OCA at 07:20, 12 July 2023

2023-07-12T07:20:59Z

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

-	~~The entry~~ 8fdm is ~~ON HOLD until Paper Publication~~	+	==Human Hemoglobin in Complex with Nitrosomethane==
-		+	<StructureSection load='8fdm' size='340' side='right'caption='[[8fdm]], [[Resolution\|resolution]] 1.91Å' scene=''>
-	~~Authors~~: ~~Powell~~, S.M., ~~Richter~~-~~Addo~~, G.B., ~~Thomas~~, L.M.	+	== Structural highlights ==
-		+	<table><tr><td colspan='2'>[[8fdm]] 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=8FDM OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=8FDM FirstGlance]. <br>
-	~~Description~~: ~~Human Hemoglobin~~ in ~~Complex~~ with ~~Nitrosomethane~~	+	</td></tr><tr id='method'><td class="sblockLbl"><b>[[Empirical_models\|Method:]]</b></td><td class="sblockDat" id="methodDat">X-ray diffraction, [[Resolution\|Resolution]] 1.91Å</td></tr>
-	[[Category: ~~Unreleased Structures~~]]	+	<tr id='ligand'><td class="sblockLbl"><b>[[Ligand\|Ligands:]]</b></td><td class="sblockDat" id="ligandDat"><scene name='pdbligand=GOL:GLYCEROL'>GOL</scene>, <scene name='pdbligand=HEM:PROTOPORPHYRIN+IX+CONTAINING+FE'>HEM</scene>, <scene name='pdbligand=MBN:TOLUENE'>MBN</scene>, <scene name='pdbligand=NSM:NITROSOMETHANE'>NSM</scene>, <scene name='pdbligand=PO4:PHOSPHATE+ION'>PO4</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=8fdm FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=8fdm OCA], [https://pdbe.org/8fdm PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=8fdm RCSB], [https://www.ebi.ac.uk/pdbsum/8fdm PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=8fdm ProSAT]</span></td></tr>
-	[[Category: Powell~~, S.M~~]]	+	</table>
-	[[Category: Thomas~~, L.M~~]]	+	== Disease ==
		+	[https://www.uniprot.org/uniprot/HBB_HUMAN HBB_HUMAN] Defects in HBB 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:186485</ref> <ref>PMID:6259091</ref> <ref>PMID:2599881</ref> <ref>PMID:8704193</ref> Defects in HBB are the cause of beta-thalassemia (B-THAL) [MIM:[https://omim.org/entry/613985 613985]. A form of thalassemia. Thalassemias are common monogenic diseases occurring mostly in Mediterranean and Southeast Asian populations. The hallmark of beta-thalassemia is an imbalance in globin-chain production in the adult HbA molecule. Absence of beta chain causes beta(0)-thalassemia, while reduced amounts of detectable beta globin causes beta(+)-thalassemia. In the severe forms of beta-thalassemia, the excess alpha globin chains accumulate in the developing erythroid precursors in the marrow. Their deposition leads to a vast increase in erythroid apoptosis that in turn causes ineffective erythropoiesis and severe microcytic hypochromic anemia. Clinically, beta-thalassemia is divided into thalassemia major which is transfusion dependent, thalassemia intermedia (of intermediate severity), and thalassemia minor that is asymptomatic.<ref>PMID:1971109</ref> Defects in HBB are the cause of sickle cell anemia (SKCA) [MIM:[https://omim.org/entry/603903 603903]; also known as sickle cell disease. Sickle cell anemia is characterized by abnormally shaped red cells resulting in chronic anemia and periodic episodes of pain, serious infections and damage to vital organs. Normal red blood cells are round and flexible and flow easily through blood vessels, but in sickle cell anemia, the abnormal hemoglobin (called Hb S) causes red blood cells to become stiff. They are C-shaped and resembles a sickle. These stiffer red blood cells can led to microvascular occlusion thus cutting off the blood supply to nearby tissues. Defects in HBB are the cause of beta-thalassemia dominant inclusion body type (B-THALIB) [MIM:[https://omim.org/entry/603902 603902]. An autosomal dominant form of beta thalassemia characterized by moderate anemia, lifelong jaundice, cholelithiasis and splenomegaly, marked morphologic changes in the red cells, erythroid hyperplasia of the bone marrow with increased numbers of multinucleate red cell precursors, and the presence of large inclusion bodies in the normoblasts, both in the marrow and in the peripheral blood after splenectomy.<ref>PMID:1971109</ref>
		+	== Function ==
		+	[https://www.uniprot.org/uniprot/HBB_HUMAN HBB_HUMAN] Involved in oxygen transport from the lung to the various peripheral tissues.<ref>PMID:16904236</ref> LVV-hemorphin-7 potentiates the activity of bradykinin, causing a decrease in blood pressure.<ref>PMID:16904236</ref>
		+	== References ==
		+	<references/>
		+	__TOC__
		+	</StructureSection>
		+	[[Category: Homo sapiens]]
		+	[[Category: Large Structures]]
		+	[[Category: Powell SM]]
		+	[[Category: Richter-Addo GB]]
		+	[[Category: Thomas LM]]

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

2022-12-14T07:42:32Z

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

←Older revision

Revision as of 07:42, 14 December 2022

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

2022-12-14T07:42:31Z

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

New page

'''Unreleased structure'''

The entry 8fdm is ON HOLD until Paper Publication

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

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

8fdm - Revision history

OCA at 07:20, 12 July 2023

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

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