Definitions Quiz

Glossary

Gangliosidosis:any of a group of lysosomal storage diseases marked by accumulation of gangliosides GM1 or GM2 and related glycoconjugates due to deficiency of specific lysosomal hydrolases, and by progressive psychomotor deterioration, usually beginning in infancy or childhood and usually fatal
GM2 gangliosidosis: that due to deficiency of activity of specific hexosaminidase isozymes, with accumulation of ganglioside GM2 and related glycoconjugates; it occurs as three biochemically distinct variants, including Sandhoff's disease and Tay-Sachs disease.
Glycoconjugate: any of the complex molecules containing glycosidic linkages, such as glycolipids, glycopeptides, oligosaccharides, or glycosaminoglycans
In Vivo: within a living organism
Ex Vivo: outside the living organism
Hexosaminidase A: an enzyme that speed up the biodegradation of fatty acid.
Lysosome: are organelles that contain digestive enzymes (acid hydrolases) to digest macromolecules. They are found only in animal cells, and are built in the Golgi apparatus.
Cherry-red: is a finding in the macula of the eye in a variety of lipid storage disorders and in central retinal artery occlusion.
Vector: any agent that acts as a carrier or transporter, as a virus or plasmid that conveys a genetically engineered DNA segment into a host cell.
Substrate: the substance on which an enzyme acts.
Nerve cells: the body of a neuron without its axon and dendrites.

All the context of these definitions that we used are taken from this website http://dictionary.reference.com

Quiz

1. Who discovered Tay-Sachs disease?
a. Warren Tay
b. Bernard Sachs
c. a and b
d none of the above
2. What is the other term for Tay-Sachs disease?
a. GM2 gangliosidosis
b. Hexosaminidase A defieciency
c. a and b
d. none of the above
3. The Hexa A enzyme is too large to pass through the blood into the brain through the _.
a. immune system
b. Blood-brain barrier
c. a and b
d. none of the above
4. Hexosaminidase A is an important enzyme, which found in lysosomes that breaks down.
a. Lipid
b. Carbohydrate
c. a and b
d. none of the above
5. The symptoms of Tay Sachs disease includes:
a. Seizure
b. cherry red spot
c. difficulties of swallowing food
d. all of the above

Answers:
1. c. a and b
2. c. a and b
3. b. Blood-brain barrier
4. a. Lipid
5. d. all of the above

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