TBL11 Flashcards

Metabolism of Fatty Acids and B Oxidation

1
Q

Fatty Acids

A
  • aliphatic
  • esters in natural fats and oils
  • free FAs in plasma
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2
Q

Nomenclature

Saturated acids

A

-anoic

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3
Q

Nomenclature

Unsaturated

A

-enoic

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4
Q

Eicosanoids: The 20-Carbon Powerhouses

A
  • Derived from eicosanoic (20-carbon) polyenoic fatty acids
  • Categories: Prostanoids, leukotrienes (LTs), and lipoxins (LXs)
  • Role: Act as local hormones with significant physiological and pharmacological effects
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5
Q

Prostanoids

A

Includes prostaglandins (PGs), prostacyclins (PGIs), and thromboxanes (TXs)
Synthesis: Formed by cyclization of arachidonic acid to create a cyclopentane ring

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6
Q

Triacylglycerols (Triglycerides)

A
  • Definition: Main storage forms of fatty acids
  • Structure: Esters of glycerol (a trihydric alcohol) and three fatty acids
  • Variants: Mono- and diacylglycerols found in tissues, significant in metabolism of triacylglycerols
  • Carbon numbered in sn-# to indicate position
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7
Q

Phospholipids

A

Basic Structure: Derivatives of phosphatidic acid, consisting of a glycerol, a phosphate group, and two fatty acids
Types of Phospholipids: Examples include phosphatidylcholine, phosphatidylethanolamine, phosphatidylserine, and cardiolipin

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8
Q

Sphingolipids

A

Structure: sphingosine, phosphate, fatty acids

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9
Q

phospholipids

Phosphatidylcholines (Lecithins)

A
  • most abundant in cell membranes
  • crucial for choline storage and nervous transmission
  • lung surfactant
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10
Q

Sphingomyelins

A
  • location and function: outer leaflet of cell membrane lipid bilayer and myelin sheath around nerve fibers
  • biological roles: cell signaling and apoptosis
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11
Q

phosphatidylinositol

in cell membrane

A
  • function: cell signaling
  • signaling pathway: involved in generating second messengers upon hormonal stimulation
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12
Q

Cardiolipin

in mitochondrial function

A
  • location: exclusive to mitochondrial membranes
  • role: essential for mitochondrial energy functions and implicated in diseases
  • metabolic intermediates: lysophospholipid
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13
Q

Ergosterol

Vitamin D Synthesis

A
  • in plants and yeast, dietary source of vit D
  • Bioconversion: transforms into Vitamin D2 under UV light
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14
Q

Polyprenoids

A
  • synthesized from isoprene units like cholesterol
  • Ex: Ubiquinone, dolichols
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15
Q

impaired Fatty acid oxidation and associated diseases

Carnitine Deficiency

A
  • Occurrence: Commonly seen in newborns, especially preterm infants, due to inadequate synthesis or renal leakage
  • Symptoms: Hypoglycemia due to impaired fatty acid oxidation and muscle weakness from lipid accumulation
  • Treatment: Oral supplementation of carnitine
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16
Q

impaired Fatty acid oxidation and associated diseases

Carnitine Palmitoyltransferase CPT Delivery

A

CPT-I Deficiency: Primarily affects the liver, leading to reduced fatty acid oxidation and ketogenesis, resulting in hypoglycemia
CPT-II Deficiency: Mainly impacts skeletal muscle

17
Q

impaired Fatty acid oxidation and associated diseases

Jamaican Vomiting Sickness

A
  • cause: eating unripe fruit of akee tree which contains hypoglycin
  • hypoglycin inactivates acyl-CoA dehydrogenase, leading to hypoglycemia
18
Q

impaired Fatty acid oxidation and associated diseases

Zellweger Syndrome

A
  • severe condition, absence of peroxisomes in tissues
  • accumulation of very long-chain FAs
  • neurological symptoms: facial features similar to Down’s, hypotonia, feeding difficulties liver dysfunction, blindness, adrenocortical failure
  • defective protein carrier: PMP70 peroxisomal membrane protein
19
Q

Acetyl CoA shuttle as citrate

What has carriers in the IMM?

A

citrate, pyruvate, malate

20
Q

Refsum disease

A
  • defective enzyme: phytanoyl-CoA hydroxylase (PHYH) for alpha-oxidation
  • Autosomal recessive
  • accumulation of phytanic acid
  • symptoms: weakness, blindness (retinitis pigmentosa), demyelinating neuropathy
  • Treatment: restrict dietary intake of dairy and meat products
21
Q

Transcription factors for lipogenic enzymes

Sterol regulatory element binding protein 1 c (SREBP-1c)

A
  • Liver X receptor (LXR) stimulates SREBP1 gene
  • Dietary polyunsaturated fatty acids (PUFA) inhibits SREBP1 gene
22
Q

Transcription factors for lipogenic enzymes

Carbohydrate response element binding protein (ChREBP)

A
  • Glucagon activated cAMP inhibits ChREBP
  • Low energy activated AMP-activated protein kinase (AMPK) inhibits ChREBP
23
Q

Regulation of Fatty Acid Synthesis

Glucagon

A
24
Q

4 Regulation of Fatty Acid Synthesis

Insulin

A
25
Q

Phosphatidylethanolamine (Cephalin)

A
  • found in most tissues
26
Q

Phosphatidylserine

A
  • involved in apoptosis
  • serine replace choline
27
Q
A