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MERP BIOCHEM > Lipid Metabolism > Flashcards

Lipid Metabolism Flashcards

1
Q

Tay Sachs disease

A

Lack of HEXA which breakdown gangliosides

Cherry red spot in macula (eye)

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2
Q

Structural lipids includes

A

Phospholipids and Glycolipids

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3
Q

Phospholipids include, and their structure

A

Glycerol phospholipids/Phosphoglyceride

Glycerol backbone with two FA via ester linkage and choline/ethanolamine via phosphodiester linkage

I.e. Plasmalogens = cardiomyocytes plasma membrane. Protection against radicals

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4
Q

Sphingolipid structure

A

Sphingosine backbone with FA via amide linkage

I.e. Sphingomyelin - neuron myelin sheath

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5
Q

Glycolipids include

A

Glycosphingolipids and Gangliosides

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6
Q

Glycosphingolipid

A

Sphingosine backbone with FA.

Involved in nerve impulses

Simple - cerebroside
Complex - 2+ goboside

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7
Q

Ganglioside

A

Nerve tissue

ABO blood group
Oligosaccharide head attached to Salic acid residues

Stem cell markers

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8
Q

Saturated fats

A

Unbranched, single bonds, solid at room temp

I.e. Palmitic acid

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9
Q

Monosaturated Fats

A

1 double bond
Waxy.

I.e. Palmitoleic acid.
Oliec

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10
Q

Polyunsaturated fats (PUFAs)

A

Two or more double bonds

Cis = healthy

I.e. Arachidonic acid
Linolenic

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11
Q

Triaglycerides include:

A

Glucagon (mobilize) and insulin (store) –> lipoprotein lipase releases TG for storage

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12
Q

Chylomicrons

A

Largest and least dense

Synthesized in ER of small intestine

Core lipid: dietary TG, cholesterol esters

B48, C,E

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13
Q

ApoC

A

A lipoprotein lipase enzyme converts TG to free FA and glycerol which is stored and oxidized in the target tissues

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14
Q

ApoE

A

Binds liver receptors involved in Chylomicron uptake to be degraded

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15
Q

B48

A

Acquires ApoC and ApoE from HDL while moving through blood and lymph system

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16
Q

VLDL

A

High in diet fat, cholesterol and converted into TG and packaged in muscle and adipose tissue for storage and usage

Core lipids - endogenous TG

B100, ApoC, ApoE

IDL is a remnant and converted to LDL

17
Q

LDL

A

Synthesized via VLDL

Core lipids- endogenous cholesterol, esters

ApoB-100

Cholesterol converted to extra hepatic tissue

18
Q

HDL

A

Synthesized in liver and nascent intestine

Core lipid - endogenous

ApoA, ApoC, ApoE = nascent HDL

Collects cholesterol via all transfer proteins towards liver to be excreted.

19
Q

Equation to determine cholesterol levels = plaque

A

LDL = VLDL - IDL

20
Q

Abetalipoproteinemia

A

Deficiency in ApoB48 and ApoB-100

Low LDL, VLDL, CHylomicrons = no absorption or transport

Symptoms: fatty stool, early death

21
Q

Familial defective ApoB-100

A

Mutant in ApoB-100

LDL receptor binding problem resulting in high cholesterol LDL levels

Autosomal dominant

22
Q

ApoA

A

Good

High HDL component

23
Q

ApoB

A

Bad

High chylomicrons, VLDLs and LDLs

MI risk assessment using ApoB/ApoA ratio

24
Q

Zellwegers syndrome

A

No peroxisomes

Facial features, liver, CNS

25
Q

Infantile Refsum Disease

A

High serum copper and iron, acidic serum and CSF

Hypomyelination

26
Q

ELISA function

A

To measure Ag-Ab quantification of hormones/enzymes/NTs that are present in blood/tissues/fluids,etc.

The target protein is the Ag, then lab made Ab binds

Direct, Indirect, and Sandwich (most sensitive)

27
Q

Neimann Pick Disease

A

Rare autosomal recessive

Acid sphingomyelinase enzyme = accumulation of sphingomyelin in brain, spleen, liver

Mental retardation

Type A: neurological, early infancy
Type B: non-neurological, no mental retardation, preteen

28
Q

HMG-CoA Reductase

A

Rate limiting step of cholesterol biosynthesis, forming Mevalonate from HMG-CoA.

Activated by insulin which dephosphorylates HMG via protein phosphatase

Inhibited by glucagon/Epi which activates protein kinase which phosphorylates HMG-CoA Reductase

29
Q

Systemic primary carnitine def.

A

No carnitine transporter

Low plasma carnitine
High urine carnitine

Muscle weakness, hypoketotic hypoglycemia

30
Q

CPT-1 def

A

Long chain FA cannot go into mito

High plasma carnitine

Early childhood

31
Q

CPT-II def

A

Adult form- not fatal, muscle damage

Severe infantile form - sudden death risk

Fatal neonate form - highly fatal

32
Q

Intermediates of the biosynthesis of cholesterol

A
  1. Isopentenyl-pyrophosphate
  2. Geranyl-pyrophosphate
  3. Farnesyl-pyrophosphate —> prenylated proteins such as: Rho/Ras, Dolichol, ubiquinone/Coenzyme Q, Heme a
33
Q

SREBP - sterol regulatory element binding protein

A

Promotes transcription of HMG CoA reductase forming endogenous cholesterol biosynthesis

During low Intracellular cholesterol

MERP BIOCHEM (6 decks)

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