Block 4 Nishimoto Questions Flashcards
(282 cards)
1
Q
Zellweger Syndrome
A
Lack of functional peroxisomes; Can’t Oxidize VLCFA’s
2
Q
Which steps of Mitochondrial Long-Chain FA Metabolism Occurs in Cytosol?
A
Fatty acyl CoA Synthetase Activates FA to FattyAcylCoA
3
Q
Which KB packs “extra punch”
A
Beta-hydroxybutyrate (NADH)
4
Q
Where is Fatty Acyl CoA Oxidized in Liver?
A
Mitochondrial Matrix
5
Q
Where does Succinyl CoA get its CoA for Ketone Oxidation
A
CAC Cycle or Oxidation of Odd-Chain or Branched-Chain FA’s
6
Q
Where does Omega-oxidation occur?
A
ER, forms Dicarboxylic Acids
7
Q
Where does maturation of Chylomicrons occur?
A
Blood, pick up ApoE and ApoCII from HDL
8
Q
Where does final oxidation of Fatty Acyl CoA occur?
A
Mitochondrial Matrix
9
Q
Where are OH Groups of Cholesterol in a Lipoprotein
A
Surface
10
Q
When is MC AcylCoA Dehydrogenase deficiency noticed?
A
When glucose levels are low and FA’s cannot be completely oxidized
11
Q
When is Gluconeogenesis main source of blood glucose
A
After ~16 hours
12
Q
When does muscle use blood glucose
A
High insulin (fed state) or during exercise
13
Q
When does muscle primarly use Glycogen?
A
Severe exersize
14
Q
When does brain start using KB’s?
A
Roughly day 3 (Starved State); KB levels in blood aren’t high enough until this day.
15
Q
When do you need extra ATP in Palmitate synthesis?
A
When starting with just Acetyl CoA (no Malonyl CoA)
16
Q
When are Malonyl CoA levels elevated?
A
Fed State; It’s the substrate for FA Synthesis produced by Acetyl CoA Carboxylase
17
Q
What size FA’s use Carnitine Cycle for Membrane Transport
A
Long Chain (12-20)
18
Q
What size FA’s are catabolized in peroxisome?
A
Very Long Chain (>20)
19
Q
What provides the omega-methyl group of palmitate in FA synthesis?
A
Acetyl CoA
20
Q
What provides the 2 carbon units that are added to the growing Fatty Acyl chain?
A
Malonyl CoA
21
Q
What products of lipid digestion do Intestinal epithelial cells absorb?
A
FA’s and 2-MG’s
22
Q
What percent of Bile Salts are excreted?
A
5
23
Q
What part of intestinal cell produces TG?
A
sER
24
Q
What part of intestinal cell produces apoB-48
A
rER
25
What part of FA Synthase reacts with Malonyl CoA
Sulfhydryl group of Phosphopantetheinyl residue
26
What is required to transfer lipid to apoB-48?
MTP
27
What is required for FA chain to start?
An AcCoA is required to be acceptor of Malonyl Transfer; Must make MalonylCoA from AcCoA
28
What is produced converting Beta-Hydroxybutyrate to Acetoacetate
NADH + H+
29
What is necessary to convert Acetoacetate to Beta-hydroxybutyrate
NADH + H+
30
What is Acetyl CoA from Long-Chain FA Metabolism used for in Liver
KB Synthesis (TCA Cycle in other tissues)
31
What is a key factor in control of Ketone Synthesis
Availability of FA's
32
What happens to remnants of Chylomicron breakdown?
Taken up by liver by receptor-mediated endocytosis; Digested by Lysosomes; Products released into Cytosol
33
What happens to HMG CoA in Ketone Synthesis
Converted to Acetoacetate, producing one AcetylCoA
34
What happens to FADH2's produced in VLCFA oxidation?
Transfer electrons to O2; Produce H2O2
35
What happens to FA's once absorbed into intestinal epithelium?
Esterfied to the 2-MG's to form TG
36
What happens to Citrate once pumped into Cytosol?
Makes OAA and AcCoA
37
What happens to Bile Salts no longer needed?
Absorbed in Ileum, Returned to Liver via Entero-hepatic Circulation
38
What happens to Acetoacyl CoA in Ketone Oxidation?
Cleaved to 2 Acetyl CoA by Thiolase
39
What happens to 2-MG's once absorbed into intestinal epithelium?
Esterfied to FA's to form TG's
40
What FA's under Alpha-Oxidation?
Branched-Chain FA's
41
What enzyme activates Fatty Acids
Fatty Acyl CoA Synthetase
42
What electron carriers are produced during Beta-oxidation?
NADH, FADH2
43
What donates a CoA to Acetoacetate in Ketone Oxidation?
Succinyl CoA
44
What does the Final Step in Oxidation of Odd Chain FA's produce
Propionyl CoA (3 Carbons)
45
What does the conversion of Proprionyl CoA to Succinyl CoA require?
ATP, Biotin, Vitamin B12
46
What does Pancreatic Lipase require to work?
Protein Colipase to stabilize and localize lipase near bile salt-TG particle
47
What does Pancreatic Lipase Colipase need to work?
Bile salts to emulsify TG's
48
What does omega-oxidation of FA's produce?
Dicarboxylic Acids -> May be excreted or conjugated to Glycine or Carnitine
49
What does muscle use during resting state or mild exersize?
KBs or FA's (FA's preferred)
50
What does Desaturase require
Molecular Oxygen
51
What does Beta-oxidation of Fatty Acyl CoA generate in mitochondria
NADH, FAD(2H), Acetyl CoA
52
What do Peroxisomes not produce from Beta-Oxidation?
FAD(2H)
53
What bond is cleaved in Beta-oxidation?
alpha-beta bond
54
What activates LPL on capillary walls in adipose and muscle tissue?
ApoCII
55
Uses for FA's from Adipose during Basal Fasted State
Liver for ATP and KB's (which go to muscle, not brain yet); Muscle for AcCoA/TCA
56
Types of FA's oxidized in Peroxisomes
VLCFA, Some LCFA's; Branched-Chain >8
57
Two products of Glycerol Metabolism in Liver
DHP, Glyceraldehyde-3-P
58
Two Main Ketone Fuels
Beta-hydroxybutyrate, Acetoacetate
59
To what part of FA Synthase is Phosphopantheine linked?
Serine residue of Acyl Carrier Protein segment
60
To what is Alanine from muscle converted?
Pyruvate
61
T/F: the Liver can use KB's as fuel if is has to
False, doesn't have correct enzymes
62
T/F: One isozyme of Acyl CoA Dehydrogenase can oxidize all FA lengths?
False, several different isozymes
63
Surface of Lipoproteins
Phospholipids and Proteins on surface, with hydrophilic regions interacting w/ water
64
Subcellular location of FA Activation
Cytosol (or Mitochondria???) (Fatty Acyl CoA Synthetase)
65
Subcellular Location of Elongase
Endoplasmic Reticulum
66
Subcellular Location of Desaturase
ER
67
Subcellular Location of CPT2
Inner Mitochondrial Membrane
68
Subcellular Location of CPT1
Outer Mitochondrial Membrane
69
Steps Unique to VLCFA Oxidation
Oxidized in peroxisomes by beta-oxidation to produce medium/short-chain; Converted to Carnitine dervatives and sent to mitochondria
70
Source of Pyruvate in FA Synthesis
Glycolysis (Glucose)
71
Source of Glycerol for Lipogenesis in Fed State?
Glycolysis
72
Source of Citrate in FA Synthesis
Mitchondria: OAA and AcCoA from Pyruvate; AcCoA + OAA = Citrate
73
Site of Catabolism and Membrane Transport of Very Long Chain FA's
Peroxisome; Unknown
74
Site of Catabolism and Membrane Transport of Short Chain (2-4) FA's
Mitochondrion; Free Diffusion
75
Site of Catabolism and Membrane Transport of Medium Chain (4-12) FA'a
Mitochondrion; Diffusion
76
Site of Catabolism and Membrane Transport of Long Chain (12-20) FA's
Mitochondrion; Carnitine Cycle
77
Role of Fatty Acyl Carnitine
Carnatine carries FA into mitochondria (Enzymes required)
78
Role of CPT1 in Ketone Synthesis Regulation
Converts FA's to FA-Carnitine (which then is converted to FA-CoA)
79
Role of Citrate in FA Synthesis
Provides AcCoA and NADPH for FA Synthesis
80
Regulation of VLCFA Oxidation
Just VLCFA availability
81
Regulation of Alpha-Oxidation of Branched-Chain FA's
None
82
Regulated step of FA Synthesis
Acetyl CoA converted to Malonyl CoA by AcCoA Carboxylase (Needs CO2, ATP, Biotin)
83
Reducing agent used by Desaturase
NADH
84
Reducing agent in FA Synthesis vs Beta-Oxidation
NADPH vs NADH, FADH2
85
Recycling of OAA back to mitochondria makes what?
NADPH
86
Pyruvate Carboxylase
Pyruvate to OAA
87
Purpose of Thiolysis in Beta-Oxidation
Cleavage step, produces 1 AcetylCoA and FACoA that is 2 carbons shorter
88
Purpose of Protein Colipase
Binds to lipase and emulsified fat increasing lipase action
89
Products of initial beta-oxidation of VLCFA in peroxisomes?
AcetylCoA (Acetyl-Carnitine); SCFA CoA/MCFA CoA (SCFA-carnitine/MCFA-Carnitine)
90
Products of Beta-Oxidation of Even-Chain FA
Acetyl CoA; FADH2, NADH
91
Production of Dicarboxylic Acids
Omega-Oxidation of FA's; May be excreted or conjugated to Glycine or Carnitine
92
Processing of Propionyl CoA produced from Odd Chain FA Oxidation
Add carbon and form Succinyl CoA
93
Primary Tissues for FA Synthesis vs Beta-Oxidation
Liver, Fat vs Liver, Muscle
94
Prevention of newly synthesized FA's from oxidation
Malonyl CoA (high during FA synthesis) inhibits CPT1, which tranports LCFAs into mitochondria
95
Preferred substrate of muscle during prolonged exercise
Glucose
96
Preferred substrate of Cardiac Muscle 2 hours after meal
FA
97
Positive Regulators of Ketogenesis
(1) High FA's; (2) High CPTI; (3) Low Malonyl CoA; (4) High ATP; (5) High NADH; (6) Low OAA
98
Positive regulation of FA Synthesis
Citrate, Insulin
99
Positive regulation of Acetyl CoA Carboxylase
Citrate, Insulin
100
Net AcetylCoA's used to make 1 Acetoacetate
1 (2 initially, but one produced)
101
Negative Regulators of Ketogenesis
(1) Low FA's; (2) Low CPTI; (3) High Malonyl CoA; (4) Low ATP; (5) Low NADH; (6) High OAA
102
Negative regulation of Acetyl CoA Carboxylase
Palmitoyl CoA, Low ATP (High AMP)
103
Negative regulation of Acetyl CoA Carboxylase
Palmitoyl CoA, Low ATP (High AMP)
104
Metabolic Changes when reach Starved State
Brain uses KB's, Muscle doesn't; Mucles stops breaking down Protein (less Urea in Urine)
105
Maturation of Chylomicrons
Occurs in blood; Pick up ApoE and ApoCII from HDL
106
Malonyl CoA
Inhibits CPT1; When FA's being synthesized, blocks FA oxidation; Intermediate in FA Synthesis
107
Malic Enzyme
Malate to Pyruvate; Producing CO2 and NADPH
108
Malate Dehydrogenase
OAA to Malate, using NADH
109
Major target of Glucagon and effect on cAMP
Liver, Increases cAMP; (Activates Lipase in Adipose?)
110
Major Component of a Chylomicron
TG
111
Major apolipoprotein in Chylormicrons
apoB-48
112
Location of HMG CoA Synthase
Liver Mitochondria
113
Lipolytic Hormones
Glucagon (fasting, starvation); Epi/NE (stress); ACTH
114
KB levels during prolonged fasting
Rise substantially thru day 20-30
115
Intracellular Lipolysis Cascade after Glucagon binds
Adenylate Cyclase -> cAMP -> PK -> TAG Lipase -> TAG to DAG to FFA's -> FFAs released
116
Insulin stimulates the secretion of what from Adipose cells?
LPL (Lipoprotein Lipase)
117
Initial Oxidation of Branched-Chain FA's
Alpha-oxidation in Peroxisomes to produce CO2, Propionyl and Acetyl CoA; MCFA's sent to mitochondria as carnitine derivatives
118
Inhibitor of CPT1
Malonyl CoA
119
In what part of intestinal cell does assembly of lipoproteins occur?
ER and Golgi
120
In what organ does the synthesis of FA from Glucose mainly occur?
Liver
121
In what form are TG's ultimately absorbed in small intestine?
FA's and 2-MG's
122
In what form are Hepatic TAG's exported from Liver
VLDL's
123
In Palmitate Synth., what extra do you need if starting from just AcetylCoA
7 ATP to add CO2
124
How many FADH/NADH molecules are produced for each cycle of Beta-oxidation?
1 (=4 ATP)
125
How many carbons added with each repeat of FA Synthesis
2 Carbons
126
How many ATPs per NADH in FA Oxidation?
2.5
127
How many ATPs per FADH2 in FA Oxidation?
1.5
128
How many ATPS from 1 Palmitate via Beta-Oxidation?
7 Cycles; 7 NADH and 7 FADH2 = 28 ATP; 8 AcCoA = 80; Minus 2 ATP for activation; Total=106 ATP
129
How many ATP are required to Activate Beta-oxidation
2
130
How many Acetyl CoA's does Palmitoyl CoA produce?
8, 7 repetitions of the Beta-oxidation spiral
131
How many Acetyl CoA's are needed to make 1 HMG CoA
2
132
How is Acetoacetate activated in Ketone Oxidation?
Accepts a CoA group from Succinyl CoA
133
How does Muscle get energy during basal fasted state?
KB's from liver (which come from FA's from adipose); FA's directly from Adipose
134
How does low calorie diet affect Acetyl CoA Carboxylase
Decreases rate of transcription -> Inhibit FA Synthesis
135
How does Liver get ATP during Basal Fasted State?
FA's from Adipose
136
How does Insulin Ratio induce TG degradation in Adipose?
(1) High Glucagon/Insulin elevates cAMP; (2) PKA Activated; (3) Hormone-Sensitive Lipase Phosphorylated; (4) TG broken down into FA and Glycerol
137
How does increased insulin stop TG degradation in Adipose tissue?
Stimulates the phosphatase that inactivates HSL in fed state
138
How does high calorie diet affect Acetyl CoA Carboxylase
Increases rate of transcription -> Promotes FA Synthesis
139
How does Brain get ATP during Basal Fasted State?
Glucose from Gluconeogenesis/Glycogenolysis
140
How do high FA levels affect CPTI
Malonyl CoA inhibition of CPTI is lifted by inactivation of AcCoA Carboxylase
141
How do Glucose levels change during Fasting
Drop Initially, then remain relatively constant indefinitely
142
How do FFA levels change during fasting
Rise initially, then remain relatively constant indefinitely
143
How do fat cells make Glycerol-3-Phosphate
From Glucose by Glycolysis; They lake Glycerol Kinase
144
How do activated Acetyl groups exit mitochondria in FA Synthesis?
As Citrate
145
How do activate Acyl groups enter mito in Beta-Oxidation
As Carnitine Derivative
146
How are the Acetyl CoA's produced in (terminal step of) Ketone Oxidation
Acetoacetyl CoA is cleaved by Thiolase
147
How are dietary FA's transported in blood?
Chylomicrons
148
How are Beta-Oxidation and CAC similar?
Hydration, production of FADH2 and NADH
149
Goal of FA Synthesis
Store excess glucose as FA's
150
From what is Acetone spontaneously formed?
Acetoacetate, giving off CO2
151
From what does Chylomicron get ApoE and ApoCII
HDL
152
From what are Bile Salts derived?
Cholesterol (maintain their ring structure)
153
For what does the liver recycle Glycerol?
Gluconeogenesis and TG Synthesis
154
First Three Steps of Ketone Synthesis (Just substrates)
(1) 2 AcetylCoA --> (2) Acetoacetyl CoA --> (3) HMG CoA
155
First Step of Ketone Oxidation when starting with Beta-Hydroxybutyrate?
Oxidation to Acetoacetate
156
First Committed Step of FA Synthesis
Acetyl CoA converted to Malonyl CoA by AcCoA Carboxylase (Needs CO2, ATP, Biotin)
157
Fate of Proteins in Muscle during Basal Fasted State
Broken down into AA's, which are used for Gluconeogenesis
158
Fate of FAD(2H) and NADH produced in beta-oxidation of FA's
Re-oxidized by ETC, producing ATP
159
Extra requirements of Beta-Oxidation of Unsaturated FA's
2 Extra Enzymes (Isomerase and Reductase) to modify double bonds
160
Epinephrine
Stimulates Glycogenolysis in Skeletal Muscle/Liver; Stimulates Lipolysis
161
Energy resevoir of Cardiac Muscle
Glycogen (few reserves)
162
End Products of Lipolysis
Acetyl CoA, Glycerol
163
Effect of Palmitoyl CoA on Acetyl CoA Carboxylase
Decreases rate of transcription -> Slows FA Synthesis
164
Effect of Malonyl CoA on CPT1
Malonyl CoA inhibits CPTI, decreasing KB synthesis
165
Effect of Low OAA levels of Ketogenesis
Diverts AcCoA into Ketogenesis from TCA; Rate of Citrate Synthase reaction reduced
166
Effect of Low ATP/High AMP on Acetyl CoA Carboxylase
Activates PK that phosphorylates (deactivates) -> Inhibit FA Synthesis
167
Effect of Insulin on Beta-Oxidation
Increase Malonyl CoA, Decrease Beta-Oxidation
168
Effect of Insulin on Acetyl CoA Carboxylase
Stimulates dephosphorylation (activation) -> Promotes FA Synthesis
169
Effect of High NADH/FADH2 on Beta-Oxidation
Inhibits
170
Effect of High NADH on Ketogenesis
High NADH depletes OAA to help shunt AcCoA to make KB's b/c less OAA is available for Citrate
171
Effect of High NADH levels on Ketogensis
Converts OAA into Malate, which causes shunting of AcCoA into Ketogenesis instead of TCA Cycle
172
Effect of High ATP on Ketogenesis
(1) High ATP = Less NADH Oxidized; (2) NADH/NAD+ ratio increases; (3) OAA converted to Malate; (4) AcCoA diverted from TCA to Ketogenesis, b/c of low OAA levels
173
Effect of Glucagon on F-2,6-BisP
Decreases, Slows Glycolysis
174
Effect of Citrate on Acetyl CoA Carboxylase
Increases rate of transcription -> Promotes FA Synthesis
175
Effect of AMP-PK (muscle,liver) on Beta-Oxidation
Decrease Malonyl CoA, Increase conversion of Fatty Acyl CoA to Fatty Acyl Carnitine
176
During what state is Urea production highest
Fasting, then protein breakdown slows
177
Difference b/t Bile Salts and Cholesterol precursor
Rings in Bile Salts contain more Hydroxyl Groups, a polar side chain, Lack a 5-6 double bond
178
Diff b/t FA elongation vs synthesis?
Elongation occurs while FA is bound to CoA, rather than to ACP
179
Cortisol
Gluconeogenesis, AA Mobilization, Lipolysis
180
Components of MTP
PDI, a chaperone; and TG Transfer portion
181
Citrate Synthase
OAA to Citrate, Using Acetyl CoA
182
Citrate Shuttle
Citrate out of Mito, Pyruvate in (Shuttling Acetyl CoA out for FA Synthesis)
183
Citrate Lyase
Citrate to OAA using ATP, Producing Acetyl CoA for FA Synthesis
184
Cholesterol Esters in Lipoproteins
OH group esterfied to a FA; Found in interior of Lipoprotein
185
Carrier of FA Synthesis vs Beta-Oxidation
Protein (ACP) vs Coenzyme A
186
Carnitine Deficiency
Inability to transport LONGCHAIN FA's into mitochondria
187
Carnitine Cycle
Exchange of Carnitine and Acyl Carnitine; Long Chain FA's into mitochondria
188
Cardiac Fuels
Primarily FA's; Will use KB's over Glucose
189
cAMP and Lipolysis
Increased cAMP activates Lipase, increasing Lipolysis in Adipose tissue
190
Apo B in Liver vs Intestine
Same gene; RNA Editing; Instestinal version (B-48) is half the size
191
Antilipolytic Hormones
Insulin
192
Acyl CoA "Desaturases"
ER; Contain FAD, Cytochrome B5, and an Iron-Sulfure Center; Various specificities, <10
193
Activities affected by MTP loss
Chylomicron and VLDL assembly, both need an apoB protein to assemble
194
Actions of MTP
Required to transfer lipid to apoB-48, and to transfer lipid from cytoplasm to ER lumen; Helps recruit TG lipid droplets as particle grows
195
Actions of Lipolytic Hormones
Increase adipose cAMP and activate Lipase
196
Action of Pancreatic Lipase
Cleaves FA's from positions 1 and 3 of TG, producing 2 FA's and a 2-MG
197
Action of LPL
Breaks down TG's to FA's; FA's taken into Adipose Cells
198
Action of Gastric and Linguinal Lipases
Hydrolyze short/medium chain FA's from TG's
199
Action of Carnitine in Mitochondrial Long-Chain FA Metabolism
Transports activated Fatty Acyl CoA into mitochondria
200
Action of Beta-Hydroxybutyrate
Interconverts Acetoacetate and Beta-hydroxybutyrate
201
Action of ApoE
Binds to receptors (in liver) that uptake chylomicron remnants
202
Abetalipoproteinemia
Loss of TG Transfer Activity (MTP); Inability to take in and distribute dietary fats
203
5 Steps of FA Synthesis
(1) Transfer of Malonyl; (2) Condensation of malonyl; (3) Reduction of beta-ketoacyl group; (4) Dehydration forms double bond; (5) Reduction of double bond w/ NADHP
204
2-Carbon Donor of Elongation and Reducing Agent
Malonyl CoA, NADPH
205
2 Steps of FA Activation
(1) ATP added to form high-energy Fatty Acyl AMP; (2) AMP is exchanged for CoA (product is Fatty Acyl CoA
206
2 Sources of NADPH for FA Synthesis
Pentose Phosphate Pathway, Malic Enzyme (Malate to Pyruvate)
207
2 Components needed for TG Synthesis in Adipose
Glycerol-3-Phosphate from Glucose and FA's from VLDL's
208
1 Main difference b/t Ether Lipid and other Glycerolipids
Ether instead of Ester bond at C1
209
2 Mechanisms to Add Head Group to Phosphatidic Acid
(1) Activate Head group with CTP; (2) Activate DG w/ CTP
210
2 Starting components of Ceramide Synthesis
Palmitoyl CoA and Serine
211
2 Types of Ether Glycerolipids
Plasmologens and PAF
212
3 Components to make Phosphatidic Acid
Glycerol-3-P, 2 FA-CoA
213
3 Steps of TG Synthesis
(1) Glycerol-3-P from Glycerol or Glucose; (2) Glyc-3-P -> Phosphatidic Acid; (3) PT Acid -> DG -> TG
214
4 Types of Glycolipids
Cerebrosides, Sulfatides, Globosides, Gangliosides
215
ABO blood groups are determined by what type of lipids
Sphingolipids
216
Action of Pancreatic Lipase and Colipase
Hydrolyzes TG and DG, forming 2-MG and 2 FA's
217
Alpha-Galactosidase deficiency
Fabry Disease
218
Backbone of Sphingolipids and Glycolipids
Ceramide, not Glycerol
219
Beta-glucocerebrosidase deficiency
Gaucher Disease
220
Ceramide
Sphingosine linked to FA at 2 w/ amide
221
Ceramide backbone with carbohydrate group attached
Glycolipids
222
Common Intermediate of Membrame Phospholipid Synthesis
Phosphatidic Acid
223
Components of Cardiolipin
Phosphotidylglycerol and CDP-DAG
224
Components of Glycerophospholipids
Glyercol, 2 FA, "Head Group"
225
Degradation of Sphingolipids
Occurs in Lysosomes
226
Difference b/t Plasmalogen and PAF
At C1: Unsaturated vs Saturated; At C2: Acyl vs Acetyl; At C3: Head group Ethanolamine vs Choline
227
Diglyceride Acyltransferase is part of what complex?
TG Synthetase
228
Ether Glycerolipid vs Glycerophospholipid
Ether bond at C1 instead of ester bond to FA
229
Fabry Disease
Alpha-Galactosidase Deficiency; Accumulation of Globosides
230
From what is Arachidonic Acid derived?
Phospholipids
231
From what other phospholipid can you make phosphotidylserine
phosphotidylethanolamine (reversive exchange)
232
Functions of Glycolipids
Cell recognition, Protective effects, Binding site for some toxins
233
Ganglioside
Ceramide - Sugars - NANA
234
Gaucher Disease
Beta-glucocerebrosidase deficiency; Ceramide-glucose not broken down; Accumulate Glucocerebroside
235
General purpose of Sphingolipids
Help cell-cell, cell-matrix interactions
236
Globosides
Ceramides attached to 2 or more sugars
237
Glycerolneogenesis during Fasting
Adipose tissue can use Lactate, Pyruvate, & AA to regulate FA release; Gluc-3-P -> DHAP -> Glyercol-3-P
238
Glycolipid Structure
Ceramide backbone with Carbohydrate group attached
239
Head group of Phosphatidic Acid
H
240
Head group of Phosphatidylcholine
Choline
241
How is Glycerol-3-P produced in Adipose tissue normally?
Glucose -> DHAP -> Glycerol-3-P (using NADH)
242
How is Glycerol-3-P produced in Liver?
Glycerol Kinase phosphorylates; Glucose to DHAP to Glycerol-3-P (Using NADH)
243
How many high energy Phosphates are necessary to activate a FA?
2, ATP -> AMP
244
How many hydrocarbon tails does PC have?
2
245
Inability to degrade Glycolipids and Sphingomyelin in membranes
Mucolipidosis
246
Neutral Glycolipids
Contain one or more sugars
247
Phenotype w/ Glycerolneogenesis Pathway KO
Release abnormally high FA in blood in fasting and have low fat stores
248
Phosphotidylethanolamine to Phosphotidylcholine
Add three Methyl Groups (3 SAM's)
249
Plasmologen locations
Mitochondria, Myelin, Muscle
250
Polar head group of Ceramides
Don't have one
251
Polar head group of Glycolipids
Carboydrate
252
Product when Dephosphorylate Phosphatidic Acid
DAG
253
Requirements for Ceramide synthesis
Serine, PalmitoylCoA, FACoA, NADHP, FAD
254
Structure of Ether Lipid (Plasmolgen)
Ether bond at 1 to Long Chain Alcohol (Double bond b/t carbons 1 and 2); FA at 2; Polar Head Group at 3
255
Subcellular Location of TG Synthetase Complex
ER
256
Sulfatide
Ceramide attached to sulfated galactose
257
Synthesis of Sphingomyelin from Ceramide requires what?
Addition of Choline from Phosphatidylcholine
258
T/F: Distribution of complex lipids is PM is symmetric
FALSE
259
T/F: Glycolipids contain a phosphate
FALSE
260
Tay-Sachs Disease
Hexosaminidase A Deficiency; GM2 Ganglioside Accumulates
261
Tissue with Glycerol Kinase
Liver
262
Type of Complex Lipid with Carbohydrate
Glycolipid
263
Type of Plasmalogen in Heart Muscle
Choline Plasmologen
264
Type of Plasmalogen in Myelin
Ethanolamine
265
What 2 components make Sphingosine
Palmitoyl CoA and Serine
266
What activates sugars in Glycolipid Synthesis
UDP or CMP
267
What causes Mucolipidosis
Inability to degrade Glycolipids and Sphingomyelin in membranes
268
What cells secrete LPL and in response to what?
Adipose, Insulin
269
What determines type of Glycolipid
Nature of carbohydrate
270
What do Sphingophospholipids and Glycolipids share?
Sphingosine backbone the 2-FA links by an Amide
271
What does Inisitol react with to form PI?
CDP-DG
272
What does synthesis of Glycerolipids require
NADPH, AcylCoA, DHAP, and activated Ethanolamine
273
What drives the reaction of Phosphatidic Acid to Glycerophospholipids
CTP
274
What helps sulfate Glycolipids
PAPS
275
What is the 2-fatty acid replaced by in PAF
Acetyl Group
276
What links FA's in Sphingolipids/Glycolipids
Amide, not ester or ether
277
What membrane is enriched with Cardiolipin?
Inner Mito Membrane
278
Where are Glycolipids predominate
Neural Cells
279
Where does synthesis of Glycolipids occur?
ER and Golgi (sugars sequentially added as activated glycosyl residues)
280
Where is PAF made?
Peroxisomes
281
Where is sphingomyelin enriched?
CNS along w/ Glycolipids
282
Which Complex Lipids do not have Polar Head Groups
Glycolipids and TAG's
