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• Phosphorylation deactivates
FATTY ACIDS
6) β-hydroxybutyryl-S-ACP
crotonyl-S-ACP + H2O
FATTY ACIDS
Steps in FA synthesis
• Diseases:
– Deficiency in peroxisomes
FATTY ACIDS
(Zellweger syndrome)
– Defect in peroxisomal
activation of VLCFA (X-linked
adrenoleuko-dystrophy)
– Lead to accumulation of
VLCFA
Alpha-oxidation of FA
• Branched-chain FA like
phytanic acid cannot be
oxidized by beta-oxidation
• Instead, hydroxylated on
alpha carbon
• Genetic deficiency
FATTY ACIDS
(Refsum disease)
Are fatty acids glucogenic?
reaction is
irreversible
Pyruvate
dehydrogenase
reaction is
irreversible
FATTY ACIDS
Ketone bodies
• Excess acetyl CoA (from FA or carbohydrate
degradation) is converted in liver to ketone
bodies: acetoacetate, acetone, and β-
hydroxybutyrate
• Ketone bodies are soluble in blood and can be
taken up and used by various tissues (muscle,
heart, renal cortex) to regenerate acetyl CoA
FATTY ACIDS