Alpha chain
Alpha Chain
The term "alpha chain" can refer to several different biological molecules, each playing a crucial role in various physiological processes. In the context of biochemistry and molecular biology, alpha chains are often associated with proteins and polypeptides that are integral to the structure and function of larger complexes.
Hemoglobin Alpha Chain
The hemoglobin alpha chain is one of the two types of polypeptide chains that make up the hemoglobin molecule, the protein responsible for oxygen transport in the blood. Hemoglobin is a tetramer composed of two alpha chains and two beta chains. Each alpha chain consists of 141 amino acids and is encoded by the HBA1 and HBA2 genes located on chromosome 16 in humans.
Function
The primary function of the hemoglobin alpha chain is to bind with the beta chain to form a stable hemoglobin molecule capable of carrying oxygen from the lungs to tissues throughout the body. The alpha chain contains a heme group, which is an iron-containing porphyrin ring that binds oxygen molecules.
Clinical Significance
Mutations in the genes encoding the alpha chain can lead to alpha thalassemia, a blood disorder characterized by reduced production of hemoglobin. This can result in anemia and other related health issues. Alpha thalassemia is more prevalent in certain populations, including those of Southeast Asian, Mediterranean, and African descent.
T-cell Receptor Alpha Chain
The T-cell receptor (TCR) alpha chain is a component of the T-cell receptor complex found on the surface of T lymphocytes, which are critical for the adaptive immune response. The TCR is composed of an alpha chain and a beta chain, both of which are necessary for antigen recognition.
Structure and Function
The TCR alpha chain is encoded by the TRA gene locus and undergoes somatic recombination to generate a diverse repertoire of receptors capable of recognizing a wide array of antigens presented by the major histocompatibility complex (MHC) molecules. The alpha chain pairs with the beta chain to form the antigen-binding site of the TCR.
Clinical Relevance
Defects in TCR alpha chain recombination can lead to immunodeficiencies, where the immune system is unable to effectively respond to infections. Additionally, aberrant TCR signaling can contribute to autoimmune diseases and certain types of cancer.
Also see
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Contributors: Prab R. Tumpati, MD