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At least 397 records · Page 22Linked to original sources
PubMed1980

Preparation of lyophilized abnormal hemoglobin control for cellulose acetate electrophoresis.

A process for the preparation of stable lyophilized normal and abnormal hemoglobin controls for alkaline cellulose acetate electrophoresis is described. The hemoglobins were converted to cyanmethemoglobin derivatives with K3Fe(CN)6 and KCN. Sucrose, 100 g/l, was added to stabilize the preparation for lyophilization and storage and to increase the rate of reconstitution. Lyophilized preparations were stable with no loss of resolution for nine months of use in the laboratory and for two weeks of accelerated aging at 37 C. The materials appeared to have an extended shelf-life of at least two years at 5 C. The controls reconstituted quickly, usually within a minute, and could be stored at 5 C for three months with only a slight increase in band streaking. The lyophilized products appeared to be suitable to assist in the development of a quality-control system in hemoglobin screening programs employing cellulose acetate electrophoresis.

Blood Preservation↗
PubMed1984

[Identification of abnormal hemoglobins by isoelectric focusing].

Using IEF on slabs of acrylamide gel was adapted for screening of abnormal Hemoglobins which are at the same level by electrophoresis on cellulose acetate strips. This method is fast, inexpensive and allowed the simultaneous analysis of 70 samples of whole blood. The characterization technique of IEF allowed us to distinguish some rare variants like Hb O Arab, HbD and T gamma in B 0-thalassemia.

Hemoglobins, Abnormal↗
PubMed1990

Abnormal hemoglobins in the Silk Road region of China.

A review is presented of the occurrence of 24 abnormal hemoglobins (13 alpha-chain variants and 11 beta-chain variants) in populations in the Silk Road area of Northwestern China. Most frequently occurring were Hb D-Punjab [beta 121(GH4)Glu----Gln] in Uygurs, Kazaks, and Khalkhas, Hb G-Taipei [beta 22(B4)Glu----Gly] in persons of the Han nationality, and Hb G-Coushatta [beta 22 (B4)Glu----Ala] in the Uygurs, Kazaks, Hans, and related nationalities. The data suggest that these variants likely originated in Central Asia, in the Han nationality of China, and in the minorities of northern China, respectively. Other variants occurred at considerably lower frequencies and were imported from other countries or arose as independent mutations. Two variants [Hb Tashikuergan or alpha 19(AB1)Ala----Glu; Hb Tianshui or beta 39(C5) Gln----Arg] were observed for the first time. The data from this study of the many variants support the movements of various populations in this area, as reported in numerous historical documents.

China↗
PubMed1989

[Abnormal hemoglobins in Negroid Ecuadorian populations].

The prevalence of hemoglobinopathies was determined in the black race located in two distinct geographical areas in Ecuador; in the coastal province of Esmeraldas, particularly the Santiago basin (Rio Cayapas and Rio Onzoles) and in the province of Imbabura, particularly in the intermoutain valley, Valle de Chota. A total of 2038 blood samples were analyzed, 1734 in Esmeraldas and 304 in Inbabura, of which 23.2% (473 individuals) were found to be carriers of abnormal hemoglobins, 25.4% (441) in Esmeraldas and 10.5% (32) in Imbabura. The abnormal hemoglobins found in Esmeraldas were Hb AS (19.2%), Hb AC (5.0%), Hb SS (0.6%) and Hb SC (0.5%) while in Imbabura only Hb AS (9.5%) and Hb AC (0.9%) were found. The factors that could influence the difference in prevalence found in the two geographical areas are discussed.

Black People↗
PubMed1986

The identification of five rare beta-chain abnormal hemoglobins by high performance liquid chromatographic procedures.

The detection, quantitation, and characterization of five relatively rare beta chain abnormal hemoglobins mainly by high performance liquid chromatographic procedures are described. The variants involved are Hb City of Hope (beta 69 Gly----Ser), Hb Austin (beta 40 Arg----Ser), Hb Leiden (beta 6 or 7 Glu----0), Hb Louisville (beta 42 Phe----Leu), and Hb Presbyterian (beta 108 Asn----Lys). Some clinical and hematological data are also included.

Amino Acid Sequence↗
PubMed1992

Biophysical changes of red cells with thalassemia-like abnormal hemoglobin.

Hemoglobin (Hb) E and Hb Constant Spring (CS) are known to have thalassemia-like effects. Investigation on the red cell physical changes in these two abnormalities has been done to clarify the diversity of red cell changes between thalassemia and thalassemia-like abnormal hemoglobin (Hb E represents beta-thalassemia and Hb CS represents alpha-thalassemia). Eleven cases with homozygous Hb CS (CS/CS), 7 homozygous Hb E subjects (E/E) and one double heterozygous case with Hb CS and Hb E were included in this study. The red cells were analyzed by the H* 1 hematology analyzer. The E/E red cells had significantly smaller MCV than the CS/CS red cells (p < 0.001). The smaller MCV of E/E red cells was attributed to markedly increased percent microcyte (p < 0.001) and significantly lower percent macrocyte (p < 0.001) as compared to CS/CS red cells. Degree of heterogeneity in cell volume as indicated by red cell distribution width (RDW) was not significantly different between the two abnormal hemoglobin containing red cells. It was also noted that the CS/CS cases had a more significant increase in small RBC than the E/E cases (p < 0.001). Significant lower intraerythrocyte hemoglobin concentration values of the CS/CS red cells as compared to E/E red cells were shown: cellular hemoglobin concentration mean (CHCM) (p < 0.001), percent hyperchromic red cell (p < 0.001) and hemoglobin distribution width (HDW) (p = 0.0367). Higher values for the CS/CS red cells were MCH (p < 0.001) and percent hypochromic red cells (p < 0.001). Red cells from both genotypes had significant decreases in red cell deformability.(ABSTRACT TRUNCATED AT 250 WORDS)

Biophysical Phenomena↗
PubMedDate not supplied

The effects of abnormal hemoglobins on a new microcolumn method to determine hemoglobin A1c.

A new cation-exchange microcolumn employing a borate buffer reportedly separates hemoglobin A1c from other labile glycosylated intermediates in a one step procedure. The effect of commonly occurring abnormal hemoglobins on the hemoglobin A1c (Hb A1c) levels as determined by this new technique has been studied and compared to the values obtained by isoelectric focusing. If a patient has hemoglobin levels in the normal range, or if HbS, HbE, or an elevated HbA2 are present, then hemoglobin A1c levels as estimated by this new column are unaffected or decreased. However, if HbF is elevated, a marked increase in the HbA1c level is observed. The increase is almost directly proportional to the HbF level up to 40 percent. This new column technique is therefore useful in eliminating labile glycosylated hemoglobin intermediates but must be coupled with other techniques if high HbF values are present.

Chromatography, Ion Exchange↗
PubMed1988

Identification of abnormal hemoglobins in cord blood by an immunoblot technique.

Variant hemoglobins S, C, and FHull were identified in newborn cord blood hemolysates with an immunoblot procedure. The abnormal hemoglobins were originally detected in cord blood hemolysates by cellulose acetate electrophoresis and presumptively identified according to their mobilities. Hemolysates that contained a hemoglobin variant again underwent electrophoresis and were transferred to nitrocellulose membranes. For identification, the membrane was treated with the appropriate rabbit monospecific antiserum and developed with peroxidase-conjugated anti-rabbit IgG or peroxidase-conjugated protein A. After the addition of substrate, a violet reaction developed at the site of the hemoglobin band, signifying an antigen-antibody reaction or a positive reactivity. The use of monospecific antisera in an immunoblot technique allows for confirmed identification of variant hemoglobins in cord blood samples in as little as 5 hours. In some cases in which the variant is near the limits of detectability by protein staining, the immunoblot allows an unequivocal identification.

Electrophoresis, Cellulose Acetate↗
PubMed1989

Identification of Hb D-Punjab gene: application of DNA amplification in the study of abnormal hemoglobins.

Hemoglobin D-Punjab (or D-Los Angeles) is a common variant worldwide. It is also the most frequent abnormal hemoglobin in Xinjiang Uygur Autonomous Region of China. A large survey of hemoglobinopathy, including 142,171 people and 21 national/ethnic groups, was carried out in Xinjiang and indicated Hb D-Punjab accounted for 55.6% of the total hemoglobin variants there. Here we describe a simple way--EcoRI mapping of the amplified beta-globin DNA sampling from dried blood spots on filter paper blotters--of identifying the Hb D-Punjab gene. The primers were designed and synthesized to emzymatically amplify a 144-bp fragment of beta-globin gene which included codons beta 121 (GAA) and 122 (TTC) representing an EcoRI recognition site. The Hb D-Punjab gene could be easily detected by EcoRI digestion of the amplified DNA sequence on agarose gel because of a single base change at codon 121. The analysis of amplified DNA sampling from dried blood provides a very useful method for population study of Hb D-Punjab and will be of significance for demonstration of the occurrence of the Hb D-Punjab gene and for understanding of the relations among various nationalities.

China↗
PubMed1978

Simple electrophoretic system for presumptive identification of abnormal hemoglobins. By the International Committee for Standardization in Hematology.

In order to determine if mutant hemoglobins can be identified by relatively simple methods, a Working Group of the ICSH Expert Panel on Abnormal Hemoglobins and Thalassemia analyzed 17 hemolysates containing 14 different mutant hemoglobins by four electrophoretic methods: (1) cellulose acetate in alkaline buffers, (2) citrate agar pH 6.0, (3) urea 2-mercaptoethanol buffer pH 8.9, and (4) urea 2-mercaptoethanol buffer pH 6.0. The examined mutants included several of great numerical and clinical importance as well as some rare ones, namely, HbS, C, D Los Angeles (Punjab), E, G Philadelphia, N Baltimore, and O Arab; also Hb Ft. Worth, Montgomery, Winnepeg, Rush, Q India, Bethesda, and Lepore. Comparative mobilities of these hemoglobins in all of the methods are presented here. The combined data permit their presumptive identification, often with a high degree of specificity. The system has been applied in Iran, where the four prevalent mutants can be differentiated by these methods, at considerable saving of time and resources previously expended on structural analyses. It is proposed as a basis for an ICSH Tentative Standard. There is little doubt that this presumptive identification of hemoglobin variants by simple electrophoresis will be improved or complemented by the introduction of newer techniques, such as immunologic analysis. However, for the present and for some time to come, the system outlined here should be found valuable. The present report does not concern itself with the numerous auxiliary techniques involved in the identification of abnormal hemoglobins-sickle-cell test, solubility tests, lability test--and no claim is made that the simple system described here eliminates these other techniques from the diagnostic armamentarium of the laboratory.

Electrophoresis↗
PubMed1981

Functional studies of two new abnormal hemoglobins with their mutation located at intersubunit contacts: Hb Hotel Dieu beta99 (G1) Asp replaced by Gly and Hb Pitie Salpetriere beta34 (B16) Val replaced by Phe.

The functional properties of two new abnormal hemoglobins with high oxygen affinity were studied. Hb Hôtel Dieu beta99 (G1) Asp replaced by Gly is situated in the alpha1beta2 contact. Hb Pitié Salpétrière beta34 (B16) Val replaced by Phe is situated in the alpha1beta1 contact. Both hemoglobins exhibited similar functional properties with a 10-fold increased oxygen affinity, a decreased cooperativity, a decreased Bohr effect and a normal or slightly decreased 2,3-diphosphoglycerate effect. The structure-function relationship is discussed in the light of these results.

2,3-Diphosphoglycerate↗
PubMedDate not supplied

A study on the biochemical genetics of abnormal hemoglobins.

In recent years, there were found in China 2 kinds of hemoglobin-M (Hb M Shanghai-1, Hb M Shanghai-2) and 2 kinds of unstable hemoglobin (Hb Shanghai-1, Hb Shanghai-2), amoung which the propositus of Hb Shanghai-2 proved to be a heterozygote for both unstable hemoglobin gene and beta-thalassaemia gene. This article gives an account of the biochemical analysis and genetic studies of these four kinds of abnormal homoglobins. Besides, we present here also the records of 2 family pedigrees of alpha-thalassaemia, and found in the blood of a patient a small quantity of another abnormal hemoglobin constituent in addition to Bart's hemoglobin and hemoglobin H. On these bases, a new hypothesis concerning the heredity of alpha-thalassaemia disease is proposed.

Adult↗