Understanding>>        
Basics of Sickle Cell Who suffers from it? What is Hemoglobin? Fiber formation Protein



Glossary of www.sicklecellinfo.net 

amino acid residues
amino acid-subunit - building blocks that covalently link to form proteins, any of a group of organic molecules that consist of a basic amino group (’NH2), an acidic carboxyl group (’COOH), and an organic R group (or side chain), which is unique to each amino acid 

 

electron microscopy - microscopy technique that attains extremely high resolution using an electron beam instead of a beam of light to illuminate the object of study

 

gene1-the basic unit of heredity. Contains the information for making one RNA, and in most cases, a DNA sequence coding for one polypeptide

 

hemoglobin sub-units
hemoglobin(Hb)
2 - a iron containing, oxygen-carrying protein occuring chiefly in red blood cells of vertebrates.

 

hydrophobic-water-repelling

 

molecules-The smallest units into which a compound can be divided without changing its physical and chemical properties. Molecules are formed from combinations of atoms, the basic building blocks of all matter.

 

polar (amino acids) amino acids that have a region which is partially positive and another region that is partially negative.

 

polypeptide chain2- a molecule made up of amino acids joined by covalent peptide bonds. An amino acid chain that has a 3-dimensional configuration.

 

protein1-A polymer, or polypeptide composed of amino acid sub-units. Sometimes the term protein denotes a functional collection of more than one polypeptides. (e.g. the hemoglobin protein consists of four polypeptides)

 

protein folding-

 

RBC's – red blood cells

 

Sickle Cell trait2 - the phenotype exhibited by individuals heterozygous (presence of HbA and HbS) for the sickle-cell gene. The red blood cells of people with the sickle-cell trait undergo much less sickling because over half their hemoglobin are normal.

Sickle Cell Anemia2 - A genetic disease caused by an autosomal recessive gene (HbS) in which abnormal B globin is produced. Because of a single amino acid change. This blood protein tends to aggregate under low-oxygen conditions, forming fibers and distorting red blood cells into a sickle shape.

 

X-ray crystallography2 - subjected to x-ray bombardment, the x-rays are scattered according to the molecule's atomic structure. The pattern of scatter (diffraction) can be captured on spots on photographic film and analyzed. The use of single-crystals diffract x-rays at 1 A, which is nearly atomic resolution.  Analysis includes shape and regularities within the molecule.


References:

1taken from Genetics 3rd Ed.
Weaver, Hedrick
Wm. C. Brown Publishers
copyrighted 1997 the Mc-Graw Hill companies. Inc. all rights reserved

2taken from Concepts of Genetics 6th Edition