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B2 Biochem > Metabolism Of Complex Lipids > Flashcards

Metabolism Of Complex Lipids Flashcards

1
Q

definition of Lipids

A

Diverse and ubiquitous group of organic compounds that are insoluble in water but soluble in organic solvents

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2
Q

Lipids are soluble in ______ but insoluble in ________

A

Organic Solvents, water

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3
Q

Examples of lipids

A

FAs, TAG, phospholipids, sterols, sphingolipids, terpenes

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4
Q

Classification of Lipids

A

GLYCEROL based and NONGLYCEROL based.

If glycerol based, then they are SIMPLE or COMPOUND

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5
Q

What are fats and oils classified as?

A

Simple glycerol based lipids

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6
Q

What are phospholipids and glycolipids classified as?

A

Complex glycerol based lipids

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7
Q

What are sphingolipids, glycophingolipids, steroids and waxes classified as?

A

Complex lipids. Or complex non-glycerol based lipids.

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8
Q

Structure of Phospholipids

A

Hydrophilic head, hydrophobic tail

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9
Q

hydrophilic head of phospholipids are composed of:

A

Phosphate group and glycerol backbone

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10
Q

Hydrophobic tails of phospholipids are composed of:

A

FA hydrocarbon tail

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11
Q

What’s the special characteristic of phospholipids? How are they arranged in water?

A

They’re amphipathic molecules.

Heads are out, tails are in, to form the lipid bilayer

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12
Q

Functions of Phospholipids

A

Structural component, reservoir for intercellular second messengers, anchor point for membrane proteins

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13
Q

Phospholipids function as a component of:

A

Lipoprotein, pulmonary surfactants, bile

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14
Q

precursor for the synthesis of all other glycerophospolipids and TAG

A

phosphatidic acid (PA)

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15
Q

phosphatidic acids’ relevance to membrane structure

A

Influences membrane curvature and vesicles formation

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16
Q

The most abundant phospholipid

A

Phosphatidylcholine (PC, lecithin)

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17
Q

PC

A

Phosphatidylcholine

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18
Q

PA

A

Phosphatidic acid

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19
Q

PC composition

A

PA + choline

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20
Q

PC is a major component of

A

Lung surfactant

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21
Q

Lung surfactant is a mixture of:

A

90% lipids and 10% proteins

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22
Q

Roles of Lung Surfactant

A

Decrease surface tension, reduce pressure for re-inflation of alveoli, and prevent alveolar collapse

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23
Q

What is atelactasis?

A

Alveolar collapse

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24
Q

Respiratory Distress Syndrome is due to:

A

Insufficient lung surfactant

Significant cause of neonatal deaths in Western Countries

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25
Phosphatidylethanolamine
PA + ethanolamine 2nd most abundant phospholipid
26
PE
Phosphatidylethanolamine
27
Phosphatidylserine
PS PA + serine
28
PS
Phophatidylserine
29
PS is only found where?
Inner leaflet of plasma membrane Required for membrane synthesis and plays a role in recognition of apoptotic cells
30
Phosphatidylinositols
PI PA+inositol
31
PI
Phosphatidylinositol
32
Why is PI an unusual lipid?
It contains a stearic acid at C1 and a arachidonic acid at C2
33
Functions of PIs
Reservoir of arachidonic acid Precursor for prostaglandins OH groups can be phosphorylated (producing PIP2, precursor for IP3 and DAG) Serve as anchor points for proteins
34
PI is a precursor for:
IP3 and DAG, after it becomes phoporylated to PIP2
35
Phosphatidylglycerol
PG PA + glycerol
36
PG
Phosphatidylglycerol
37
PG is a precursor for:
Surfactant And for synthesis of cardiolipin
38
2 PA molecules, exclusive to the innermitochondrial membrane, maintains the structure and function of ETC complexes
Cardiolipin
39
FA at c-1 is attached via ether-linkage (not ester)
Ether glycerophospholipids
40
2 types of ether glycerophospholipids, which are based on what?
Based on the FA at c-1 linkage, 1. Plasmalogens (unsaturated FA arc-1) 2. Platelet-activating factor (PAF)(saturated FA at c-1)
41
Type of ether glycerophospholipid with unsaturated FA at c-1
Plasmalogens
42
What type of Plasmalogens are in the heart muscle? Nerve tissue?
PhosphatidALcholine, heart PhosphatidALethanolamine, nerve
43
What is the PAF?
Type of ether glycerophospholipid with a saturated FA at c-1 and a short acetyl group at c-2 (instead of an acyl
44
What is one of the most potent bio active molecules (triggers thrombotic and inflammatory events)?
PAF Platelet-activating factor
45
What is PA synthesized from?
Glycerol-3-P or 2 fatty acyl CoA
46
once PA is produced, it can go through what 2 pathways?
CDP-DAG pathway to produce PI, PG, or Cardiolipin DAG to produce PC or PE
47
Ether lipids are produced in what molecules?
Peroxisomes
48
CDP-DAG pathways is used for:
PI, PG and Cardiolipin
49
Transfer of activated CDP-choline or CDP-ethanolamine to DAG is used for:
PC, PE synthesis
50
PS is synthesized from:
PE is the substrate Base-exchange reaction Reversible step
51
PS is decarboxylated to form:
PE
52
PC is produced where?
In the liver
53
PC is secreted from liver cells in:
The bile
54
PC are incorporated into:
Lipoproteins and are secreted
55
Pathway for production of PC
PS -> PE -> PC PS loses its' carboxyl group, then PE adds 3 methyl groups to become PC
56
Functions of Phospholipases (PLPs)
Hydrolysis the phospho digester bonds of glycerophospholipids Site specific cleavage (each PLP acts on a specific bond) Release bioactive molecules that can serve as second messengers (DAG, IP3) Participate in remodeling of phospholipids
57
Structure of sphingolipids
Sphingomyelin + ceramide
58
What is a ceramide?
FA + sphingosine
59
What is SM?
Sphingomyelin, ceramide + phosphocholine Predominant sphingophospholipid in mammalian cells Major structural sphingolipid in the plasma membrane Abundant in nerve tissue
60
What sphingolipid is a major structural sphingolipid in the plasma membrane and is abundant in nerve tissue?
Sphingomyelin (SM)
61
What is the main precursor for SM and all glycophingolipids?
Ceramide
62
What do ceramides do in the skin?
Maintain the skins' water-permeability barrier Decreased levels of ceramides are associated with skin diseases
63
What is a sphingosine composed of?
Palmitic acid + serine
64
Functions of GSLs in Human cells
Regulation of cellular interactions regulation of growth and development Antigenic (source of blood group antigens) in embryonic development, tumors, and toxins/viruses
65
What is a neutral GSL?
Cerebroside
66
What is a Cerebroside made of?
Ceramide and a sugar Ex: galactosylceramide
67
What do cerebrosides do?
Found in membranes Lipid rafts formation Predominant in nerve tissue
68
What GSLs are negatively charged at pH of 7?
Acidic GSLs
69
What is a ganglioside?
Type of acidic GSL Ceramide + oligosaccharide + NANA
70
What is a NANA?
N-acetylneuramic acid
71
Where are gangliosides found?
Ganglion cells in the CNS High amounts of these lipids are from lipid storage disorders
72
What is a sulfatide?
A type of Acidic GSL Galactocerebroside + sulfate group Found in brain and kidney
73
Sphingolipid synthesis
Begins in ER with condensation of palmitoyl CoA and L-serine Forms sphingoid backbone and produces ceramide via several steps Ceramide is transferred to Golgi, where is is used a substrate to synthesize Sphingomyelin and GSLs SM and GSLs are then transported/secreted into lipoproteins
74
Local (limited) degradation of SM
In plasma membrane to produce ceramides for cell signaling
75
Complete Degradation of SM and GSLs
Occurs in lysosomes by different acidic enzymes at pH 4.5
76
Sphingolipid synthesis and degradation are:
Balanced tightly at constant levels
77
Deficiency in an enzyme in the lysosomes results in:
Sphingolipidose, common name for a lysosomal lipid storage disease
78
Sphingolipidoses
If particular acid hydrolase is defective, SL substrate accumulates. Nervous tissues are affected because of high abundance of SLs. Autosomal Recessive May be fatal. Genetic variability. Incidence is low in most populations. More frequent in Ashkenazi Jewish population
79
Tay-Sachs disease (enzyme deficiency, Lipid accumulation, clinical symptoms)
Hexosaminidase A Gm2 Ganglioside Mental retardation, blindness, muscular weakness
80
Fabry's Disease (enzyme deficiency, Lipid accumulation, clinical symptoms)
alpha-Galactosidase Globotriasylceramide Skin rash, kidney failure (x-linked)
81
Metachromic leukodystrophy (enzyme deficiency, Lipid accumulation, clinical symptoms)
Arylsulfatase A 3-sulfogalactosylceramide Mental retardation, demyelination
82
Krabbe's disease (enzyme deficiency, Lipid accumulation, clinical symptoms)
Beta-galactosidase Galactosylceramide Mental retardation, myelin almost absent
83
Gaucher's disease (enzyme deficiency, Lipid accumulation, clinical symptoms)
Beta-glucosidase Glucosylceramide Enlarged liver and spleen, erosion of long bones, mental retardation in infants
84
Niemann-Pick disease (enzyme deficiency, Lipid accumulation, clinical symptoms)
Sphingomelinase Sphingomyelin Enlarged liver and spleen, mental retardation, fatal in early life
85
Farber's disease (enzyme deficiency, Lipid accumulation, clinical symptoms)
Ceramidase Ceramide Hoarseness, dermatitis, skeletal deformation, mental retardation, fatal in early life
86
What is the most common form of Gm2 gangliosidosis?
TaySachs disease
87
Sandy off disease is similar to:
TaySachs disease
88
Which Sphingolipidoses are related to Cherry Red macula?
TaySachs disease Sandhoff disease Niemann-Pick disease
89
Neman-Pick disease is the accumulation of:
Sphingomyelin Causing enlarged spleen and liver (hepatosplenomegaly) Cherry-red macula
90
Cherry-red spot in macula may indicate
Cherry Red Macula Taysachs, sandhoff or Niemann-pick Accumulation of Sphingomyelin (Enlarged spleen/liver) Accumulation of gangliosides (blindness)

B2 Biochem (13 decks)

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