Following is the structural formula of ganglioside GM, a macromolecular gly- colipid (meaning that it contains lipid and monosaccharide units joined by glycosidic bonds). (b) Но CH,OH (d) HO CH,OH (f) NH CH,OH OH (a) C=0- OCH,CHCHCH=CH(CH,)12CH3 OH ОН Но. ČH3 (c) NHC(CH,) 16CH3 (e) НО ОН OH НООС NH HO C=O CH3 Ganglioside GM or Tay-Sachs ganglioside In normal cells, this and other gangliosides are synthesized continuously and degraded by lysosomes, which are cell organelles containing digestive enzymes. If pathways for the degradation of gangliosides are inhibited, the gangliosides accumulate in the cen- tral nervous system, causing all sorts of life-threatening consequences. In inherited diseases of ganglioside metabolism, death usually occurs at an early age. Diseases of ganglioside metabolism include Gaucher's disease, Niemann-Pick disease, and Tay- Sachs disease. Tay-Sachs disease is a hereditary defect that is transmitted as an au- tosomal recessive gene. The concentration of ganglioside GM, is abnormally high in this disease because the enzyme responsible for catalyzing the hydrolysis of glycosidic bond (b) is absent.
Neutral Amino Acids
Amino acids which do not have any charge on them are neutral amino acids.
Globular Protein
The globular proteins refer to the shape of protein specifically spherical in nature apart from spherical form fibrous, disordered and membrane-bound proteins exist. These globular proteins are miscible in water and form a colloidal solution rather than other types which might not exhibit solubility. Many classes of the fold are found in globular proteins, which render them a sphere shape. Globular fold containing proteins usually are referred to by the term globin.
Dimer
Dimers are basic organic compounds, which are derivates of oligomers. It is formed by the combination of two monomers which could potentially be strong or weak and in most cases covalent or intermolecular in nature. Identical monomers are called homodimer, the non-identical dimers are called heterodimer. The method by which dimers are formed is known as “dimerization”.
Dipeptide
A dipeptide is considered a mixture of two distinct amino acids. Since the amino acids are distinct, based on their composition, two dipeptide's isomers can be produced. Various dipeptides are biologically essential and are therefore crucial to industry.
This unit is N-acetylneuraminic acid, the most abundant member of a family of amino sugars containing nine or more carbons and distributed widely throughout the animal kingdom. Draw the open-chain form of this amino sugar. Do not be concerned with the configuration of the five chiral centers in the open-chain form.
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