共查询到20条相似文献，搜索用时 15 毫秒
1.
Biosynthesis of hemoglobin Ann Arbor: evidence for catabolic and feedback regulation 总被引：5，自引：0，他引：5
Hemoglobin Ann Arbor, in which arginine replaces leucine in position 80 of the a chain, occurs in aflected individuals in low proportion to hemoglobin A. Biosynthetic studies were perforined on reticulocytes of a patient heterozygous for this hemoglobin. These studies suggested that the low percentage of hemoglobin Ann Arbor is prinlarily due to preferential destruction of the abnormal component. The reduced concentration of alpha Ann Arbor chains was also reflected in a decreased synthesis of normal beta chains. 相似文献
2.
Hemoglobin Gun Hill: deletion of five amino acid residues and impaired heme-globin binding 总被引：10，自引：0，他引：10
Hemoglobin Gun Hill, a new variant of adult hemoglobin, was found in a Caucasian and one of his three daughters. The abnormal hemoglobin had only half of the expected number of heme groups. Five amino acid residues appeared to be missing from the beta-globin chains. These residues occur in linear sequence in normal beta-chains in a region involved in heme-globin binding. A deletion of five amino acids in the beta-chains of hemoglobin Gun Hill is postulated. The most likely mechanism for the origin of such a hemoglobin variant would appear to be unequal crossing-over during meiosis. 相似文献
3.
T Miyaji Y Oba K Yamamoto S Shibata I Iuchi H B Hamilton 《Science (New York, N.Y.)》1968,159(811):204-206
A variant of hemoglobin A, named Hb Hijiyama, found in two generations of a Japanese family living in Hiroshima, Japan, has a higher anodal electrophoretic mobility than hemoglobin A; a gain of two negative charges per molecule is indicated. Fingerprinting and amino acid analysis showed the biochemical anomaly to be in the beta chain at residue 120, where lysine is replaced by glutamic acid. In the heterozygote carriers of the abnormal hemoglobin there is no apparent association with clinical or hematologic abnormalities. 相似文献
4.
Hemoglobin Freiburg: abnormal hemoglobin due to deletion of a single amino acid residue 总被引：5，自引：0，他引：5
R T Jones B Brimhall T H Huisman E Kleihauer K Betke 《Science (New York, N.Y.)》1966,154(752):1024-1027
Structural characterization of a new variant of human hemoglobin (adult), designated hemoglobin Freiburg, indicates the deletion of the valyl residue No. 23 from an otherwise normal beta-chain. The formula may be written (alpha2)beta(2)(23val-0). The abnormal hemoglobin is present with hemoglobin A in the proposita and in two of her three living children, but is not detectable in her parents. We postulate that this variant represents a triplet base deletion which most likely resulted from an unequal crossing-over between two normal betachain loci during meiosis in one of the parents of the proposita. 相似文献
5.
Stuidy of the amino acid composition and amino-terminal residue in the abnormal peptide of an electrophoretically slow hemoglobin revealed that the globin's structure was alpha(2)beta(2)(121 Lys) (Obeta). This hemoglobin had been provisionally classified as Hb E or as a new heemoglobin. The clinical significance of this abnormality is not yet defined. 相似文献
6.
Isolation of an abnormal methemoglobin from two families exhibiting dominantly transmitted cyanosis have permitted the recognition of two different pigments of the hemoglobin M type. It is possible that the abnormal properties which characterize the acidic methemoglobin derivatives result from a crevice configuration of the heme, with two Feprotein bonds. 相似文献
7.
We studied the hemoglobin types of 440 individuals (306 unrelated) cytologically and by paper electrophoresis. These included 334 (252 unrelated) putative "full bloods." No abnormal types were found. 相似文献
8.
A new variant of the normal minor component Hb A(2) has been detected in a family that lives in Sphakiá, Crete. Chemical studies of this abnormal hemoglobin, designated Hb A(2) delta Sphakiá, indicates a substitution of the histidyl residue number two of the delta-chain by an arginyl residue. 相似文献
9.
A dimeric hemoglobin was purified from nitrogen-fixing root nodules formed by association of Rhizobium with a nonleguminous plant, Parasponia. The oxygen dissociation rate constant is probably sufficiently high to allow Parasponia hemoglobin to function in a fashion similar to that of leghemoglobin, by oxygen buffering and transport during symbiotic nitrogen fixation. The identification of hemoglobin in a nonlegume raises important questions about the evolution of plant hemoglobin genes. 相似文献
10.
Hemoglobin M variants, M Iwate, M Boston, M Saskatoon are easily and accurately identified by electron spin resonance with small amounts of patients' blood. In hemoglobin M Iwate and M Boston the electron spin resonance of both fresh blood (unprocessed) and isolated pure ferrihemoglobin M revealed similar signal shapes; whereas that of hemoglobin M Saskatoon was doublet in fresh blood and triplet in pure ferrihemoglobin M. 相似文献
11.
对葡萄血红蛋白基因的结构、进化关系、序列特征及蛋白结构进行剖析。基因结构和蛋白序列特征分析表明:尽管葡萄3个血红蛋白基因外显子个数和保守基序组织结构变异较大,但功能结构域具有高度保守性。结构模拟显示,葡萄VvHb1以单体形式存在,而VvHb2和VvHb3则以二聚体形式存在,尽管它们三维构象以螺旋结构为主,但结合血红素分子活性不同。进化分析揭示,植物血红蛋白具有4个类群,其中葡萄的血红蛋白落入主要类群外,并且单独形成了一个进化分支,说明葡萄血红蛋白基因在物种形成后发生了快速进化和基因扩增现象。 相似文献
12.
甘肃滩羊血红蛋白(Hb)及红细胞蛋白质(Ep)多态性的研究 总被引：6，自引：3，他引：3
运用聚丙烯酰胺凝胶电泳技术(PAGE)对甘肃滩羊产区皋兰、景泰及靖远3县285只滩羊的血红蛋白(Hb)基因座、红细胞蛋白质-1(Ep-1)和红细胞蛋白质-2(Ep-2)基因座多态性进行了检测,结果发现甘肃滩羊Hb基因座存在3种基因型HbAA、HbAB、HbBB,它们受HbA和HbB两个共显性等位基因控制,其中HbB和HbBB在3个群体中均占优势;本研究未在Ep-1和Ep-2基因座上检测到多态性,它们均呈现单态。 相似文献
13.
作者经对太原市北城区和北郊区两个幼儿园118名3～7周岁学龄前儿童进行血清中微量元素钢、铁、锌和血紅蛋白水平测定,并进行相关分析。结果表明:学龄前儿童低锌和低铁与血紅蛋白的关系及低锌与低铁的关系,经单侧相关分析,得出均呈正相关关系,低铁与低锌呈—致性降低;而低铁与低铜无相关关系。 相似文献
14.
Hemoglobin of 30 Macaca mulatta monkeys and of 15 Macaca irus monkeys consisted of one electrophoretic component similar to human hemoglobin A. Twenty-one M. irus monkeys had two types of hemoglobin. In 20 animals the hemoglobin resembled human hemoglobin AJ, and in one animal it resembled human hemoglobin AI. 相似文献
15.
Two populations of North Carolina have been analyzed for hemoglobin patterns by paper electrophoresis. Of 534 Cherokee Indians, both mixed and full bloods, all showed normal hemoglobin. Lumbee Indians of less certain ethnic status had 1.7 percent of hemoglobin S, an equal amount of hemoglobin C, and one possible hemoglobin D trait among 1332 bloods studied. 相似文献
16.
A striking similarity has been found between the composition of peptides obtained from tryptic digestion of normal adult hemoglobin (hemoglobin A) and fetal hemoglobin (hemoglobin F). 相似文献
17.
Structure, dynamics, and reactivity in hemoglobin 总被引：10，自引：0，他引：10
J M Friedman 《Science (New York, N.Y.)》1985,228(4705):1273-1280
The static structure of hemoglobin and its functional properties are very well characterized. It is still not known how energy is stored and used within the structure of the protein to promote function and functional diversity. An essential part of this question is understanding the mechanism through which the overall protein structure (quaternary structure) couples to the local environment about the oxygen binding sites. Time-resolved resonance Raman spectroscopy has been used to probe the vibrational degrees of the freedom of the binding site as a function of protein structure. Comparison of the spectra from both equilibrium and transient forms of deoxy hemoglobin from a variety of mammalian, reptilian, and fish hemoglobins reveals that for each quaternary structure there exist two tertiary states stabilized by the presence or absence of an iron-bound ligand. Pulse-probe Raman experiments show that for photodissociated, ligated hemoglobins the local tertiary structure relaxes at a solution-dependent rate extending from tens of nanoseconds to microseconds. In this local environment, the linkage between the iron and the proximal histidine proves to be the single observed structural feature that responds in a systematic and substantial manner to structural changes in the protein. The additional finding of a correlation between the frequency of the iron-proximal histidine stretching motion (nu Fe-His) and various parameters of ligand reactivity, including geminate recombination, implicates the associated localized structural element in the mechanism of protein control of ligand binding. On the basis of these and related finds, a model is presented to account for both coarse and fine control of ligand binding by the protein structure. 相似文献
18.
A heterodont bivalve mollusk Calyptogena magnifica, from the East Pacific Rise and the Galápagos Rift hydrothermal vent areas, contains abundant hemoglobin in circulating erythrocytes. No other known heterodont clam contains a circulating intracellular hemoglobin. The hemoglobin is tetrameric and has a relatively high oxygen affinity, which varies only slightly between 2 degrees and 10 degrees C. The presence of hemoglobin in the clam may facilitate the transport of oxygen to be used in chemoautotrophic hydrogen sulfide metabolism. 相似文献
19.
The glycosylation of hemoglobin: relevance to diabetes mellitus 总被引：34，自引：0，他引：34
Glucose reacts nonenzymatically with the NH2-terminal amino acid of the beta chain of human hemoglobin by way of a ketoamine linkage, resulting in the formation of hemoglobin AIc. Other minor components appear to be adducts of glucose 6-phosphate and fructose 1,6-diphosphate. These hemoglobins are formed slowly and continuously throughout the 120-day life-span of the red cell. There is a two- to threefold increase in hemoglobin AIc in the red cells of patients with diabetes mellitus. By providing an integrated measurement of blood glucose, hemoglobin AIc is useful in assessing the degree of diabetic control. Furthermore, this hemoglobin is a useful model of nonenzymatic glycosylation of other proteins that may be involved in the long-term complications of the disease. 相似文献
20.
Two genetic sources of variation influence the percentage of sickle cell hemoglobin found in heterozygotes. One factor is strongly related to the percentage of hemoglobin S in the carrier parent and appears to be determined by sickle hemoglobin isoalleles, whereas the other is related to racial background and may well be polygenic. 相似文献