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Biochemistry
Mini 1
12
Medical
Graduate
01/30/2009

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Term
There are certain fatty acids that our bodies cannot make and are essential, they are
Definition
Linoleic acid which is converted to arachidonic acid, arachidonic acid is the precursor to prostoglandins, prostocyclins, and thromboxanes (via the COX1 or oxogenase pathway) and leukotrienes (via the COX2 or lipogenase pathway)
Term
Lipoprotein lipase is an important enzyme that binds chylomicrons and hydrolyzes triglycerides into FFA and monoacylglyercol which is taken up by the cell. This enzyme is attached by _______ to the capillary endothelium
Definition
heparin sulfate proteoglycans, to release the LPL, one can give a pt heparin and then measure the LPL activity in the serum. LPL is not found in the liver or brain
Term
After a meal, high levels of insulin will _______ regulate LPL?
Definition
they will upregulate LPL so as to synthesize triglycerides
Term
Adipose tissue is important in fat metabolism because
Definition
HSL (hormone sensitive lipase) is only found here and when its active, it breaks down fat to release FFA which can be for energy. HSL is upregulated by glucagon, norep, epi, MSH, ACTH, and TSH. Insulin inactivates protein synthesis because this means the body has a rich supply of energy.
Term
the important enzymes involved in beta oxidation are
Definition
Acetyl CoA dedehydrogenase which is the committed step of the Beta oxidation pathway and whose cofactor is riboflavin and the two carnitine transferases which are important in transfering FFA (Acyl) into the inner mitochondrial membrane
Term
Beta oxidation is controlled by
Definition
the availability of FFA and malonyl CoA
Term
Some FFA contain an odd number of carbons and so need to undergo a diff pathway after finishing beta oxidation, this pathway uses
Definition
methylmalonyl mutase to convert methylmalonyl CoA to Succinyl CoA. This pathway relies on biotin and Vit b12. If the mutase is defective or there is a deficiency of B12, pt can develop methylmalonyl aciduria.
Term
some very long chain fatty acids (VLCFA) need to be digested in peroxisomes, what disease is associated with this
Definition
Zellweger Syndrome, which is a defect in peroxisome synthesis and X linked adrenoleukoadenopathy where VLCFA collect in blood and builds up in nervous system, peroxisomes unable to synthesize enough plasminogens
Term
Refsum's Disease occurs when phytanic acid accumulates and results in peripheral neuropathy and muscle wasting. This condition is characterized by what deficiency and is alleviated how?
Definition
it is a deficiency of the alpha hydroxylase and its alleviated by cutting green vegis and ruminant milk and meat from diet
Term
Medium Chain acyl-CoA dehydrogenase deficiency can occur if a pt ingests
Definition
ackee fruit which halts the beta oxidative pathway, fatty acids accumulate in plasma, fat accumulates in liver, more glucose must be oxidized
Term
What is the rate limiting step of FA synthesis and how is it regulated?
Definition
Rate limiting step is Acyl CoA carboxylase and its allosterically activated by citrate (high E), stimulate by insulin, inhibited by glucagon and epi, inhibited by phosphorylation of PKA
Term
production of keton bodies is regulated by
Definition
insulin, glucagon, and epi
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