Lecture 37 Flashcards Preview

Medical Biochem and Genetics > Lecture 37 > Flashcards

Flashcards in Lecture 37 Deck (12)
Loading flashcards...
1
Q

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.

A

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

2
Q

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

A

POST-TRANSLATIONAL

3
Q

_____ is a proteoglycan well known for inhibiting blood clotting.

A

HEPARIN

4
Q

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

A

SERINE or THREONINE

5
Q

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

A

ASPARIGINE

6
Q

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

A

High MANNOSE

7
Q

____-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.

A

I-cell disease (I for inclusion bodies.)

8
Q

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.

A

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

9
Q

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.

A

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

10
Q

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

A

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

11
Q

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

A

Severe mental RETARDATION

12
Q

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.

A

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