WordNet

fasten or secure with chains; "Chain the chairs together"
a unit of length
a necklace made by a stringing objects together; "a string of beads"; "a strand of pearls"; (同)string, strand
a linked or connected series of objects; "a chain of daisies"
a series of (usually metal) rings or links fitted into one another to make a flexible ligament
anything that acts as a restraint
(chemistry) a series of linked atoms (generally in an organic molecule) (同)chemical chain
a series of things depending on each other as if linked together; "the chain of command"; "a complicated concatenation of circumstances" (同)concatenation
(business) a number of similar establishments (stores or restaurants or banks or hotels or theaters) under one ownership
connect or arrange into a chain by linking
a colorless protein obtained by removing heme from hemoglobin; the oxygen carrying compound in red blood cells (同)hematohiston, haematohiston
British biochemist (born in Germany) who isolated and purified penicillin, which had been discovered in 1928 by Sir Alexander Fleming (1906-1979) (同)Ernst Boris Chain, Sir Ernst Boris Chain
bound with chains; "enchained demons strained in anger to gnaw on his bones"; "prisoners in chains" (同)enchained

PrepTutorEJDIC

『鎖』;(装飾用の)鎖 / 《複数形で》『束縛』,拘束;囚人をつなぐ鎖 / (物事の)『連続』,つながり《+『of』+『名』》 / (商店・銀行・ホテルなどの)チェーン(一連の店が同一資本のもとで連携して経営される方式;その店) / チェーン(測量で用いられる単位;約21.7m) / …'を'『鎖でつなぐ』《+『up』(『together』)+『名,』+『名』+『up』(『together』)》
(液体の)小滴,しずく / (粘土・クリームなどの)球形の固まり

UpToDate Contents

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1. βサラセミアの病態生理 pathophysiology of beta thalassemia
2. 正常なヘモグロビンの構造および機能 structure and function of normal hemoglobins
3. αサラセミアの病態生理 pathophysiology of alpha thalassemia
4. サラセミア症候群の分子病理 molecular pathology of the thalassemic syndromes
5. 鎌状細胞貧血における臨床的ばらつき clinical variability in sickle cell anemia

English Journal

Nonenzymatic polyubiquitination of expressed proteins.

Hemantha HP, Bavikar SN, Herman-Bachinsky Y, Haj-Yahya N, Bondalapati S, Ciechanover A, Brik A.Author information Department of Chemistry, Ben-Gurion University of the Negev , Beer Sheva 84105, Israel.AbstractUbiquitination is one of the most ubiquitous posttranslational modifications in eukaryotes and is involved in various cellular events such as proteasomal degradation and DNA repair. The overwhelming majority of studies aiming to understand ubiquitination and deubiquitination have employed unanchored ubiquitin chains and mono-ubiquitinated proteins. To shed light on these processes at the molecular level, it is crucial to have facile access to ubiquitin chains linked to protein substrates. Such conjugates are highly difficult to prepare homogenously and in workable quantities using the enzymatic machinery. To address this formidable challenge we developed new chemical approaches to covalently attach ubiquitin chains to a protein substrate through its Cys residue. A key aspect of this approach is the installation of acyl hydrazide functionality at the C-terminus of the proximal Ub, which allows, after ubiquitin chain assembly, the introduction of various reactive electrophiles for protein conjugation. Employing α-globin as a model substrate, we demonstrate the facile conjugation to K48-linked ubiquitin chains, bearing up to four ubiquitins, through disulfide and thioether linkages. These bioconjugates were examined for their behavior with the USP2 enzyme, which was found to cleave the ubiquitin chain in a similar manner to unanchored ones. Furthermore, proteasomal degradation study showed that di-ubiquitinated α-globin is rapidly degraded in contrast to the mono-ubiquitinated counterpart, highlighting the importance of the chain lengths on proteasomal degradation. The present work opens unprecedented opportunities in studying the ubiquitin signal by enabling access to site-specifically polyubiquitinated proteins with an increased size and complexity.
Journal of the American Chemical Society.J Am Chem Soc.2014 Feb 12;136(6):2665-73. doi: 10.1021/ja412594d. Epub 2014 Jan 31.
Ubiquitination is one of the most ubiquitous posttranslational modifications in eukaryotes and is involved in various cellular events such as proteasomal degradation and DNA repair. The overwhelming majority of studies aiming to understand ubiquitination and deubiquitination have employed unanchored
PMID 24437386

[Association between hemoglobin Groene Hart and hemoglobin J-Paris-I: First case in Spain.]

de la Fuente-Gonzalo F1, Ropero P2, Martínez-Nieto J2, Villegas A2, González FA2, Díaz-Mediavilla J2.Author information 1Servicio de Hematología, Hospital Clínico San Carlos, Madrid, España. Electronic address: felixfuenteg@hotmail.com.2Servicio de Hematología, Hospital Clínico San Carlos, Madrid, España.AbstractBACKGROUND AND OBJECTIVE: Thalassemias are the most frequent monogenic disorder around the world. α-thalassemias are due to a deficiency of synthesis in the alpha-globin chain of the hemoglobin (Hb). Hb Groene Hart is a hyperunstable variant. In this work, we have studied 24 cases affected by Hb Groene Hart, one of them associated with Hb J-Paris-I.
Medicina clinica.Med Clin (Barc).2014 Feb 11. pii: S0025-7753(14)00015-3. doi: 10.1016/j.medcli.2013.10.026. [Epub ahead of print]
BACKGROUND AND OBJECTIVE: Thalassemias are the most frequent monogenic disorder around the world. α-thalassemias are due to a deficiency of synthesis in the alpha-globin chain of the hemoglobin (Hb). Hb Groene Hart is a hyperunstable variant. In this work, we have studied 24 cases affected by Hb Gr
PMID 24530052

Chronic inflammatory state in sickle cell anemia patients is associated with HBB(*)S haplotype.

Bandeira IC1, Rocha LB2, Barbosa MC3, Elias DB2, Querioz JA4, Freitas MV4, Gonçalves RP4.Author information 1Post-Graduate Program in Pathology, Federal University of Ceará, Fortaleza, Ceará, Brazil. Electronic address: izabelcristina.farmacia@yahoo.com.br.2Post-Graduate Program in Development and Technological Innovation in Drugs, Federal University of Ceará, Fortaleza, Ceará, Brazil.3Post-Graduate Program in Pharmaceutical Sciences, Federal University of Ceará, Fortaleza, Ceará, Brazil.4Post-Graduate Program in Pathology, Federal University of Ceará, Fortaleza, Ceará, Brazil.AbstractThe chronic inflammatory state in sickle cell anemia (SCA) is associated with several factors such as the following: endothelial damage; increased production of reactive oxygen species; hemolysis; increased expression of adhesion molecules by leukocytes, erythrocytes, and platelets; and increased production of proinflammatory cytokines. Genetic characteristics affecting the clinical severity of SCA include variations in the hemoglobin F (HbF) level, coexistence of alpha-thalassemia, and the haplotype associated with the HbS gene. The different haplotypes of SCA are Bantu, Benin, Senegal, Cameroon, and Arab-Indian. These haplotypes are associated with ethnic groups and also based on the geographical origin. Studies have shown that the Bantu haplotype is associated with higher incidence of clinical complications than the other haplotypes and is therefore considered to have the worst prognosis. This study aimed to evaluate the profile of the proinflammatory cytokines interleukin-6, tumor necrosis factor-α, and interleukin-17 in patients with SCA and also to assess the haplotypes associated with beta globin cluster S (HBB(*)S). We analyzed a total of 62 patients who had SCA and had been treated with hydroxyurea; they had received a dose ranging between 15 and 25 (20.0±0.6)mg/kg/day for 6-60 (18±3.4)months; their data were compared with those for 30 normal individuals. The presence of HbS was detected and the haplotypes of the beta S gene cluster were analyzed by polymerase chain reaction-restriction fragment length polymorphism (PCR-RFLP). Our study demonstrated that SCA patients have increased inflammatory profile when compared to the healthy individuals. Further, analysis of the association between the haplotypes and inflammatory profile showed that the levels of IL-6 and TNF-α were greater in subjects with the Bantu/Bantu haplotype than in subjects with the Benin/Benin haplotype. The Bantu/Benin haplotype individuals had lower levels of cytokines than those with the Bantu/Bantu haplotype and greater levels than those of subjects with the Benin/Benin haplotype. For IL-17, a slight trend toward decreased levels was observed in the subjects with the Benin/Benin haplotype, when compared to those with the Bantu/Bantu and Bantu/Benin haplotypes; however, this difference was not statistically significant. Our results show that genetic polymorphisms in sickle cell anemia are associated with the inflammatory profile.
Cytokine.Cytokine.2014 Feb;65(2):217-21. doi: 10.1016/j.cyto.2013.10.009. Epub 2013 Nov 27.
The chronic inflammatory state in sickle cell anemia (SCA) is associated with several factors such as the following: endothelial damage; increased production of reactive oxygen species; hemolysis; increased expression of adhesion molecules by leukocytes, erythrocytes, and platelets; and increased pr
PMID 24290434