Lecture 37

Question 1

______, molecules composed of mostly sugar and some protein, are often connective tissue and extra-cellular matrix material. THe sugar part of these molecules is often _________, which are repeat sugar groups with amino or sulfate groups.

Answer 1

Proteoglycans

Glycosaminoglycans

Question 2

______ disease arises when glycosaminoglycans can’t be broken down. Symptoms include short neck, deformed feet, compressed spinal cord, ____ degeneration and clouded ______ (buildup of dermatin sulfate and thus proteoglycans), as well as heart disease. Symptoms arise when patients are between 3 and 8 years of age. Enzyme therapy using ______ is the current treatment.

Answer 2

Hurler’s disease

Retinal

Corneas

Iduronidase

Question 3

______ are mostly comprised of protein with a some sugar (usually not repeats), and are typicaly surface proteins. There are three types.

Type A are ___- linked (e.g. Immunoglobulins). When some of these ___-linked sugars cannot be broken down, you end up with ____-______ (swelling of the frontal portion of the head).

Answer 3

Glycoproteins

N-linked

N-linked

Alpha-Mannosidosis

Question 4

Type B glycoproteins are ______, which are __-linked. These are often present on viruses (e.g. Ebola), and our bodies use these molecules to detect the viruses. One of the lysosomal storage diseases caused by inability to breakdown these __-linked sugars is _____ or Kanzaki disease (caused by alpha-______ B deficiency). Symtptoms include intellectual impairment and _________ (harden and raised areas of skin that are blue or purple in color.)

Answer 4

Mucins

O-linked

O-linked

Schindler

alpha-Galactosidase B

Angiokeratomas

Question 5

Type C glycoproteins are _____. Hydroxyllysine serves as an attachment site for polysaccharides on these glycoproteins. ____-____ syndrome is a disease due to deficiency in lysyl hydroxylase. Symptoms include stretchy skin, hyperextendable joints, and kyphosis/scoliosis.

Answer 5

Collagen

Ehlers-Danlos syndrome

Question 6

____ disease, a lysosomal storage disease involving sugars linked to sugars, arises from the inability to breakdown glycogen. Patients have a deficiency in ____-______, but this can be treated with enzyme therapy.

Answer 6

Pompe

Alpha-glucosidase

Question 7

_____ disease, another lysosomal storage disease, arises from the inability to degrade a sugar-containing lipid biomolecule. Patients have a deficiency in ______. It is the most common lysosomal storage disease.

Answer 7

Gaucher’s

Glucocerebrosidase