What is this disease related to abnormality in the oxygen-carrying protein haemoglobin that Chukwuedu Nwokolo is carrying out research on?
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Chukwuedu Nwokolo Chukwuedu Nathaniel II Nwokolo (19 April 1921 – 18 May 2014) was an internationally distinguished tropical diseases, nutrition, human, medical, biological and life sciences expert; plus research scientist, scholar, pioneer medical doctor, author, humanitarian and acclaimed professor of medicine. He was Nigerian, and is listed in the "Who's Who in Nigeria" series by Newswatch. Nwokolo was outstandingly recognised worldwide for discovering and mapping out the area of paragonimiasis lung disease in Eastern Nigeria, with a study of the disease in Africa and clinical research for its control. Nwokolo founded SICREP: Sickle Cell Research Programme to effectively fight the disease in Nigeria and globally.
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Sickle-cell disease Sickle-cell disease (SCD) is a group of blood disorders typically inherited from a person's parents. The most common type is known as sickle-cell anaemia (SCA). It results in an abnormality in the oxygen-carrying protein haemoglobin (hemoglobin S) found in red blood cells. This leads to a rigid, sickle-like shape under certain circumstances. Problems in sickle cell disease typically begin around 5 to 6 months of age. A number of health problems may develop, such as attacks of pain ("sickle-cell crisis"), anemia, swelling in the hands and feet, bacterial infections, and stroke. Long term pain may develop as people get older. The average life expectancy in the developed world is 40 to 60 years.
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African Health Sciences African Health Sciences is a peer-reviewed medical journal covering clinical practice and public health policy relevant to Africa and low-income countries. The editor-in-chief is James K. Tumwine (Makerere University), who established the journal in 2001.
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Owen Smith (physician) Owen Patrick Smith {'1': ", '2': ", '3': ", '4': "} is an Irish haematologist and the Regius Professor of Physic at Trinity College, Dublin, a position he has held since 2014. He has also been Professor of Haematology since 2002 and is a principal investigator at the Institute of Molecular Medicine. His research focuses on the protein C activation pathway and the systemic inflammatory response syndrome.
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HBB Beta globin (also referred to as HBB, β-globin, haemoglobin beta, hemoglobin beta, or preferably haemoglobin subunit beta) is a globin protein, which along with alpha globin (HBA), makes up the most common form of haemoglobin in adult humans, the HbA. It is 146 amino acids long and has a molecular weight of 15,867 Da. Normal adult human HbA is a heterotetramer consisting of two alpha chains and two beta chains.
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Anemia of chronic disease Anemia of chronic disease, or anemia of chronic inflammation, is a form of anemia seen in chronic infection, chronic immune activation, and malignancy. These conditions all produce massive elevation of Interleukin-6, which stimulates hepcidin production and release from the liver, which in turn reduces the iron carrier protein ferroportin so that access of iron to the circulation is reduced. Other mechanisms may also play a role, such as reduced erythropoiesis.
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Phytoglobin Phytoglobins are globular plant (algae and land plant) proteins classified into the globin superfamily, which contain a heme, "i"."e". protoporphyrin IX-Fe, prosthetic group. Phytoglobins were discovered in 1939 by Kubo after spectroscopic and chemical analysis of the red pigment of soybean root nodules. A few decades after Kubo's report the crystallization of a lupin phytoglobin (known as leghemoglobin) by Vainshtein and collaborator revealed that the tertiary structure of this protein and that of the sperm whale myoglobin was remarkably similar, thus indicating that the phytoglobin discovered by Kubo did indeed correspond to a globin.
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Protein C deficiency Protein C deficiency is a rare genetic trait that predisposes to thrombotic disease. It was first described in 1981. The disease belongs to a group of genetic disorders known as thrombophilias. Protein C deficiency is associated with an increased incidence of venous thromboembolism (relative risk 8–10), whereas no association with arterial thrombotic disease has been found.
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Leukemia Leukemia, also spelled leukaemia, is a group of cancers that usually begin in the bone marrow and result in high numbers of abnormal white blood cells. These white blood cells are not fully developed and are called "blasts" or "leukemia cells". Symptoms may include bleeding and bruising problems, feeling tired, fever, and an increased risk of infections. These symptoms occur due to a lack of normal blood cells. Diagnosis is typically made by blood tests or bone marrow biopsy.
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Samuel Manuwa Sir Samuel Layinka Ayodeji Manuwa, CMG, OBE (1903–1976) was a pioneering Nigerian surgeon, Inspector General of Medical Services and former Chief Medical Adviser to the Federal Government of Nigeria. He was the first Nigerian to pass the FRCS and he obtained the postgraduate Doctor of Medicine degree from the University of Edinburgh in 1934. In 1966, he was elected president of the World Federation for Mental Health. During his lifetime he served as a Nigerian aristocrat, holding the chieftaincy titles of the Obadugba of Ondoland, the Olowa Luwagboye of Ijebuland and the Iyasere of Itebu - Manuwa, all of the south-western region of the country.
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Chronic eosinophilic leukemia Chronic eosinophilic leukemia (CEL) is a disease in which too many eosinophils (a type of white blood cell) are found in the bone marrow, blood, and other tissues. CEL may stay the same for many years, or it may progress quickly to acute leukemia. It is generally caused by overactivation of the oncogene, e.g. PDGFRA through a chromosome translocation or fusion between two genes on the same chromosome, e.g. "FIP1L1-PDGFRA" gene fusion-induced eosinophilic leukemia. Though a highly rare disease, CEL is extremely manageable with the use of Gleevec, which suppresses the oncogenic effects of PDGFRA.
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Felix Konotey-Ahulu Felix I. D. Konotey-Ahulu (born 12 July 1930) is a Ghanaian physician and scientist who is the Dr Kwegyir Aggrey Distinguished Professor of Human Genetics at the University of Cape Coast, Ghana, and a consultant physician/genetic counsellor, Haemoglobinopathy/Sickle Cell States, in Harley Street, London. He is one of the world’s foremost experts on sickle-cell disease.
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Lagos bat virus Lagos bat virus (LBV) is a lyssavirus of southern and central Africa that causes a rabies-like illness in mammals. It was first isolated from a fruit bat ("Eidolon helvum") from Lagos Island, Nigeria in 1956. Brain samples from the bat showed poor cross-reactivity to rabies antibodies but the virus was found to be closely related to the rabies virus. This was the first discovery of a rabies-related virus. Until this time, rabies was thought to have a single causal agent.
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Gunther disease Gunther disease, also known as congenital erythropoietic porphyria (CEP), uroporphyrinogen III synthase deficiency and UROS deficiency, is a congenital form of erythropoietic porphyria. The word porphyria originated from the Greek word porphura. Porphura actually means "purple pigment", which, in suggestion, the color that the body fluid changes when a person has Gunther's disease. It is a rare, autosomal recessive metabolic disorder affecting heme, caused by deficiency of the enzyme uroporphyrinogen cosynthetase. It is extremely rare, with a prevalence estimated at 1 in 1,000,000 or less. There have been times that prior to birth of a fetus, Gunther's disease has been shown to lead to anemia. In milder cases patients have not presented any symptoms until they have reached adulthood. In Gunther's disease, porphyrins are accumulated in the teeth and bones and an increased amount are seen in the plasma, bone marrow, feces, red blood cells, and urine.
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Sideroblastic anemia Sideroblastic anemia or sideroachrestic anemia is a form of anemia in which the bone marrow produces ringed sideroblasts rather than healthy red blood cells (erythrocytes). In sideroblastic anemia, the body has iron available but cannot incorporate it into hemoglobin, which red blood cells need in order to transport oxygen efficiently. The disorder may be caused either by a genetic disorder or indirectly as part of myelodysplastic syndrome, which can evolve into hematological malignancies (especially acute myelogenous leukemia).
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Spherocytosis Spherocytosis is an auto-hemolytic anemia (a disease of the blood) characterized by the production of spherocytes (red blood cells (RBCs)) or erythrocytes that are sphere-shaped rather than bi-concave disk shaped. Spherocytes are found in all hemolytic anemias to some degree. Hereditary spherocytosis and autoimmune hemolytic anemia are characterized by having "only" spherocytes.
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Myeloma protein A myeloma protein is an abnormal immunoglobulin fragment, such as an immunoglobulin light chain, that is produced in excess by an abnormal monoclonal proliferation of plasma cells, typically in multiple myeloma. Other terms for such a protein are M protein, M component, M spike, spike protein, or paraprotein. This proliferation of the myeloma protein has several deleterious effects on the body, including impaired immune function, abnormally high blood viscosity ("thickness" of the blood), and kidney damage.
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Isuikwuato Isuikwuato is a local government area in Abia State in southeastern Nigeria. The name Isu-Ikwu-Ato translates from Igbo as 'three Isu families or lineage' and refers to the three lineages descended from the Isu people, in what is now a local government area. The three brothers are Imenyi which was Considered the Eldest, Oguduasaa the second eldest and their younger sibling Isuamawo, these three major clans which also harbours some various communities in each of them makes up the present day Isuikwuato.It has an estimated population of over 50,000 people. Isuikwuato has natural resources such as iron ore and kaolin. Oil lines flow through Isuikwuato and there have been cases of burst pipe which have had severe effects on the local economy and environment. The major cash crops are palm oil and cassava. Isuikwuato is also home to Abia State University Uturu.
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Eijkman Institute for Molecular Biology The Eijkman Institute for Molecular Biology is a laboratory in Jakarta, Indonesia. It is most known for the discovery by Christiaan Eijkman that Beriberi was caused by a lack of thiamine in the human body.
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David R. Brown (neuroscientist) Professor David Ronald Brown (born 7 September 1964, Sydney, Australia) is an Australian-born research scientist notable for his work on prion diseases, which include bovine spongiform encephalopathy and vCJD. His most notable research relates to the metal binding of the protein central to these diseases, the prion protein, and its possible cellular role as an antioxidant.
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Bilirubin Bilirubin (formerly referred to as haematoidin and discovered by Rudolf Virchow in 1847) is a yellow compound that occurs in the normal catabolic pathway that breaks down heme in vertebrates. This catabolism is a necessary process in the body's clearance of waste products that arise from the destruction of aged red blood cells. First the hemoglobin gets stripped of the heme molecule which thereafter passes through various processes of porphyrin catabolism, depending on the part of the body in which the breakdown occurs. For example, the molecules excreted in the urine differ from those in the faeces. The production of biliverdin from heme is the first major step in the catabolic pathway, after which the enzyme biliverdin reductase performs the second step, producing bilirubin from biliverdin.
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Human β-globin locus The human β-globin locus is composed of five genes located on a short region of chromosome 11, responsible for the creation of the beta parts (roughly half) of the oxygen transport protein Hemoglobin. This locus contains not only the beta globin gene but also delta, gamma-A, gamma-G, and epsilon globin. Expression of all of these genes is controlled by single locus control region (LCR), and the genes are differentially expressed throughout development.