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34 Cards in this Set
- Front
- Back
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hormone-sensitive lipase catalyzes
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TGL --> glycerol + FAs
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hormone-sensitive lipase stimulated by
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fall in insulin
epi cortisol no glucagon receptors on adipose tissue |
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niacin inhibits
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HSL
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glycerol fate
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transported to liver for gluconeogenesis
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FA fate
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transported to liver for beta-oxidation to acetyl-CoA
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acetyl-CoA undergoes ketogenesis to produce
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ketone bodies + TCA cycle intermed
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substrate for gluconeogenesis
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dihydroxyacetone phosphate (DHAP)
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tissues that perform beta-oxidation of FAs
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liver
muscle adipose not brain or RBCs |
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oxidation of very long chain FAs (>20) occurs where
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peroxisomes
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what FAs diffuse into mito to be oxidaized
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short and medium chain
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short chain FAs
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2-4C
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medium chain FAs
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6-12
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carnitine acyltransferase=
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carnitine palmitoyl transferase
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what allows long-chain FAs to enter mito
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carnitine transport
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fatty acyl-CoA synthetase located where
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outer mito membrane
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Fatty acyl-CoA synthetase function
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activates FAs by attaching CoA
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carnitine acyltransferase-1 function
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transfers fatty acyl to carnitine (outer mito membrane) - CoA released
fatty acylcarnitine then shuttled across inner membrane |
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carnitine acyltransferase-2 function
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transfers fatty acyl group back to CoA (mito matrix)
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RLS of FA entry into mito
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carnitine acyltransferase-1
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carinitine acyltransferase-1 inhibited by
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malonyl-CoA from FA synthesis
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beta-oxidation of palmitate (16:0) yields
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8 acetyl-CoA
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addition of CoA to FA by fatty acyl-CoA synthetase requires
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ATP
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FA-CoA oxidized to acetyl CoA by what enzyme
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fatty acyl-CoA dehydrogenase (MCAD, LCAD)
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MCAD/LCAD rxns require what
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FAD --> FADH2
NAD --> NADH |
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muscle aches
weakness myoglobinuria provoked by prolonged exercise, esp during fast |
myopathic carnitine acyltransferase (CAT)/CPT deficiency
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CAT deficiency dx
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bx: elevated mm. triglyceride (lipid droplets in cyto)
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myopathic CAT defieciency inheritance
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AR
late-onset |
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fasting hypoglycemia
no ketone bodies (hypoketosis) C8-C10 acyl carnitines in blood vomiting coma, death |
MCAD deficiency
no ATP to support gluconeogenesis pyruvate carboxylase inhibited |
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MCAD deficiency inheritance
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AR with variable expression
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possible reason for SIDS
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MCAD deficiency
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nonketotic hypoglycemia
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block in hepatic beta-oxidation
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may be provoked by illness (flu) that causes n/v, or by overnight fast in infant
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severe hypoglycemia in MCAD deficiency
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MCAD deifiency tx
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IV glucose
prevent with frequent feeding, high carb, low fat diet |
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propionyl acid pathway
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final beta-oxidation step of odd #C FAs yields 1 acetyl-CoA and one propionyl-CoA
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