Membrane Lipids & Bile Metabolism

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Author:
Anonymous
ID:
139259
Filename:
Membrane Lipids & Bile Metabolism
Updated:
2012-03-03 09:00:21
Tags:
membrane lipids
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Description:
membrane lipids
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  1. Lipids found in ALL cell membranes
    • P-choline (lecithin; heart)
    • P-serine
    • P-inositol
    • P-ethanolamine (nervous tissue)
  2. Cardiolipin
    Function? C/C?
    Function: component of lung surfactant

    • C/C: Respiratory Distress Syndrome
    • prevalent in premature infants
    • DPPC:S ratio >2, normal
  3. Platelet Activating Factor
    Function?
    platelet aggregation, degranulation & super oxide radicals (from neutrophils & macrophages)
  4. Plasmalogen (ethanolamine)
    Function?
    • Function: unknown; made in peroxisomes
    • Associated w/ Alzheimers & Zellweger's
  5. Phospholipases
    PLA1
    PLA2
    PLC
    PLD
    • PLA1: FA @ C1
    • PLA2: FA @ C2; inhibited by glucocorticoids & releases arachidonic acid
    • PLC & PLD: head group
  6. Sphingolipids & glycolipids
    • intercellular communication
    • antigenic ABO determinants (A-NALG & B-Galactose transferase)
    • abundant in nerve tissue
  7. Glycerophospholipid Synthesis
    DHAP --> G3P --> Phosphatidate --> CDP - Diglyceride --> P-C, P-E, P-S, P-I

    CTP supplies the energy & phosphate
  8. Glycerophospholipid Interconversion
    P-S <--PLD--> P-E (SAM)-->(SAM)-->(SAM)-->P-C
  9. Glycerophospholipid Salvage Pathway
    Ethanolamine & Choline --CTP--> CDP-E/CDP-C --DG--> P-E & P-C
  10. Tay Sachs
    • Disorder: AR
    • Enzyme Deficiency: Hexosaminidase
    • Accumulating Substance: GM2 ganglioside
    • Symptoms: cherry red macula, seizures, early mortality
  11. Gaucher
    • Disorder: AR
    • Enzyme Deficiency: Glucoceribrosidase
    • Accumulating Substance: Glucocerebroside
    • Symptoms: hepatosplenomegaly; retardation (2 & 3) & skeletal disorders (1)
    • Tx: enzyme replacement therapy
  12. Fabry
    • Disorder: X-linked
    • Enzyme Deficiency: alpha-galactosidase A
    • Accumulating Substance: globosides
    • Symptoms: kidney & heat failure; reddish purple skin rash, burning hand sensation
    • Tx: enzyme replacement therapy
  13. Niemann Pick
    • Disorder: AR
    • Enzyme Deficiency: sphingomyelinase
    • Accumulating Substance: sphingomyelin
    • Symptoms:
    • A: jaundice, hepatomegaly, brain damage (infant)
    • B: hepatosplenomegaly (teens)
    • C/D: moderate hepatospleno-enlargement & brain damage (adult)
  14. Krabbes
    • Disorder: AR
    • Enzyme Deficiency: beta galactosidase
    • Accumulating Substance: galactocerebroside
    • Symptoms: feeding difficulties, mental & motor deterioration, seizures, deaf & blind
  15. Sandhoff
    • Disorder: AR
    • Enzyme Deficiency: Hexosaminidase A & B
    • Accumulating Substance: GM2 & globosides
    • Symptoms: severe Tay Sachs, macrocephaly, cherry red macula
  16. Metachromatic Leukodystrophy - MLD
    • Disorder: AR
    • Enzyme Deficiency: Arylsulfatase A
    • Accumulating Substance: Sulfatides
    • Symptoms: impaired swallowing, paralysis, dementia (infantile & juvenile), psych disorder (adult)
  17. Farber
    • Disorder: AR
    • Enzyme Deficiency: Ceraminidase
    • Accumulating Substance: Ceramide
    • Symptoms: impaired motor & mental ability, arthritis, swollen LN & joints; hoarseness (infantile)

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