IV - Lipids Flashcards
(260 cards)
1
Q
Lipids are hydro___, are soluble in _____ solvents and are compartmentalized to protect themselves for the _____ environment of cells.
A
hydrophobic, non-polar, watery cytoplasm
2
Q
Functions of Lipids
A
major source of energy, provide hydrophobic barries, serve as coenzymes/regulators, hormones, mediators of inflammation
3
Q
Phospholipids are _____ which enables formation of _____.
A
amphipathic, bilayers
4
Q
Long chains of carboxylic acids
A
Fatty Acids
5
Q
Fatty acids without double bonds
A
saturated
6
Q
Fatty acids with one double bond
A
monounsaturated
7
Q
Fatty acids with two or more double bonds
A
polyunsaturated
8
Q
Fatty acids that increase risk for cardiovascular diseases
A
trans- and saturated FAs
9
Q
Fatty acids that are protective against cardiovascular diseases
A
mono- and polyunsaturated FAs
10
Q
Essential Fatty Acids
A
Linoleic Acid (Omega 6), Linolenic Acid (Omega 3)
11
Q
Geometric isomer with the carbon moieties on the SAME side of the double bond
A
Cis fatty acid
12
Q
Geometric isomer with the carbon moieties on the OPPOSITE side of the double bond
A
Trans fatty acid
13
Q
Fluidity decreases with
A
increasing chain length (more C atoms, increasing saturation (less double bonds)
14
Q
Becomes essential if linoleic acid is deficient
A
Arachidonic Acid
15
Q
Decrease risk for cardiovascular disease by lowering thromboxane production reducing the tendency of platelts to aggregate
A
Omega Fatty Acids
16
Q
FA Activation
A
FA + CoA + ATP → Fatty acyl-CoA + AMP + PPi
17
Q
FA Activation: Enzyme
A
Fatty acyl-CoA synthetase
18
Q
FA Activation: Cofactor
A
Panthotenic Acid - B5
19
Q
FA Activation: Energy Use
A
2 ATP equivalents
20
Q
Formation of palmitate (16:0)
A
Lipogenesis, FA Synthesis
21
Q
Lipogenesis: Location
A
Cytosol, major: liver and lactating mammary glands, minor: adipose
22
Q
Lipogenesis: Substrates
A
1 acetyl CoA, 7 malonyl CoA, 14 NADPH, ATP
23
Q
Lipogenesis: Product
A
Palmitate
24
Q
Lipogenesis: Rate-Limiting Step
A
acetyl CoA + ATP → malonyl CoA
25
Lipogenesis: Rate-Limiting Enzyme
Acetyl CoA carboxylase (ACC)
26
Acetyl CoA carboxylase (ACC) requires
Biotin
27
Important Steps in Lipogenesis
synthesis of cytoplasmic acetyl CoA, carboxylation of acetyl CoA to malonyl CoA, assembly of palmitate
28
Lipogenesis: Step 1
synthesis of cytoplasmic acetyl CoA - transfer of acetyl CoA from the mitochondria to the cytoplasm through a CITRATE shuttle in the well-fed state
29
Lipogenesis: Step 2
acetyl CoA + ATP → malonyl CoA (acetyl CoA carboxylase) - rate-limiting step
30
Acetyl CoA carboxylase cofactor
Biotin
31
Acetyl CoA carboxylase activators
insulin, citrate
32
Lipogenesis: Step 3
assembly of palmitate (fatty acid synthase)
33
A multienzyme complex that has an acyl carrier protein (ACP) with panthoenic acid as a cofactor
fatty acid synthase
34
Fatty acid synthase cofactor
Pantothenic Acid (B5)
35
Synthesizes palmitate from 1 acetyl CoA + 7 malonyl CoA, uses NADPH as a reducing agent
fatty acid synthase
36
Sequence of Palmitate Assembly
condensation → reduction → dehydration → reduction
37
Lipogenesis: Activators
citrate (allosteric), insulin (by dephosphorylation and induction of enzyme synthesis)
38
Lipogenesis: Inhibitors
fatty acyl-CoA (allosteric), glucagon and epinephrine (by phosphorylation and repression of enzyme synthesis)
39
FAs further elongate in _____ and _____.
SER, mitochondria
40
Lipogenesis is limited to
16C Palmitate
41
SER and mitochondria form 22C and 24C FAs for
Sphingolipids
42
FAs are desaturated in the ___ through mixed function oxidases (cytochrome _).
SER, cytochrome bs
43
The body can generate double bonds on FA's but not beyond
Carbon 9
44
Short/Medium-chain FAs are bound to _____ until they are taken up by cells.
Albumin
45
Long-chain FAs (>12C) are transported in the bloodstream through _____.
Lipoproteins
46
FAs are converted to _____ before being stored as triacyglycerols
fatty acyl-CoA (active form)
47
Ester of trihydric glycerol and fatty acids, main storage form of FA, slightly soluble in water
Triacylglycerol
48
Coalesce within adipocytes to form oily droplets that are the major energy reserves of the body
Triacylglycerol
49
Triacylglycerol Synthesis
glycerol 3-P + 3FA → triglyceride
50
Triacylglycerol Synthesis: Location
liver, adipose
51
Source of glycerol 3-P in the liver & adipose
DHAP from glycolysis (glycerol 3-P dehydrogenase)
52
Source of glycerol 3-P in the liver only
phosphorylation of free glycerol (glycerol kinase)
53
Mobilization of Stored Fats
triglyceride → glycerol 3-P + 3FA (hormone-sensitive lipase + monoacylglycerol lipase)
54
Hydrolyzes TAGs yielding 2-monoacylglycerol + 2FA
Hormone-Sensitive Lipase
55
Hormone-Sensitive Lipase activators
glucagon, epinephrine, cortisol
56
Hormone-Sensitive Lipase inhibitor
insulin
57
Hormone-Sensitive Lipase can only release fatty acids from ___ and ___ of the TAG stored in fat
Carbon 1, Carbon 3
58
2-Monoacylglycerol → FA + Glycerol
Monoacylglycerol lipase
59
Removal of acetyl CoA fragments from the ends of FAs
Lipolysis, β-Oxidation of FAs
60
Lipolysis: Location
Mitochondria of all cells except neurons, RBCs, testis, kidney medulla
61
FA Activation: Location
Cytosol
62
Lipolysis: Substrates
palmitate, 7 NAD, 7 FAD, ATP
63
Lipolysis: Products
8 acetyl CoA, 7 FADH2, 7 NADH
64
Lipolysis: Rate-Limiting Step
fatty acyl CoA + carnitine → fatty acyl carnitine + CoA
65
Lipolysis: Rate-Limiting Enzyme
Carnitine acyltransferase I (carnitine palmitoyl transferase I)
66
Long-chain FAs have _ carbons and must be shuttles through the inner mitochondrial membrane via _____
> 12C, carnitine shuttle
67
Short-chain FAs have ___ carbons
2-4C
68
Medium-chain FAS have ___ carbons
6-12
69
fatty acyl carnitine + CoA → fatty acyl CoA + carnitine
Carnitine acyltransferase II - inside mitochondrial matrix
70
Catalyzed by carnitine acyltransferase II
fatty acyl carnitine + CoA → fatty acyl CoA + carnitine
71
Sequence of Lipolysis
oxidation → hydration → oxidation → thiolysis
72
FAs with an odd number of carbons will yield
acetyl CoA and propionyl CoA
73
Propionyl CoA is converted to _____.
succinyl CoA - a TCA Cycle intermediate
74
Catalyzed by propionyl CoA carboxylase
propionyl CoA + CO2 + ATP → methylmalonyl CoA + ADP + Pi
75
propionyl CoA + CO2 + ATP → methylmalonyl CoA + ADP + Pi
propionyl CoA carboxylase
76
Propionyl CoA carboxylase requires
Biotin
77
Catalyzed by methylmalonyl CoA mutase
methylmalonyl CoA → succinylt CoA
78
methylmalonyl CoA → succinylt CoA
methylmalonyl CoA mutase
79
Methylmalonyl CoA mutase requires
Vitamin B12
80
Oxidizes very long chain FAs (20C, 22C)
Peroxisomes
81
Oxidizes unsaturated FAs
3,2-enoyl CoA isomerase
82
Lipolysis yields __ ATP
129 ATP
83
Carnitine Palmitoyl Transferase I inhibitor
malonyl CoA
84
Indirectly inhibits lipolysis by activation acetyl CoA carboxylase and increasing malonyl CoA in the cytoplasm
Insulin
85
Alcohol leady to fat accumulation in the liver called _____ which ultimately leads to _____.
steatosis (fatty liver), cirrhosis
86
_____ eats up NAD to decrease _____ in the liver.
Alcohol dehydrogenase, lipolysis
87
Can occur in the newborn and manifest as hypoglcemia from impaired FA oxidation and muscle weakness from lipid accumulation
Carnitine Deficiency
88
Affects only the liver resulting in reduces FA oxidation and ketogenesis with hypoglycemia
Carnitine Palmitoyl Transferase I Deficiency
89
Affects skeletal muscle and, when severe, the liver
Carnitine Palmitoyl Transferase II Deficiency
90
Decreased FA oxidation, profound hypoglycemia during fasting due to lack of ATP for gluconeogenesis, can cause Sudden Infant Death Syndrome (SIDS)
Medium-Chain Fatty Acyl-CoA Dehydrogenase (MCAD) Deficiency
91
Medium-Chain Fatty Acyl-CoA Dehydrogenase (MCAD) Deficiency treatment and prevention
treatment: IV glucose, prevention: frequent feeding, high carbohydrate and low fat diet
92
Caused by eating unripe fruit of the akee tree which contains hypoglycin, a toxin that inactivates MCAD and SCAD leading to hypoglycemia
Jamaican Vomiting Sickness
93
Rare neurologic disorder due to a defect that causes accumulation of phytanic acid from plants which blocks lipolysis, causes neurologic symptoms due to improper myelinization
Refsum's Disease
94
Cerebrohepatorenal syndrome from the absence of peroxisomes, liver dysfunction with jaundice, mental retardation, weakness, hypotonia, craniofacial dysmorphism
Zellweger's Syndrome
95
Defect in the peroxisomal activation of VLCFA leads to accumulation of VLCFA leading to apathy, behavioral change, visual loss, spasticity, ataxia and death
X-linked Adrenoleukodystrophy
96
Converts acetyl CoA to ketone bodies
Ketogenesis
97
Ketogenesis: Location
liver mitochondria
98
Ketogenesis: Substrate
Acetyl CoA
99
Ketogenesis: Products
Ketone Bodies (polar): Acetoacetate & β-hydroxybutyrate (can be used as fuel), Acetone
100
Ketogenesis: Rate-Limiting Step
acetoacetyl CoA + acetyl CoA → HMG CoA
101
Ketogenesis: Rate-Limiting Enzyme
HMG CoA Synthase
102
Ketolysis
β-hydroxybutyrate → Acetoacetate → Acetyl CoA
103
β-hydroxybutyrate → Acetoacetate → Acetyl CoA
Ketolysis
104
Ketone bodies can serve as fuel for _____ tissues especially during fasting.
extrahepatic
105
Periheral tissues with mitochondria that can oxidize ketone bodies
skeletal muscle, renal cortex, brain (fasting > 2 weeks)
106
The liver cannot convert acetoacetate to acetyl CoA since it lacks
succinyl CoA acetoacetyl CoA transferase (thiophorase)
107
In prolonged starvation and _____, oxaloacetate is depleted for gluconeogenesis.
diabetic ketoacidosis
108
In alcoholism, excess NADH shunts oxaloacetate to _____.
malate
109
Seen in uncontrolled DM, starvation and chronic alcoholics
Ketoacidosis
110
Dehydration, CNS depression, coma, potassium depletion, metabolic acidosis, sweet/fruity odor of breath
Ketoacidosis
111
A very hydrophobic steroid alcohol with 27 carbons
Cholesterol
112
Cholesterol is a precursor/raw material for
cell membranes, vitamin D, adrenal hormones, sex hormones, bile salts
113
Cholesterol has _ fused hydrocarbon rings with an _-membered branched hydrocarbon chain attached to the _ ring.
4 fused hydrocarbon rings, 8-membered branched hydrocarbon chain, D ring
114
Cholesterol has a single ___ group located at the carbon _ of the _ ring to which FA can be attached to form cholesterol esters.
single hydroxyl group, carbon 3, A ring
115
Cholesterol Synthesis: Location
SER and cytosol, all cells (mainly in the liver and intestines)
116
Cholesterol Synthesis: Substrates
acetyl CoA, NADPH, ATP
117
Cholesterol Synthesis: Products
Lanosterol → Cholesterol
118
Cholesterol Synthesis: Rate-Limiting Step
HMG CoA → Mevalonate
119
Cholesterol Synthesis: Rate-Limiting Enzyme
HMG CoA Reductase
120
Drugs used for the treatment of hypercholesterolemia to reduce the risk for cardiovascular disease, competitive inhibitors of HMG CoA reductase
Statins
121
Important Steps in Cholesterol Synthesis
biosynthesis of mevalonate, formation of isoprenoid units (isopentenyl diphosphate), 6 isoprenoid units form isoprene, formation of lanosterol, formation of cholesterol
122
Cholesterol synthesis intermediate used for synthesis of coenzyme Q for the ETC, synthesis of dolichol pyrophosphate (cofactor in N-linked glycosylation of proteins in the RER), prenylation of proteins that need to be held in the cell membrane by a lipid tail
Farnesyl pyrophosphate
123
HMG CoA reductase inhibitors
glucagon, cortisol, epinephrine, phosphorylation, high cholesterol (limits the transcription factor SREBP - Sterol Regulatory Element-Binding Protein)
124
Cholesterol rings _____ be metabolized in humans.
cannot
125
Cholesterol is eliminated though _____ the secreted into the _____.
bile salts, bile
126
_____ can reduce cholesterol to _____ and _____.
Intestinal bacteria, coprostanol, cholestanol,
127
Synthesized in the liver from cholesterol
bile acids
128
Bile Acid Synthesis: Rate-Limiting Step
cholesterol → cholic acid
129
Bile Acid Synthesis: Rate-Limiting Enzyme
cholesterol-7-α-hydroxylase
130
Cholesterol-7-α-hydroxylase activator
cholesterol
131
Cholesterol-7-α-hydroxylase inhibitor
bile acids
132
Primary Bile Acids
cholic acid, chenodeoxycholic acid
133
Bile acids conjugated with either glycine or taurine
bile salts
134
Emulsify lipids in the intestines, provide the only significant mechanism for cholesterol excretion, both as a metabolic product of cholesterol and as a solubilizer of cholesterol in bile
bile salts
135
Secondary Bile Acids
deoxycholic acid, lithocholic acid
136
95% of excreted bile is reabsorbed in the _____ through the _____.
terminal ileum, enterohepatic circulation (5% excreted in feces = amount liver must make)
137
Mineralocorticoids
Zona Glomerulosa
138
Glucocorticoids
Zona Fasciculata
139
Sex Hormones
Zona Reticularis
140
Zona Glomerulosa
Mineralocorticoids
141
Zona Fasciculata
Glucocorticoids
142
Zona Reticularis
Sex Hormones
143
Steroid Hormone Synthesis: Location
SER of adrenal cortex, ovaries, testes, placenta
144
Steroid Hormone Synthesis: Substrates
cholesterol, pregnenolone (mother enzyme)
145
Steroid Hormone Synthesis: Rate-Limiting Step
cholesterol → pregnenolone
146
Steroid Hormone Synthesis: Rate-Limiting Enzyme
desmolase
147
Desmolase inhibitor
aminogluthetimide
148
Lingual lipase and gastric lipase are both ___ labile hence they are destroyed in the _____. This is important in _____ who do not have very acidic stomachs.
acid labile, stomach, neonates
149
In the gut, lipid components become enclosed in _____ then absorbed into _____.
micelles, enterocytes
150
Mixed micelles contain the products of lipid digestion by _____ and _____.
lipase, cholesteryl esterase
151
Secreted to lymphatics, reach the capillaries of skeletal muscle and adipose where TGs are broken into FA and glycerol via lipoprotein lipase
Chylomicrons
152
Directly enter adjacent muscle cells or adipocytes or may be transported in blood bound to albumin
Free Fatty Acids
153
Converted to DHAP then enters glycolysis or gluconeogenesis
Glycerol
154
Pancreatic Lipase: Substrate
TAG from diet
155
Pancreatic Lipase: Product
2-monoacylglycerol (MAG)
156
Pancreatic Lipase: Activator
trypsin
157
Lipoprotin Lipase: Substrate
TAG from chylomicrons and VLDL
158
Lipoprotin Lipase: Product
free glycerol
159
Lipoprotin Lipase: Activator
insulin
160
Hormone-Sensitive Lipase: Substrate
TAG from adipose
161
Hormone-Sensitive Lipase: Product
2-monoacylglycerol (MAG)
162
Hormone-Sensitive Lipase: Activator
glucagon
163
Manifests as steatorrhea (greasy stools), deficiency in fat-soluble vitamins and essential fatty acids
Lipid Malabsorption
164
Lipid Malabsorption causes
liver disease (not enough bile), pancreatic disease (enzyme deficiencies), cholelithiasis (bile obstruction), shortened bowel (decreased absorption time), intestinal mucosal defects
165
Spherical macromolecular complexes composed of neutral lipid core surrounded by a shell of amphipathic apolipoproteins, phospholiid and nonesterified cholesterol
Plasma Lipoproteins
166
Keep their component lipids soluble as they transport them in plasma, provides an efficient mechanism for transporting their lipid contents to and from the tissues
Plasma Lipoproteins
167
Plasma Lipoproteins: Highest in Protein
HDL (50%)
168
Plasma Lipoproteins: Highest in TG
Chylomicrons (88%)
169
Plasma Lipoproteins: Highest in Cholesterol
LDL (10%)
170
Plasma Lipoproteins: Highest in Cholesterol Esters
LDL (48%)
171
HIghest component of VLDL
TG (56%)
172
HIghest component of IDL
Cholesterol Esters (34%)
173
Transports dietary TG and cholesterol from the intestines to the tissues
Chylomicrons
174
Chylomicron Apoproteins
Apo B-48 (secreted by epithellial cells), Apo C-11 (activates lipoprotein lipase), Apo E (uptake by the liver)
175
Transports triglycerides from the liver to the tissues
VLDL
176
VLDL Apoproteins
Apo B-100 (secreted by the liver), Apo C-11 (activates lipoprotein lipase), Apo E (uptake by the liver)
177
Picks up cholesterol from HDL to become LDL
IDL
178
IDL Apoprotein
Apo E (uptake by the liver)
179
Delivers cholesterol into cells
Apo B-100 (uptake by the liver and other tissues via LDL receptor)
180
Picks up cholesterol accumulation in blood vessels (reverse cholesterol transport)
HDL
181
Delivers cholesterol to liver and steroidogenic tissues via scavenger receptor (SR-B1)
HDL
182
Shuttles Apo C-11 and Apo E in blood
HDL
183
HDL Apoprotein
Apo A-1 (activates lecithin cholesterol acyltransferase or LCAT to produce cholesterol esters)
184
Activates LCAT
Apo A-1
185
Binds to LDL and VLDL receptors
Apo B-100
186
Cofactor for lipoprotein lipase, shuttled by HDLs
Apo C-11
187
Chylomicron assembly and secretion
Apo B-48
188
Mediates uptake of chylomicron remnant
Apo E
189
Deposition of cholesterol and cholesteryl esters in the artery walls especially oxidized LDL, more sever in DM, lipid nephrosis and hypothyroidism
Atherosclerosis
190
Oxidized LDLs can cause endothelial damage which predisposes to _____.
atherosclerosis
191
Type I Familial Dyslipidemia
Hyperchylomicronemia
192
Type II Familial Dyslipidemia
Hypercholesterolemia
193
Type III Familial Dyslipidemia
Dysbetalipoproteinemia
194
Type IV Familial Dyslipidemia
Hypertriglyceridemia
195
Hyperchylomicronemia: Deficiency
Lipoprotein Lipase: high chylomicrons and VLDL, low LDL and HDL
196
Xanthomas and pancreatitis without increased risk of coronary heart disease
Hyperchylomicronemia
197
Hypercholesterolemia: Deficiency
LDL Receptors: high LDL
198
Xanthomas and xanthelasmas with increased risk of atherosclerosis and coronary heart disease
Hypercholesterolemia
199
Dysbetalipoproteinemia: Deficiency
Apo E: high remnants of chylomicrons and VLDL
200
Increased risk of atherosclerosis and coronary heart disease without xanthomas
Dysbetalipoproteinemia
201
Hypertriglyceridemia: Pathogenesis
increased VLDL productin
202
Triad: coronary artery disease, T2DM, obesity
Hypertriglyceridemia
203
Apo B-48 and Apo B-100 deficiency
Abetalipoproteinemia: no chylomicrons/VLDL/LDL
204
Intestinal malabsorption with accumulation of lipids in the intestine and liver
Abetalipoproteinemia
205
Apo A-1 deficiency
Familial α-Lipoprotein Deficiency (Tangier's/Fisheye Disease): no HDL
206
High triglycerides, premature atherosclerosis, neuropathy, enlarged yellow-orange tonsils
Familial α-Lipoprotein Deficiency (Tangier's/Fisheye Disease)
207
Increased HDL production
Familial Hyper-α-Lipoproteinemia: associated with benefits to health and longevity
208
High Lipoprotein A
Familial Lipoprotein A Excess
209
Early atherosclerosis and thrombosis
Familial Lipoprotein A Excess
210
Phospholipids are _____ compounds composed of _____, _____ and _____.
amphipathic, alcohol, diacylglycerol or sphingosine backbone, phosphodiester bond
211
Predominant lipids of cell membranes, degraded by phospholipases
Phospholipids
212
Most abundant phispholipids
Phosphatidylcholine
213
Large proportion of body's store of choline, important in nervous transmission (acetylcholine), store of labile methyl groups
Phosphatidylcholine
214
Plays a role in apoptosis, also found in cell membranes
Phosphatidylethanolamine (cephalin) and Phosphatidylserine
215
Major component of lung surfactant, deficiency leads to RDS
Dipalmitoylphosphatidylcholine (DPPC) or Dipalmitoyllecithin
216
Reservoir for arachidonic acid in membranes, source of 2nd messengers
Phosphatidylinositol
217
2 molecules of phosphatidic acid esterified through their phosphate groups to an additional molecule of glycerol
Cardiolipin
218
Found only in mitochondria and is essential for mitochondrial function
Cardiolipin
219
Deficiency or defect can cause mitochondrial dysfunction in aging and in heart failure, hypothyroidism and Barth syndrome (cardioskeletal myopathy)
Cardiolipin
220
_____ is antigenic. It reacts with antibodies produced against Treponema pallidum (syphilis). One of the non-treponemal tests for syphilis is an anti-_____ test.
Cardiolipin
221
Part of the glycocalyx located on the outer layer of the cell membrane and functions in cell recognition and adhesion, found in high concentrations in nervous tissue
Glycolipids
222
Ceramide
Sphingosine + Fatty Acid
223
Cerebroside
Ceramide + Glucose or Galactose
224
Globoside
Ceramide + Oligosaccharide
225
Ganglioside
Ceramide + N-acetylneuramic Acid
226
Sulfatides
Ceramide + Sulfatide Galactose
227
Sphingosine + Fatty Acid
Ceramide
228
Ceramide + Glucose or Galactose
Cerebroside
229
Ceramide + Oligosaccharide
Globoside
230
Ceramide + N-acetylneuramic Acid
Ganglioside
231
Ceramide + Sulfatide Galactose
Sulfatides
232
The only significant sphingophospholipid in humans, an important constituent of the myelin sheath in nerves
Sphingomyelin
233
Deficiency in phospholipids and sphingolipids from white matter resulting in increased CSF phospholipids
Demyelinating Diseases
234
Lipid storage diseases often manifested in childhood, lipid synthesis is normal, lipid degradation in lysosomes is abnormal
Sphingolipidoses
235
Complex lipids accumulate in cells, neurodegeneration, enzyme deficiency is similar in all tissues
Sphingolipidoses
236
Tay-Sach's Disease: Deficiency
Hexosaminidase A
237
Tay-Sach's Disease: Accumulating Lipid
Ganglioside
238
Tay-Sach's Disease: Findings
cherry red macula, mental retardation, hypotonia
239
Hexosaminidase A Deficiency
Tay-Sach's Disease
240
Fabry's Disease: Deficiency
α-Galactosidase
241
Fabry's Disease: Accumulating Lipid
Globotriaosylceramide
242
Fabry's Disease: Findings
3 Rs: Recessive (X-linked), Rash, Renal failure
243
α-Galactosidase Deficiency
Fabry's Disease
244
Gaucher's Disease: Deficiency
β-Glucosidase
245
Gaucher's Disease: Accumulating Lipid
Glucosylceramide
246
Gaucher's Disease: Findings
hepatosplenomegaly, erosion of long bones, Gaucher cells: accumulation of fibrils in macrophage, crumpled tissue paper
247
β-Glucosidase Deficiency
Gaucher's Disease
248
Niemann-Pick Disease: Deficiency
Sphingomyelinase
249
Niemann-Pick Disease: Accumulating Lipid
Sphingomyelin
250
Niemann-Pick Disease: Findings
hepatosplenomegaly, foam cells: small vacuoles, lipid-laden macrophages, sea-blue histiocytes
251
Potents compunds that elicit a wide range of physiologic and pathologic responses
Eicosanoids
252
3 Main Kinds of Eicosanoids
prostaglandin, thromboxane, leukotriene
253
Eicosanoids: Dietary Precursor
Linoleic Acid
254
Eicosanoids: Immediate Precursor
Arachidonic Acid
255
Released from membrane lipids by phospholipase A2
Eicosanoids
256
Synthesized by platelets, causes vasoconstriction and platelet aggregation
Thromboxane (TXA2)
257
Produced by blood vessel walls, inhibitors of platelet aggregation
Prostacyclin (PGI2)
258
mixture of leukotrienes C4, D4 and E4, potent bronchoconstrictors
Slow-Reacting Substances of Anaphylaxis (SRS-A)
259
Slow-Reacting Substances of Anaphylaxis (SRS-A)
Leukotrienes C4, D4 and E4
260
All sphingolipidoses are _____ recessive except _____.
autosomal recessive (sphingolipidoses), Fabry's Disease (X-linked recessive)
