TBL 12 Flashcards

Cellular organelles and lipid catabolism

1
Q

Rough ER Functions

A
  • synthesis of exported proteins: mucus
  • proteins for other organelles: lysosome proteins
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2
Q

Smooth ER functions

A
  1. storage of G6P
  2. synthesis of steroid hormones ex: estrogen
  3. detoxfication
  4. plasma membrane maintainence
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3
Q

Golgi Functions

A
  1. Takes proteins from ER
    * sort proteins by destination
    * package into vesicles for transport
    * membrane lipid maintainence
    * chemical modifications (O-glycan, N-glycan, Barcode)
  2. Transport Proteins
    * COPII, Clathrin, COPI
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4
Q

golgi functions

transport proteins

A
  • Exocytosis (Anterograde): COPII and Clathrin (ex: neuron–>Neurotransmitters, Pancreas -> digestive enzymes)
  • Exocytosis (retrograde) COP I: golgi to ER (Immune cells phagocytosis, duodenum, transporter)
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5
Q

mitochondria

A

double membrane organelle essential for metabolism

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6
Q

lysosomes

A
  • cell digestion and recycling
  • low pH, many hydrolase enzymes
  • impermeable membrane
  • made from Golgi sorting and break off
    1. autophagy
    2. recycle biomolecules
    3. G6P stored here
    4. move lipids via fusion
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7
Q

Peroxisomes

A
  • contain oxidative enzymes (Beta, alpha)
  • synthesizes bile and cholesterol
  • catalase to eliminate H2O2
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8
Q

Zellweger Spectrum disorders

A

errors in peroxisome formation due to PEX genes
1. Zellweger: low peroxisome formation
2. Infant refsum: accumulate very long chain FA

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9
Q

Nucleus

A

nucleolus is where functional RNAs are transcribed and assembled

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10
Q

Karyotyping

A
  • visualize during metaphase
  • colchicine stops in metaphase
  • FISH technique
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11
Q

Tay Sach’s

A
  • deficient b-hexoaminidase A (HEXA mutation)
  • GM2 ganglioside accumulates
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12
Q

linoleic acid active lipid pathway

Thromboxanes (TXA2)

A

causes vasoconstriction and thrombosis (coagulation

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13
Q

Prostaglandins (PGI2)

A

causes vasodilation and inhibits thrombosis (coagulation)

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14
Q

endocannabinoids

A

Gi signaling ligands that act on CNS and PNS

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15
Q

Niemann-Pick Disease

A
  • enzyme missing: sphingomyelinase
  • Accumulate: sphingomyelin
  • Findings: cherry red spots in macula, hepatomegaly, microcephaly, intellectual delay, progressive neurodegeneration
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16
Q

Gaucher’s Disease

A
  • enzyme missing: glucocerebrosidase
  • accumulate: glucocerebroside
  • findings