OCA at 07:20, 12 July 2023

2023-07-12T07:20:57Z

←Older revision		Revision as of 07:20, 12 July 2023
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-	'''Unreleased structure'''

-	~~The entry~~ 8fdl is ~~ON HOLD until Paper Publication~~	+	==Human Hemoglobin with Nitrosochloramphenicol==
-		+	<StructureSection load='8fdl' size='340' side='right'caption='[[8fdl]], [[Resolution\|resolution]] 1.75Å' scene=''>
-	~~Authors~~: ~~Powell~~, S.M., ~~Richter~~-~~Addo~~, G.B., ~~Thomas~~, L.M.	+	== Structural highlights ==
-		+	<table><tr><td colspan='2'>[[8fdl]] is a 4 chain structure with sequence from [https://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=8FDL OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=8FDL FirstGlance]. <br>
-	~~Description~~: ~~Human Hemoglobin~~ with ~~Nitrosochloramphenicol~~	+	</td></tr><tr id='method'><td class="sblockLbl"><b>[[Empirical_models\|Method:]]</b></td><td class="sblockDat" id="methodDat">X-ray diffraction, [[Resolution\|Resolution]] 1.75Å</td></tr>
-	[[Category: ~~Unreleased Structures~~]]	+	<tr id='ligand'><td class="sblockLbl"><b>[[Ligand\|Ligands:]]</b></td><td class="sblockDat" id="ligandDat"><scene name='pdbligand=CSD:3-SULFINOALANINE'>CSD</scene>, <scene name='pdbligand=GOL:GLYCEROL'>GOL</scene>, <scene name='pdbligand=HEM:PROTOPORPHYRIN+IX+CONTAINING+FE'>HEM</scene>, <scene name='pdbligand=SO4:SULFATE+ION'>SO4</scene>, <scene name='pdbligand=XQU:nitrosochloramphenicol'>XQU</scene></td></tr>
-	[[Category: ~~Richter-Addo, G.B~~]]	+	<tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[https://proteopedia.org/fgij/fg.htm?mol=8fdl FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=8fdl OCA], [https://pdbe.org/8fdl PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=8fdl RCSB], [https://www.ebi.ac.uk/pdbsum/8fdl PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=8fdl ProSAT]</span></td></tr>
-	[[Category: Powell~~, S.M~~]]	+	</table>
-	[[Category: Thomas~~, L.M~~]]	+	== Disease ==
		+	[https://www.uniprot.org/uniprot/HBA_HUMAN HBA_HUMAN] Defects in HBA1 may be a cause of Heinz body anemias (HEIBAN) [MIM:[https://omim.org/entry/140700 140700]. This is a form of non-spherocytic hemolytic anemia of Dacie type 1. After splenectomy, which has little benefit, basophilic inclusions called Heinz bodies are demonstrable in the erythrocytes. Before splenectomy, diffuse or punctate basophilia may be evident. Most of these cases are probably instances of hemoglobinopathy. The hemoglobin demonstrates heat lability. Heinz bodies are observed also with the Ivemark syndrome (asplenia with cardiovascular anomalies) and with glutathione peroxidase deficiency.<ref>PMID:2833478</ref> Defects in HBA1 are the cause of alpha-thalassemia (A-THAL) [MIM:[https://omim.org/entry/604131 604131]. The thalassemias are the most common monogenic diseases and occur mostly in Mediterranean and Southeast Asian populations. The hallmark of alpha-thalassemia is an imbalance in globin-chain production in the adult HbA molecule. The level of alpha chain production can range from none to very nearly normal levels. Deletion of both copies of each of the two alpha-globin genes causes alpha(0)-thalassemia, also known as homozygous alpha thalassemia. Due to the complete absence of alpha chains, the predominant fetal hemoglobin is a tetramer of gamma-chains (Bart hemoglobin) that has essentially no oxygen carrying capacity. This causes oxygen starvation in the fetal tissues leading to prenatal lethality or early neonatal death. The loss of three alpha genes results in high levels of a tetramer of four beta chains (hemoglobin H), causing a severe and life-threatening anemia known as hemoglobin H disease. Untreated, most patients die in childhood or early adolescence. The loss of two alpha genes results in mild alpha-thalassemia, also known as heterozygous alpha-thalassemia. Affected individuals have small red cells and a mild anemia (microcytosis). If three of the four alpha-globin genes are functional, individuals are completely asymptomatic. Some rare forms of alpha-thalassemia are due to point mutations (non-deletional alpha-thalassemia). The thalassemic phenotype is due to unstable globin alpha chains that are rapidly catabolized prior to formation of the alpha-beta heterotetramers. Note=Alpha(0)-thalassemia is associated with non-immune hydrops fetalis, a generalized edema of the fetus with fluid accumulation in the body cavities due to non-immune causes. Non-immune hydrops fetalis is not a diagnosis in itself but a symptom, a feature of many genetic disorders, and the end-stage of a wide variety of disorders. Defects in HBA1 are the cause of hemoglobin H disease (HBH) [MIM:[https://omim.org/entry/613978 613978]. HBH is a form of alpha-thalassemia due to the loss of three alpha genes. This results in high levels of a tetramer of four beta chains (hemoglobin H), causing a severe and life-threatening anemia. Untreated, most patients die in childhood or early adolescence.<ref>PMID:10569720</ref>
		+	== Function ==
		+	[https://www.uniprot.org/uniprot/HBA_HUMAN HBA_HUMAN] Involved in oxygen transport from the lung to the various peripheral tissues.
		+	== References ==
		+	<references/>
		+	__TOC__
		+	</StructureSection>
		+	[[Category: Homo sapiens]]
		+	[[Category: Large Structures]]
		+	[[Category: Powell SM]]
		+	[[Category: Richter-Addo GB]]
		+	[[Category: Thomas LM]]

OCA: Protected "8fdl" [edit=sysop:move=sysop]

2022-12-14T07:42:29Z

Protected "8fdl" [edit=sysop:move=sysop]

←Older revision

Revision as of 07:42, 14 December 2022

OCA: New page: '''Unreleased structure''' The entry 8fdl is ON HOLD until Paper Publication Authors: Powell, S.M., Richter-Addo, G.B., Thomas, L.M. Description: Human Hemoglobin with Nitrosochloramph...

2022-12-14T07:42:29Z

New page: '''Unreleased structure''' The entry 8fdl is ON HOLD until Paper Publication Authors: Powell, S.M., Richter-Addo, G.B., Thomas, L.M. Description: Human Hemoglobin with Nitrosochloramph...

New page

'''Unreleased structure'''

The entry 8fdl is ON HOLD until Paper Publication

Authors: Powell, S.M., Richter-Addo, G.B., Thomas, L.M.

Description: Human Hemoglobin with Nitrosochloramphenicol
[[Category: Unreleased Structures]]
[[Category: Richter-Addo, G.B]]
[[Category: Powell, S.M]]
[[Category: Thomas, L.M]]

8fdl - Revision history

OCA at 07:20, 12 July 2023

OCA: Protected "8fdl" [edit=sysop:move=sysop]

OCA: New page: '''Unreleased structure''' The entry 8fdl is ON HOLD until Paper Publication Authors: Powell, S.M., Richter-Addo, G.B., Thomas, L.M. Description: Human Hemoglobin with Nitrosochloramph...