Protein as a means of transportation

Protein as a means of transportation
Carrier protein has the ability to bind to specific molecules and transporting various substances through the blood stream. For instance: hemoglobin, consisting of group-containing compound heme iron bound to the protein globin, serves as an oxygen carrier in the blood of vertebrates; hemosianin, befungsi as an oxygen carrier in the blood of some sort of Invertebrate; myoglobin, the oxygen transporter in muscle tissue ; serum albumin, as a transporter of fatty acids in the blood; β-lipoprotein, a lipid transporter in the blood; ceruloplasmin, a copper ion transporters in the blood.
Hemoglobin is metaloprotein (iron-containing protein) in red blood cells that serves as a carrier of oxygen from the lungs throughout the body, in mammals and other animals. Hemoglobin is also the bearer of carbon dioxide back to the lungs to the body exhaled. Hemoglobin molecule consists of globin, Apoprotein, and four heme groups, an organic molecule with an iron atom.

Mutations in these genes lead to a class of proteins hemoglobin decreased disease called hemoglobinopathy, among which the most common is sickle cell anemia and thalassemia.

Thalassemia is a result of an imbalance manufacture chain amino acids that make up hemoglobin contained by red blood cells. Red blood cells carry oxygen around the body with the help of a substance called hemoglobin. Hemoglobin is made of two different kinds of proteins, namely alpha globin and beta globin. Globin protein is made by a gene located on different chromosomes. If one or more of the globin genes that produce abnormal proteins or lost, there will be a decrease in globin proteins that cause thalassemia. Alpha globin gene mutations cause alpha-thalassemia disease and if it occurs in the beta globin causes beta-thalassemia disease.
Treatment of thalassemia depends on the type and severity of the disorder. Someone carriers or who have alpha or beta thalassemia trait tends to be mild or no symptoms and require little or no treatment. There are 3 (standard) general treatment for thalassemia intermediate level or weight, that blood transfusions, iron chelation therapy, and folic acid supplements mmenggunakan. In addition, there are other treatment is bone marrow transplantation cord, cord blood donation, and HLA (human leukocyte antigens).

• Blood transfusion
Transfusion is done is red blood cell transfusions. This therapy is the mainstay of therapy for people who suffer from moderate or severe thalassemia. Blood transfusion is done through the veins and gives red blood cells with normal hemoglobin. To maintain such circumstances, blood transfusions should be routine for within 120 days of the red blood cells will die. Especially for patients with beta thalassemia intermedia, blood transfusions only done occasionally, not regularly. As for the beta thalssemia major (Cooley's anemia) should be done on a regular basis (2 or 4 weeks).

• Iron Chelation Therapy (Iron Chelation)
Hemoglobin in red blood cells is iron-rich protein. When doing regular blood ransfusi can lead to the buildup of iron in the blood. These conditions can damage the liver, heart, and other organs. To prevent this damage, iron chelation therapy is needed to remove excess iron from the body. There are two drugs that are used in iron chelation therapy, namely:

a) Deferoxamine
Deferoxamine is a liquid medicine given by subcutaneous slowly and usually with the help of a small pump that is used in the overnight period. This therapy is time consuming and a bit of give pain. The side effects of this medication can cause loss of vision and hearing.

b) Deferasirox
Deferasirox is a pill taken once a day. The side effects are headache, nausea, vomiting, diarrhea, joint pain, and fatigue (tiredness).

• Folic Acid Supplements
Folic acid is a B vitamin that can help build red blood cells healthy. This supplement should still be taken in addition to a blood transfusion or iron chelation therapy.

• Transplantation of bone marrow from the back
Bone Marrow Transplantation (BMT) has been conducted since 1900. Blood and marrow stem cell transplant replaces the normal stem cells are damaged. Stem cells are cells in the bone marrow to make red blood cells. Stem cell transplantation is the only treatment that can cure thalassemia. However, it has disadvantages because only a small number of people can find a good match between donor and recipient.

• donation of umbilical cord blood (Cord Blood)
Cord blood is the blood in the umbilical cord and placenta. Such as bone marrow, it is a rich source of stem cells, the building blocks of the human immune system. Compared with donor bone marrow, cord blood non-invasive, painless, more inexpensive and relatively simple.

• HLA (human leukocyte antigens)
Human leukocyte antigens (HLA) are proteins found on the surface of cells in the body. Our immune system is to recognize our own cells as 'self,' and cells 'foreign' as opposed to based on HLA proteins displayed on the surface of our cells. In bone marrow transplantation, HLA can prevent the rejection of the body and Graft versus Host Disease (GVHD). HLA best to avoid rejection is to be genetically related to the donor resipen (receiver).