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7TM
is the receptor of adrenalin catecholamine , cortisol , clucagon that activates the lipolysis
brain and erythrocytes
.......... And ........... Cannot use the fatty acids to produce energy
long chain fatty adcis
fatty acids that need transporter to get into the mitochondria (carnitine )
very long fatty acid
fatty acid enter peroxisomes via an unknown mechanism for oxidation
tha half time of fatty acids
its about 1-3 minutes
long chain free fatty acid
FA whch is water insoluble and are combined with albumin
heart
80 % of its fuel is from b-oxidation
thiokinase
the enzyme that activates B-oxidation and needs 2ATP and CoASH to work
pyrophosphatase
ensures complet activation
carnitine
is synthesized from lysine and methionine in liver and kidney and responsible for transport fatty acid into mitochondria
types of carnitine
1- transferase I : out of mitochondria 2- acylcarnitine translocase 3- transferase II : in the mitochondria
crotonase
also called CoA hydratase enzyme and adds H2O to saturated double bond
thiolase
also called B-ketothiokinase and splits acyl coA into acetyl coA and acyl CoA which is shorter than 2 carbon atom
acetyl CoA
converted to several useful compounds such as : cholesterol , acetyle choline
acetoacetic acid
one of the keton bodies , it is the last 4 carbons remained from B-oxidation
hypoglycemia
مرض يحدث نتيجة نقص الطاقة في الجسم
MCAD
is one of the highest of the inborn errors of metabolism 1%10,000