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Topic: Globin

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Dalton

	Dissection of the Enhancer Activity of {beta}-Globin 5' DNase I- Hypersensitive Site 2 in Transgenic Mice -- Liu et ...
		The ß-globin locus control region (LCR) consists of four erythroid-specific DNase I-hypersensitive sites, which are necessary for high-level expression of the ß-like globin genes in erythroid tissues.
		Mutation of a transcriptional motif of a distant regulatory element reduces the expression of embryonic and fetal globin genes
		Activation of the {beta}-like globin genes in transgenic mice is dependent on the presence of the {beta}-locus control region
	www.pnas.org /cgi/content/abstract/89/9/3899 (0 words)

	Beta Globin Testing - Hemoglobin Disorders, Sickle Cell Disease, Beta Thalassemia Plus - Ambry Genetics
		Approximately 500 beta globin mutations have been discovered, many of which cause serious clinical effects.
		Identification of specific beta globin mutations can confirm a differential diagnosis and improve care for the patient as well as for the patient’s family.
		The Ambry Test: Beta Thalassemia Plus is designed and validated to be capable of detecting about 97-99% of the disease-causing mutations (considering the other ~1-3% to be gross abnormalities, variants in non-coding regions including introns and untranslated sequences, gross deletions, or unknown abnormalities).
	www.ambrygen.com /ts/ts_betaglobin.aspx (975 words)

	Hemoglobinopathies
		Alpha subunits are rapidly degraded in the absence of a partner from the beta-globin gene cluster (gamma, delta, beta globin subunits).
		During normal embryonic development, the episilon gene of the alpha globin gene locus combines with genes from the beta globin locus to form functional hemoglobin molecules.
		This syndrome is a compound heterozygous state of the alpha globin gene cluster.
	sickle.bwh.harvard.edu /hemoglobinopathy.html (1416 words)

	Hemoglobin Synthesis
		The genes in the beta globin locus are arranged sequentially from 5' to 3' beginning with the gene expressed in embryonic development (the first 12 weeks after conception; called episolon).
		The globin genes are activated in sequence during development, moving from 5' to 3' on the chromosome.
		For the beta globin gene cluster, the epsilon gene is expressed initially during embryogensis.
	sickle.bwh.harvard.edu /hbsynthesis.html (1077 words)

	Beta Globin
		The globins are the protein products of a large and ancient multigene family, including the alpha and beta globins of hemoglobin, myoglobin, and even the leghemoglobins of plants.
		The common function of the globins is to bind oxygen.
		The beta globins of four mammals are represented in this piece: human, whale, bat and echidna (an egg-laying mammal).
	www.algoart.com /music/bglobin.htm (272 words)

	BioMed Central \| Full text \| Search for antisense copies of beta-globin mRNA in anemic mouse spleen
		Contrary to earlier reports, induction of a major increase in globin transcripts in the mouse spleen was not associated with a detectable level of antisense RNA to globin mRNA.
		Relative to DNA markers, the size of the globin species was around 600 nt, as expected for the globin mRNA [20].
		Second, and of more significance, not only were the signal strengths of putative antisense relative to sense globin the same (about 3%) for each of the 6 spleen samples (lanes 4-9, panels C and D) but the same ratio was obtained for the standards of antisense and sense standards, that were synthesized in vitro.
	www.biomedcentral.com /1471-2091/2/3 (3149 words)

	Hemoglobin Home
		Hemoglobin is a tetramer composed of 4 globin molecules; 2 alpha globins and 2 beta globins.
		The alpha globin chain is composed of 141 amino acids and the beta globin chain is composed of 146 amino acids (Perutz, 1978).
		Therefore, the globin molecules allow the iron atom to form loose bonds with the oxygen, and therefore, the ability to bind to oxygen and then release it into the tissues without becoming permanently oxidized in the process.
	www.bio.davidson.edu /Courses/Molbio/MolStudents/spring2005/Heiner/hemoglobin.html (1648 words)

	Thalassemia \| AHealthyMe.com (Site not responding. Last check: )
		No beta globin is produced with a beta0 mutation, and only a small fraction of the normal amount of beta globin is produced with a beta+ mutation.
		When an individual has one normal beta globin gene and one with a beta thalassemia mutation, he or she is said to carry the beta thalassemia trait.
		Because alpha globin is a necessary component of all major hemoglobins and some minor hemoglobins, absence of all functioning alpha globin genes leads to serious medical consequences that begin even before birth.
	www.ahealthyme.com /topic/topic100587547 (5079 words)

	Center for BioMolecular Modeling: Welcome to the Center for BioMolecular Modeling
		Globin is one of those proteins; it is composed of eight alpha helical segments, each connected by a short, variable non-helical segment.
		Getting a globin pdb file is not straightforward, since there is no such file called “globin”; rather, it is part of a file for hemoglobin.
		After students have constructed their helix, they should be able to examine the model and determine which “side” of the helix is directed toward the center of the protein, and which side is exposed to the outside surface of the protein.
	www.rpc.msoe.edu /cbm2/globin4.htm (1643 words)

	Myoglobin and Hemoglobin
		The a-globin genes are on chromosome 16 and the b-globin genes are on chromosome 11.
		Given the pattern of globin gene activity throughout fetal development and in the adult the composition of the hemoglobin tetramers is of course distinct.
		The a-globin homotetramers are extremely insoluble which leads to premature red cell destruction in the bone marrow and spleen.
	web.indstate.edu /thcme/mwking/hemoglobin-myoglobin.html (4119 words)

	Genomics\|HuGENet\|Reviews\|Hb S Allele and Sickle Cell Disease\|PubMed ID: 10791557
		Two beta globin protein chains combine with two alpha globin protein chains and a heme to form the predominant hemoglobin found in human adults, Hb A. At least 476 beta globin gene variants exist and several result in life threatening illness (1).
		Haplotype of the beta globin gene cluster (14), as well as alpha globin gene complement (11,15,16) and levels of fetal hemoglobin (Hb F) (16) have all been shown to be associated with clinical expression of sickle cell disease.
		Coinheritance of the alpha globin gene variant, alpha-thalassemia, in individuals with sickle cell disease appears to be protective against some sickle cell complications such as acute chest syndrome, anemia and cerebrovascular accidents (11,15), but it increases susceptibility to other sickle cell complications such as pain crises (11).
	www.cdc.gov /genomics/hugenet/reviews/sickle.htm (3441 words)

	Hemoglobin Summary
		The name hemoglobin is the concatenation of heme and globin, reflecting the fact that each subunit of hemoglobin is a globular protein with an embedded heme (or haem) group; each heme group contains an iron atom, and this is responsible for the binding of oxygen.
		Mutations in the globin chain are associated with the hemoglobinopathies, such as sickle-cell disease and thalassemia.
		Since many of these proteins contain globins, and also the heme moiety (iron in a flat porphyrin support), these substances are often simply referred to as hemoglobins, even if their overall tertiary structure is very different from that of vertebrate hemoglobin.
	www.bookrags.com /Hemoglobin (3946 words)

	Center for BioMolecular Modeling: Welcome to the Center for BioMolecular Modeling
		This is followed by information about the different globin genes (epsilon, G-gamma, A-gamma, delta, and beta) and then “features”; scrolling almost to the bottom of the page you will find the complete sequence of 73,308 nucleotides located at the tip of chromosome 11.
		Construct a paper model of the beta globin gene by cutting the nucleotide sequence (and the accompanying amino acid sequence) of the "Map", found at the back of these instructions, and taping them together to form one long, continuous "gene".
		The replacement of a negatively-charged glutamic acid with a hydrophobic valine at this position of the beta globin protein is responsible for the aggregation of hemoglobin in red blood cells under reduced oxygen concentration, that results in the sickling of the cells.
	www.rpc.msoe.edu /cbm2/globin3.htm (2239 words)

	Looking for the Globin Switch
		According to Goldstrohm's thesis, globins go way back -- "well over 1 billion years, based on their existence in bacteria and lower eucaryotes," simple cells, like yeast, with well-defined nuclei.
		At 600 million years ago, the globins in animals with jaws began to differ from that found in jawless ones.
		What he wanted was the protein, the "transcription factor." Before any copying of the gene could start -- way before that RNA copy could itself be turned into a globin protein -- this transcription factor would have to arrive, settle into its favorite chair, and create the proper environment.
	www.rps.psu.edu /sep96/globin.html (2213 words)

	Induction of Human Fetal Globin Gene Expression by a Novel Erythroid Factor, NF-E4 -- Zhou et al. 20 (20): 7662 -- ...
		The ultimate goal of defining factors which activate fetal globin is their potential for therapeutic intervention in the hemoglobinopathies.
		Globin gene expression in hereditary persistence of fetal hemoglobin and
		globin genes of a prosimian primate (Galago crassicaudatus).
	mcb.asm.org /cgi/content/full/20/20/7662 (7600 words)

	Substitution of the Human beta -Spectrin Promoter for the Human Agamma -Globin Promoter Prevents Silencing of a Linked ...
		in the globin genes that are expressed (51).
		for high-level expression of globin genes in transgenic mice (15,
		An embryonic pattern of expression of a human fetal globin gene in transgenic mice.
	mcb.asm.org /cgi/content/full/18/11/6634 (3693 words)

	Coregulated human globin genes are frequently in spatial proximity when active -- Brown et al. 172 (2): 177 -- The ...
		Globin gene transcription in nonerythroid cells and sorted successive stages of differentiating erythroblasts.
		globin genes at nuclear speckles is not obligatory for transcription.
		Globin gene activation during haemopoiesis is driven by protein complexes nucleated by GATA-1 and GATA-2.
	www.jcb.org /cgi/content/full/172/2/177 (7007 words)

	NIH Guide: DEVELOPMENTAL PROCESSES IN DIFFERENTIAL EXPRESSION OF GLOBIN GENES
		RESEARCH OBJECTIVES Background Mutations in the globin gene cluster are among the most common inherited diseases in humans, leading to disorders that affect many individuals in the U.S. and around the world, such as sickle cell anemia and thalassemia (Cooley's anemia).
		In addition, studies of the developmental- and tissue-specific control of the globin gene cluster are important for optimizing gene transfer, via viral or chemical vectors, for gene replacement therapy.
		The temporal processes dictating globin gene expression and the mechanisms for the developmental timetable are important unresolved issues in erythropoiesis of relevance to this initiative.
	grants.nih.gov /grants/guide/pa-files/PAS-98-060.html (2014 words)

	Hemoglobinopathies and Thalassemias
		Congenital absence of NADH-diaphorase causes an accumulation of metheme, despite the fact that the structure of the globin chain is normal.
		Certain abnormalities in the globin chain sequence produce a hemoglobin that is intrinsically unstable.
		Whatever the genetics, the clinical problem in the thalassemias is the inability to maintain a balance between the synthesis rate of one type of globin chain vis-à-vis that of its mate.
	web2.iadfw.net /uthman/hemoglobinopathy/hemoglobinopathy.html (4522 words)

	GLOBIN GENES (Site not responding. Last check: )
		To determine which sequences are necessary for correct expression of globin genes, genes with different amounts of flanking DNA were introduced into cultured erythroid cells and their expression measured.
		globin in the liver in the early foetus.
		The use of transgenic mice therefore allows us to ask questions about the stage specificity as well as the tissue specificity of globin gene expression and, by comparison with levels of the endogenous mouse globins, ask whether the introduced gene is being expressed at the correct level.
	bssv01.lancs.ac.uk /ADS/BIOS336/336L9.html (2243 words)

	Functional and comparative analysis of globin loci in pufferfish and humans -- Gillemans et al. 101 (7): 2842 -- Blood (Site not responding. Last check: )
		Both pufferfish globin loci are flanked by a highly conserved gene encoding a protein homologous to Drosophila rhomboid.
		Globin loci of the pufferfish F rubripes are located on different chromosomes.
		One of the aims of the present study was to gain insight in the regulatory mechanisms underlying globin gene expression.
	www.bloodjournal.org /cgi/content/full/101/7/2842 (4730 words)

	Department Of Medicine
		It is a complex molecule consisting of 4 globin chains (polypeptide chains or proteins) with a heme group inserted into each globin "pocket".
		The heme group consists of a porphyrin ring and an iron (Fe) atom occupying the center of the ring.
		It is the iron atom that binds oxygen for transport through the blood.
	www.urmc.rochester.edu /medicine/genetics/hemoglobin.aspx (166 words)

	GEORGE LE'S BETA-GLOBIN SITE (Site not responding. Last check: )
		The ß-globin gene is one of a series of members of the Globin family of genes at the ß-globin locus of chromosome 11.
		However, although the genes belong to the same family of Globin genes, the alpha genes are located on another chromosome in the human genome, which is chromosome 16.
		Since two of each globin gene, ß and alpha, are needed to make a functional Hemoglobin protein, the expression of the genes should be balanced.
	student.ucr.edu /~leg01 (1707 words)

	[No title]
		The major groups of globins are hemoglobins (Hb) and myoglobins (Mg) from vertebrates, invertebrate globins, leghemoglobins from plants, and flavohemoproteins from bacteria.
		Molluscs generally have one or two muscle globins which are either monomeric or dimeric, while insects, such as the midge Chironomus thummi, have a large set of extracellular globins.
		Nematodes and annelids have a variety of intracellular and extracellular globins, some of them are multi-domain polypeptides (from two up to nine-domain globins), and some produce large, disulphide-bonded aggregates.
	www.ebi.ac.uk /interpro/IEntry?ac=IPR000971 (529 words)

	Beta Globin: Genetic Music
		The globins are the protein products of a large and ancient multigene family, including the alpha and beta globins of hemoglobin (image left), myoglobin, and even the leghemoglobins of plants.
		In this setting, the beta globins of four mammals are represented: human, whale, bat and echidna (an egg-laying mammal).
		The second beta globin was set by M. Clark, using John Dunn's recently developed BankStep program.
	www.whozoo.org /mac/Music/bglobin.htm (409 words)

	The human {beta}-globin locus control region: A center of attraction -- Levings and Bungert 269 (6): 1589 -- FEBS ... (Site not responding. Last check: )
		It should be noted that additional HS sites were mapped 5' to HS5 [95], but it is currently not known whether these sites participate in globin gene regulation or whether they are associated with the regulation of genes located upstream of the globin locus.
		We propose that the initial events in activating the human globin gene locus during differentiation involves the partial unfolding of the chromatin structure into a DNase I-sensitive domain and the binding of protein complexes to the LCR HS sites.
		Once the LCR holocomplex is generated, the globin locus is relocated to an area of the nucleus enriched for macromolecular complexes involved in coactivation, chromatin remodeling (or modification of histone tails) and transcription.
	content.febsjournal.org /cgi/content/full/269/6/1589 (7310 words)

	Beta Globin: Genetic Music
		The alpha and beta globins, which combine to form the hemoglobin (image left) of red blood cells, are members of a large and ancient multigene family.
		Although some amino acid substitutions in the beta globin sequence can render the molecule nonfunctional (as in sickle-cell anemia), many others are tolerated, and have accumulated over the millions of years that separate the major lineages of the placental mammals.
		These changes can be seen as improvisational riffs by different species on the basic beta globin sequence.
	www.whozoo.org /mac/Music/Primer/Primer_bglobin.htm (118 words)

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