Lecture 37

Question 1

The main difference between Glycoproteins and Proteoglycan is the _____ of their carbohydrate side chains. Typically, glycoproteins have shorter _______saccharide chains while Proteoglycans have longer chains.

Answer 1

They differ in the LENGTH of their carb side chains. Typically, Glycoproteins have shorter OLIGOSACCHARIDE chains.

Question 2

The addition of carb side chains to proteoglycans and glycoproteins occurs during ___-_______ modification.

Answer 2

POST-TRANSLATIONAL

Question 3

_____ is a proteoglycan well known for inhibiting blood clotting.

Answer 3

HEPARIN

Question 4

Typically, O-linked glycoproteins exhibit an O-glycosidic link between glactose/glucose and either _____ or _____.

Answer 4

SERINE or THREONINE

Question 5

N-glycosidic linkage in glycoproteins occus between N-acetylglucosamine and _____

Answer 5

ASPARIGINE

Question 6

There are two types of N-linked glycoproteins: High _____ or complex.

Answer 6

High MANNOSE

Question 7

____-cell disease (inclusion bodies) aka mucolipidosis type 2 is characterized by a course facies and lack of the mannose-6-phosphate signal for proteins destined for Lysosomes.

Answer 7

I-cell disease (I for inclusion bodies.)

Question 8

Proteoglycans usually consist of a core protein covalently linked to a __________, which typically consists of a long polysaccharide with a repeating ______ motif and contain anionic character.

Answer 8

Covalently linked to a GLUCOSAMINOGLYCAN, which typically consists of a long polysaccharide with a repeating DISACCHARIDE motif.

Question 9

MPS II aka Hunter Syndrome, exhibits a ______-linked recessive mode of inheritance. All the other MPS diseases are autosomal recessive. Hunter Syndrome is caused by a deficiency in L-______ sulfatase.

Answer 9

X-Linked recessive. It is caused by a deficiency in L-IDURONATE SULFATASE

Question 10

MPS I (Hurler Syndrome) is caused by a deficiency in L-______, leading to a buildup of _____ sulfate and _____ sulfate.

Answer 10

deficiency in L-iduronidase, leading to a buildup of DERMATAN sulfate and HEPARAN sulfate

Question 11

MPS III (Sanfillipo syndrome) have normal physical appearance but severe ______ retardation.

Answer 11

Severe mental RETARDATION

Question 12

MPS IV (Moroquio Syndrome) shows normal ______ but severe physical deformity. It is caused by a deficiency in either galactose-6-sulfatase or beta-______, which leads to an accumulations of _____ sulfate.

Answer 12

Normal INTELLIGENCE. Caused by a deficiency in either galactose-6-sulfatase or beta-GALACTOSIDASE, which leads to an accumulation of KERATAN sulfate.