Block 6 Nishimoto Flashcards

1
Q

ZP3 Glycoprotein

A

Sperm Receptor

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2
Q

Xylose-Galactose-Galactose

A

Link Trisaccharide

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3
Q

X and Y AA’s in collagen

A

Often proline or hydroxyproline

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4
Q

With what does type IX collagen associate?

A

Type 2 collagen

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5
Q

Why do Proteoglycans absorb water?

A

Negative charges attract Na+ and then water

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6
Q

Why are some nondisjunction errors more common?

A

Many errors are not observed due to lack of viability

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7
Q

Which linked oligosaccharide starts as pre-assembled scaffold?

A

N-Linked, then modified

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8
Q

Which linked oligosaccharide is addedone sugar at a time

A

O-Linked

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9
Q

Where does Glycosylation of N-Linked oligo’s occur?

A

Lumen of rER

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10
Q

Where does Glycosylation of N-Linked Oligo’s occur?

A

Lumen of rER

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11
Q

Where are all rRNA genes located

A

Stalks of Acrocentric chromosomes

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12
Q

When is reduction division step?

A

Meiosis I

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13
Q

When is Mosaicism heritable?

A

When involving the germline

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14
Q

When is greatest risk of nondisjunction seen?

A

Maternal Meiosis I and is associated with advanced maternal age

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15
Q

When does Recombination occur?

A

Prophase I

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16
Q

When does oocyte resume meiosis after after birth?

A

At time of ovulation

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17
Q

When does Glycosylation of N-Linked Oligo’s occur?

A

As polypeptide is being synthesized

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18
Q

When does Glycosylation of N-Linked Oligo’s occur?

A

As polypeptide is being synthesized

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19
Q

When does error occur if 47,XXY?

A

Meiosis II in Paternal Gamete

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20
Q

When does collage cross-linking occur?

A

After globular domains have been removed

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21
Q

When do homologous chromosomes separate?

A

Meiosis I

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22
Q

When can nondisjunction occur?

A

In any cell division

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23
Q

What structural feature determines chromosome number?

A

Centromere

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24
Q

What stabilizes elastic networks

A

Desmosine: Crosslinks derived from aldehydes of lysine

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25
Q

What resembles mitotic division?

A

Meiosis II

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26
Q

What provides collagen the strength necessary for mechanical strength to withstand body stresses

A

Cross-linking of Polypeptides

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27
Q

What must happen before collages can self-assemble into fibers

A

Globular domains must be removed

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28
Q

What is located in stalks of Acrocentric chromosomes

A

All rRNA genes

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29
Q

What is in core of Collagen triple helix

A

Glycine R-group hydrogen

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30
Q

What is Glycosylation

A

the process of biosynthetically sugar coating proteins

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31
Q

What is Glycosylation

A

The process of biosynthetically sugar-coating proteins

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32
Q

What is glycosylated in collagen

A

Hydroxylysine

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33
Q

What is a Haplotype?

A

Linear order of closely linked alleles; Generally inherited as a unit

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34
Q

What helps recognize a protein is collagen?

A

Hydroxyproline and Hydroxylysine

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35
Q

What has a special O-glycosylation in collagen

A

Hydroxylysine

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36
Q

What forms clot

A

Fibrin

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37
Q

What does Fibronectin associate with intracellularly?

A

Actin

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38
Q

What does Fibrinogen do after cleavage?

A

Aggregates

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39
Q

What does FGF bind to for storage?

A

GAG PG’s

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40
Q

What does Desmosine require to form?

A

Lysyl Oxidase

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41
Q

What do GAG’s take a large volume relative to their mass

A

They are highly hydrated gels

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42
Q

What decreases half-life of Ceruloplasmin

A

Unbinding of Sialic Acid

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43
Q

What connects ECM collagen w/ integrin?

A

Fibronectin

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44
Q

What causes collagen to melt in scurvy

A

Low Hydroxyproline

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45
Q

What carries preassembled oligosaccharide

A

Dolichol Phosphate

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46
Q

What can be measured in urine to estimate collagen turnover

A

Hydroxyproline

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47
Q

What are O-Linked Oligosaccharides attached to?

A

Serine, Threonine, Hydroxylysine

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48
Q

What activates platelets to change surface receptors and shape?

A

Thrombin

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49
Q

What activates Fibrinogen and Protein C

A

Thrombin

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50
Q

What activates Factor VII

A

TF exposure on non-vascular cell

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51
Q

Wafarin

A

Inhibits synthesis of many blood coag factors

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52
Q

Vitamin K

A

Cofactor for synthesis of Prothrombin, Factor X, etc.; Target of Warfarin

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53
Q

Venter’s Method of dealing with repeats

A

Paired-end sequences from clones of known size

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54
Q

Type of oligosaccharide attached to Ser, Thr, or Hydroxylysine

A

O-Linked

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55
Q

Type of oligosaccharide attached to Asparagine

A

N-Linked

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56
Q

Type of Collagen that forms sievelike network

A

4

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57
Q

TM Glycoproteins that mediate cell-matrix binding

A

Integrins

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58
Q

TM Glycoprotein receptor for TGF-beta

A

Beta-Glycan

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59
Q

The process of biosynthetically sugar-coating proteins

A

Glycosylation

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60
Q

T/F: The preassemble oligosacchardie is always the same

A

TRUE

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61
Q

T/F: Only 1 type of GAG chain can be found on a Proteoglycan

A

False, more than 1 type can be found, but each GAG chain is a pure repeating disaccharide

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62
Q

T/F: N-Linked Oligosaccharides are not modified after addition to polypeptide

A

FALSE

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63
Q

T/F: Glycoproteins are necessary for fertilization

A

TRUE

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64
Q

T/F: GAG chains can be composed of multiple repeating disaccharides

A

False, although more than 1 type of GAG chain can be found on a proteoglycan, each GAG chain is a pure repeating disaccharide

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65
Q

T/F: Fibronectin does not have distinct domains?

A

FALSE

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66
Q

T/F: All N-Linked Oligo’s started out with same pre-assembled Dolichol Phosphate Oligo

A

True, then modifications occur in Golgi

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67
Q

T/F: After mitotic nondisjunction, there are two abornmal clones

A

False, one dies; Cells with 21+ continue

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68
Q

Submetacentric

A

P arm is much shorter than q arm

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69
Q

Structure of Integrin

A

2 TM polypeptides

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70
Q

Structure of Fibronectin

A

A dimer of 2 similar subunits joined by disulfide bonds at the C-terminal

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71
Q

Steps of Collagen Synthesis

A

(1) Synthesis of pro-alpha chain; (2) Hydroxylation of select PRO and LYS; (3) Glycosylation of select Hydroxylysines; (4) Self-assembly of 3 chains; (5) Procollagen triple-helix formation; (6) Secretion; (7) Cleavage of peptides

72
Q

Sperm Capacitation

A

Altered membrane glycoprotein and gal transferase recognizes egg

73
Q

Serine O-glycosylation recognition seq.

A

Asp/Glu-X-Ser-Gly

74
Q

Sequence to which N-Linked Oligo’s are added?

A

ASN-X-Ser/Thr

75
Q

Sequence of Fibronectin recognized by integrins

A

RGD

76
Q

Selectins

A

Glycoproteins that recognize sugars that allow WBC to adhere to endothelium

77
Q

Role of Basal Lamina in kidney

A

Solute barrier

78
Q

Result of Meiosis II Nondisjunction

A

2 daughter cells normal, 1 with 2 copies, 1 with none

79
Q

Result of Meiosis I Nondisjunction

A

2 daughter cells with 2 copies, 2 with none

80
Q

Recombination hotspots divide the genome into:

A

Haplotype Blocks: Islands of reduced meiotic recombination

81
Q

Receptor on non-vascular cells

A

Factor VII

82
Q

Proteoglycans/Glycosaminoglycans have ____ sugars

A

O-Linked

83
Q

Proteoglycans vs Avg Glycoprotein

A

Proteoglycans have higher carb content

84
Q

Proteoglycans tend to have ____ carb content than avg. glycoprotein

A

Higher

85
Q

Proteoglycans

A

Glycoprotein with Glycosaminoglycan chains

86
Q

Proteoglycans

A

Glycoproteins with Glycosaminoglycan chains

87
Q

Problems in collagen cleavage

A

weaker interactions, packing problem

88
Q

Polyanions w/ many negative charges that are hydrated with water

A

Proteoglycans

89
Q

Percent of genome that is repeats?

A

78%

90
Q

Oligosaccharides with sugars attached to oxygen of Ser, Thr, or Hydroxylysine

A

O-Linked Oligosaccharides

91
Q

Oligosaccharides with sugars attached to Asparagine

A

N-Linked Oligosaccharides

92
Q

Oligosaccharide side chains are hydrophilic or phobic

A

Hydrophilic

93
Q

O-Linked Glycosylation

A

One sugar at a time, with specific glycosyl transferases using sugar nucleotides

94
Q

New mutations in males vs females?

A

Increase in males as age; No age effect for females

95
Q

Network Collagens

A

IV and VIII

96
Q

Mucopolysaccharidosis

A

Defects in integral membrane PG recycling; GAGs in urine from accumulation

97
Q

Most secreted proteins are what?

A

Glycoproteins

98
Q

Most extracellular proteins are glycosylated, except

A

Albumin, abudant serum transporter

99
Q

Most extracellular proteins are _____, except _____

A

Glycosylated, except Albumin

100
Q

Metacentric

A

Centromere is close to middle

101
Q

Mannose is a ______ of glucose

A

2’ epimer

102
Q

Locations of Type 2 Collagen

A

Cartilage, Vitreous of Eye

103
Q

Locations of Type 1 Collagen

A

Bone, Skin, Tendon, Cornea, etc

104
Q

Location of Type 4 Collagen

A

Basal lamina sheets

105
Q

Linkage Disequilibrium

A

Co-occurrence of alleles on the same chromosome more often than by chance; Over-representation of specific haplotypes

106
Q

Link Trisaccharide

A

Xylose-Galactose-Galactose

107
Q

Linear order of closely linked alleles; Generally inherited as a unit

A

Haplotype

108
Q

Last step in adding GAG to core protein

A

Adding negatively charged sulfate group

109
Q

Is Hyaluronate sulfated?

A

No

110
Q

Integrins

A

TM Glycoproteins that mediate cell-matrix binding

111
Q

Inhibitin a Selectin

A

Can inhibit extravasation; Protect tissue from leuckocyte induced damage

112
Q

Inheritance of most OI

A

Autosomal Dominant

113
Q

Indy Assortment

A

Unlinked loci are transmitted independently

114
Q

Increased incidence of rare autosomal dominant disorders is associated with:

A

Advanced paternal age

115
Q

Include most extracellular matrix proteins, coagulation proteins, fibronectin, laminin, aggrecan

A

Glycoproteins

116
Q

I-Cell Disease

A

Lysosomal proteins don’t receive P-Mannose “ticket” and are secreted

117
Q

Hydrophilicity of Elastin

A

Hydrophobic, oppositie of proteoglycans

118
Q

Hyaluronate

A

Free GAG; Not sulfated; Found in cartilage, synovial fluid, vitreous humor

119
Q

How to Proteoglycans often interact with proteins of the matrix

A

GAG chains with X-X-B-B-X-B-X (Arg or Lys); gives them a postively charged region

120
Q

How many crossover events per homologous pair?

A

2-Jan

121
Q

How does Glycosylation of N-Linked Oligo’s occur?

A

A preassembed oligo is transferred to an appropriate Asn in the sequence ASN-X-Ser/Thr

122
Q

How does Glycosylation of N-Linked Oligo’s occur?

A

A preassembled oligo is transferred to an appropriate Asn

123
Q

How does a GAG interact with Collagen?

A

B-B-X-B sequence

124
Q

How do oligosaccharides affect protease activity?

A

Mask protease-sensitive sites

125
Q

How do Chondroitin, Dermatan, and Heparan Sulfate GAG’s attached to proteins

A

Covalently attached thru a Serine and a link trisaccharide

126
Q

How are GAG chains linked to Core Protein

A

O-Glycosidically-Linked to Serine

127
Q

How are clots digested?

A

Plasmin, a protease

128
Q

High energy sulfate donor

A

PAPs

129
Q

Glycosylation occuring one sugar at a time

A

O-Linked Glycosylation

130
Q

Glycoproteins with Glycosaminoglycan chains

A

Proteoglycans

131
Q

Glycoproteins that recognize sugars that allow WBC to adhere to endothelium

A

Selectins

132
Q

Glycoproteins targeted to lysosomes are modified with ______

A

P-Mannose as part of sugars on N-Linked Oligosaccharides

133
Q

Glycoproteins on Zona Pellucida

A

Act as barrier or a sperm receptor

134
Q

Glycoprotein with Glycosaminoglycan chains

A

Proteoglycans

135
Q

Glucose ring derivitized at the 2 position

A

N-Acetyl-Glucosamine (GlcNac)

136
Q

Functions of Glycoproteins

A

Recognition, Solubility, Protection

137
Q

Function of Fibronectin

A

Binds both cells and ECM macromolecules

138
Q

Free GAG example

A

Hyaluronate

139
Q

First 3 sugars added in O-linked glycosylation

A

Xylose-Galactose-Galactose-(Glucaronic acid)

140
Q

First 3 steps at injured site

A

VII –> X –> Thrombin

141
Q

Fibronectin

A

Dimer joined by disulfide bond; 3 forms from 1 gene; Multifunctional

142
Q

Fibril Associated Collagen

A

IX

143
Q

Effect of Oligosaccharides on solubility

A

Hydrophilic, so make proteins soluble

144
Q

Effect of Oligosaccharides on protease activity

A

Can mask protease-sensitive sites, prevent degradation

145
Q

Dolichol phosphate carrier

A

Holds pre-assembled N-Linked Oligosaccharide before transfer to polypeptide

146
Q

Dolichol Phosphate

A

Carrier of preassembled oligo in Glycosylation of N-Linked Oligo’s

147
Q

Desmosine

A

Cyclic crosslinks derived from 4 hydroxylysines; brings 4 elastin monomers brought together

148
Q

Defects in integral membrane PG recycling

A

Mucopolysaccharidosis

149
Q

Cysteine for Glycine sub in collagen

A

OI

150
Q

Copper-binding and transport

A

Ceruloplasmin

151
Q

Collagen that forms scaffold for basal lamina

A

4

152
Q

Co-occurrence of alleles on the same chromosome more often than by chance; Over-representation of specific haplotypes

A

Linkage Disequilibrium

153
Q

Class that includes most ECM proteins, coagulation proteins, fibronectin, laminin, aggrecan

A

Glycoproteins

154
Q

Chance of recombination b/t 2 loci is function of what?

A

Distance (Closer = Less chance of recombination)

155
Q

Ceruloplasmin w/out Sialic Acid

A

Binds membrane receptor and endocytosed; Decreases half-life

156
Q

Ceruloplasmin bound to Sialic Acid

A

Doesn’t bind membrane receptor; Increase half-life dramatically

157
Q

Ceruloplasmin

A

Copper-binding and transport

158
Q

Carrier of preassembled oligo in Glycosylation of N-Linked Oligo’s

A

Dolichol Phosphate

159
Q

Carb-binding proteins for cell attachment, immune response, lung surfactant, and sperm-egg binding

A

Lectins

160
Q

Carb content of Proteoglycans

A

Higher than average Glycoprotein

161
Q

Binding of ____ can serve to store growth factors

A

FGF

162
Q

Beta-Glycan

A

TM Glycoprotein receptor for TGF-beta

163
Q

Asp/Glu-X-Ser-Gly

A

Serine O-glycosylation recognition seq.

164
Q

Are Oligo side chains hydrophilic or phobic?

A

Philic: Increase solubility

165
Q

Are CS, DS, and HS GAG’s sulfated?

A

Yes

166
Q

Alternating components of GAGs

A

Amino Sugars and Acid Sugars

167
Q

Altered membrane glycoprotein and gal transferase recognizes egg

A

Sperm Capacitation

168
Q

Almost all glycoproteins are ____

A

extracellular

169
Q

Alleles within a Haplotype block tend to be in:

A

Linkage Disequilibrium

170
Q

Aggrecan vs Decorin

A

Decorin is very small/short

171
Q

Aggrecan

A

Large Proteoglycan that can aggregate w/ link proteins and Hyaluronate to form very large aggregates

172
Q

Acrocentric

A

Stalks and Satellites compose p arm; All rRNA genes are located in stalks

173
Q

Abundant Proline and Hydroxyproline in collage do what

A

Force a collagen helix coiled structure

174
Q

A protein with covalently attached carb

A

Glycoprotein

175
Q

3 Functions of Glycoproteins

A

Solubility, Protection, Recognition

176
Q

3 Components of Proteoglycan aggregates

A

Proteoglycan Monomers, Link Protein, Hyaluronate

177
Q

“Fibrin Stapler”

A

Factor XIIIa