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Biomedical subjects

V A Spivak

Publications and source records attributed to V A Spivak.

At least 19 recordsLinked to original sources
PubMed1993

[The first case of Hb Camden carriage in Russia].

Structural hemoglobinopathy due to carriage of Hb Camden was diagnosed in a male patient and his mother. Anomalous Hb was detected after a detailed analysis of the clinical evidence, blood smears microscopy, marrow examination, data obtained by a complex of methods to identify unstable Hb. It was defined as Hb Camden beta 131 GLN-->GLU. The carriage of this variant brings no clinical manifestations, but leads to impaired red cells morphology, decline of their life span, enhanced hemolysis. These properties are typical for mutant Hb lacking stability. Hb Camden carriage is considered as a compensatory hemolytic process.

Adolescent↗
PubMed1992

[Distribution of abnormal hemoglobins S and C in the Republic of Guinea].

Distribution of the HbS and HbC in the Guinean Republic was determined by the analysis of the excerption of 2213 inhabitants representing different ethnic groups of the country. It was found that the mean frequency of the HbS heterozygotes is 21.2 +/- 0.9% and of the HbC heterozygotes is 2.6 +/- 0.3%. Among major nationalities of the country the frequencies of the HbS heterozygotes make up 21.0 +/- 1.9% for the Fulbe, 22.2 +/- 1.6% for the Malinke and 26.5 +/- 1.6% for the Susu and frequencies of the HbC heterozygotes make up 3.0 +/- 0.8% for the Fulbe, 2.5 +/- 0.6% for the Malinke and 1.6 +/- 0.4% for the Susu. Relative viability of the HbS and HbC carriers for major Guinean nationalities is estimated. The relative fitnesses account is 1.05-1.13 for HbC heterozygotes and 1.07-1.16 for HbC heterozygotes.

Ethnicity↗
PubMed1991

Plasma desorption mass spectrometry of haemoglobin tryptic peptides for the characterization of a Hungarian alpha-chain variant.

S-Aminoethylated-alpha A and -beta A globin tryptic peptides separated by reversed-phase high-performance liquid chromatography have been analysed by plasma desorption mass spectrometry. Almost all the expected alpha A and beta A tryptic fragments were tentatively assigned relative to the known globin chain sequences based on the molecular weight obtained by plasma desorption mass spectrometric analysis of the purified peptides. The application of plasma desorption mass spectrometry for structure elucidation of a haemoglobin alpha-chain variant revealed the first case of Hb Hasharon in Hungary.

Adult↗
PubMed1990

[The second family with hemoglobin Dagestan (alpha 60 Lys----Glu)].

Data have been presented on a new hemoglobin detected in a family of the Lezghin population in the Daghestan ASSR. The structural analysis of this hemoglobin has shown its correspondence to hemoglobin "Daghestan" that was previously detected among the population living in the Tabasaransk region. A detailed genetic analysis has shown a possibility of the gene drift. It was found that the proband's grandmother (his father's mother) had come from the Tabasaransk population of the Republic.

Adult↗
PubMed1989

[Analysis of phenotypic manifestations of abnormal human hemoglobins].

Phenotypic manifestations of abnormal human hemoglobins are discussed using the data of hematology, protein chemistry and molecular biology. On the basis of the presented analysis it is proposed to distinguish between phenotypic manifestations characterizing the primary molecular defect, i.e. properties of a mutant protein and their expression on the molecular and cellular levels; manifestations characterizing the equilibrium of the primary defect and compensatory potentialities of the organism; and finally the unbalanced state when the compensatory abilities of the organism are depleted. These different manifestations of the same defective gene reflect the most relevant peculiarities of mutant protein properties per se, expression of the properties in the living organism, the influence on the homeostatic system "mutant protein--organism" of the genetic and environmental factors both at the compensation state and at stress.

Hemoglobins, Abnormal↗
PubMed1988

Micropreparative electrophoresis of globin chains on cellulose acetate film in structural identification of abnormal human hemoglobins.

A simple and rapid micropreparative method for isolating 5 to 30 nmol of globin chain, followed by structural identification of abnormal human hemoglobins, is described. The method is based on the electrophoretic separation of globins on ordinary Cellogel films under denaturating conditions with subsequent cutting out of the protein zones and solubilization of Cellogel and the electrophoretic buffer components in a specially selected solvent in which the globin chain undergoes quantitative precipitation. The method makes it possible to simplify and speed up the structural identification of commonly occurring abnormal hemoglobins. The advantages and limitations of the method are discussed along with its potential uses in structural protein chemistry.

Cellulose↗
PubMed1988

[A new strategy for structural identification of abnormal human hemoglobins].

A new strategy for structural identification of abnormal human hemoglobins is proposed. It is based on micropreparative modification of electrophoretic separation of globins on Cellogel strips with subsequent quantitative isolation of a pure, desalted globin chain, in a form suitable for its subsequent structural investigation. Among the major advantages of the new strategy age possibility to use small blood samples (0.1-0.2 ml), short analysis time, relative simplicity and low cost.

Blood Protein Electrophoresis↗
PubMed1988

[Micropreparative thin-layer peptide maps in the molecular diagnosis of abnormal human hemoglobins].

General factors determining the possibility of application of peptide maps in thin layer of microcrystalline cellulose as a micropreparative method in molecular diagnostics of abnormal hemoglobins were studied. The effects of absorbtional capacity of cellulose and amino acid impurities in it, choice of eluent and elution technique, peptide structure and extent of its modification in staining as well as completeness and specificity of globin chain enzymatic digestion on peptides extraction from thin layer were analysed. The results of structural identification of Hb D Punjab beta 121 Glu----Gln at a Cypriot; Hb O Arab; beta 121 Glu----Lys at a Bulgarian woman, living in Kalinin region (the second case in the USSR); Hb M Saskatoon beta 63 His----Tyr at a woman from Georgia (the second case in the USSR); Hb Buenos Aires beta 85 Phe----Ser at a Russian girl (the first case in the USSR and the third case in the world); Hb Dagestan alpha 60 Lys----Glu at two members of a Lesgin family from Dagestan; Hb Agenogi beta 90 Glu----Lys at a Hungarian woman; Hb Setif alpha 94 Asp----Tyr at three patients from Cyprus and Hb Detroit beta 95 Lys----Asn at an Azerbaijanian woman (the first case in the USSR and the second case in the world) are presented.

Amino Acids↗
PubMed1986

[Rare variants of Hb D Punjab, Hb O Arab and polymorphism of human hemoglobins].

This report describes the occurrence, study and molecular diagnostics of 40 Hb O Arab beta 121 Glu Lys cases and 4 Hb D punjab beta 121 Glu Gln cases in Bulgaria. Hematological, morphological and clinical data for 12 patients with Hb O arab are listed. Among them we observed 7 simple heterozygotes for Hb O Arab/Hb A, two double heterozygotes-compounds for Hb O/beta+-thalassemia and three compounds for Hb O/beta 0-thalassemia (the latter assumed). Also, general hematological, morphological and clinical data are presented for 4 Hb D Punjab carriers, from which two are simple heterozygotes and two are assumed, as compounds for Hb D/beta 0-thalassemia. The consideration of heterozygosity, homozygosity for both abnormal hemoglobins and of the compound state of Hb O or Hb D/beta-thalassemia or HbS types let us suggest the relative neutrality of the variants and the limitation in their distribution, depending on genetic structure of populations, where they spread. It may be concluded that human hemoglobin is characterized by marked monomorphism. At the same time, the high frequency of HbS, HbE and HbC in some populations can be well explained by contemporary selectionism; the distribution of relatively neutral Hb D Punjab and Hb O Arab with some limitations can follow Kimura's neutralism concept.

Amino Acids↗