increasing. A more detailed study of the work over 12 months has demonstrated how fallacious impressions about one's work can be--the work being spread over a very much larger proportion of the patients than had been originally suspected.

Adolescent↗
PubMed1975

Clinical measurement of trend in fertility of women attending family planning clinics in Ghana.

1. The need for a simple method of measuring trend in fertility in family planning programme has been stated. 2. The theory of the numerator analysis of age and parity or age and number of living children and its variants the "three area" and the "four quadrant methods" have also been stated. 3. The data from the Korle Bu Teaching Hospital have been analysed. 4. The finding was that the proportion of women of the high fertility group is reducing while that of women in lower fertility is increasing with time. 5. The relevance of this type of analysis to national family planning programme has been emphasised.

Adult↗