Lipid Metabolism Flashcards
(24 cards)
Activation of fatty acids into
acyl coenzyme A (acyl-CoA)
occurs mainly in this cellular
compartment
Cytosol
Sequence of steps repeated in
the process of lipogenesis
Condensation → Reduction
→ Dehydration → Reduction
Sequence of steps repeated in
the process of beta-oxidation
of fatty acids
Oxidation → Hydration →
Oxidation → Thiolysis
Site of beta-oxidation of fatty
acids
Mitochondria
Site of oxidation of very long
chain fatty acids
Peroxisomes
Most common inborn error of
fatty acid oxidation
Medium-chain fatty acyl
CoA dehydrogenase
(MCAD) deficiency
This condition occurs due to
the consumption of unripe
fruit of the akee tree due to
the toxin hypoglycin that inactivates medium- and short-chain acyl-CoA
dehydrogenase, inhibiting β-
oxidation and causing
hypoglycemia
Jamaican vomiting
sickness
Deficiency of phytanoyl-CoA
hydroxylase leading to
accumulation of phytanic acid
*Retinitis Pigmentosa
Refsum disease
Inherited absences of
peroxisomes in all tissues
leads to marked
accumulation of very-longchain,
saturated, unbranched
fatty acids in liver and central
nervous system
Zellweger syndrome
Refers to the inability to
transport VLCFAs across the
peroxisomal membrane leads
to accumulation in the brain,
adrenals, and testes
Adrenoleukodystrophy
Autosomal recessive disorder
due to mutations in the CFTR
gene
Cystic fibrosis
This value of sweat chloride
may be used to diagnose
cystic fibrosis
Sweat chloride >60
mmol/L
Products of ketogenesis
Acetoacetate, β- hydroxybutyrate,
Acetone
7-dehydrocholesterol
reductase deficiency
leading to low plasma
cholesterol and elevated 7-
dehydrocholesterol
Smith-Lemli-Opitz
syndrome
Most common form of
congenital adrenal
hyperplasia
21-alpha-hydroxylase
deficiency
Refers to the autoimmune
destruction of the adrenal
cortex leading to
adrenocortical insufficiency
Addison disease
Lipoprotein with the largest
diameter, lowest density,
and highest triacylglycerol
content
Chylomicron
Lipoprotein with the highest
cholesterol content
LDL
Lipoprotein with the highest
protein content
HDL
Cofactor of lecithin :
cholesterol acyltransferase,
and structural component
of HDL
Apolipoprotein A-1
Mediates assembly and
secretion of VLDL, and
structural protein of VLDL,
IDL, LDL
Apolipoprotein B-100
Main apoprotein and
mediates secretion of
chylomicrons from small
intestines
Apolipoprotein B-48
Cofactor of lipoprotein lipase
Apolipoprotein C-II
Mediates uptake of
chylomicron remnants and
IDLs (in the liver)
Apolipoprotein E
