6 beta oxidation

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Created by:

aeng05007  on October 5, 2011

Subjects:

biochem lee

Description:

biochem

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6 beta oxidation

What effect does inceasing dbl bonds and dec c# have on MP of a F.A.
decreases m.p.
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What effect does inceasing dbl bonds and dec c# have on MP of a F.A. decreases m.p.
what is the omega f.a? linoleate
what is carnitine found in? meat
name the enzymes and whats happening in beta oxidation acyl-CoA dehydrogenase puts down a dbl bond and gives you FADH2

enoyl-Coa hydratase adds OH to beta

beta hydroxy acyl coa dehydrogenase makes a ketone to give NADH

thiolase cuts with CoA-SH to make acetyl CoA + long chain F.A.
what other rxns are very similar to beta ox? TCA starting from the enzyme that gives us FADH2
if you have a 16 Carbon fa, how do we calculate the # of acetyl CoA and Fadh and nadh? divide by two to get the acetyl coa
divide by 2 minus one for the # of fadh2 and nadh
how many ATP per acetyl CoA? 12 atp
1 atp + 3 Nadh(9atp) + 1 fadh2(2ATP)
however, in leading up to beta ox, we consumed some ATP. Where and how many? to create fatty acyl coa
2 atp
What are we lacking when we have methylmalonyl acidosis? B12 (bc the enzyme that uses this cofactor is named methylmalonyl coa mutase)
how many acyl coa dehydrogenase enzymes are there? Why? 4, for various length F.A.
whats the name of the acyl coa DH enz that works on medium chain F.A.? MCAD
medium chain fatty acyl-coa dehydrogenase
what happens with a MCAD deficiency? hypoglycemia
lactic acidosis
dicarboxylic aciduria
where does omega FA oxidation occur? ER and MITO
whats the product of omega oxidation? dicarboxylic acid(their production is accelerated by MCAD)
what FA is broken in alpha FA oxidation? phytanic acid
where is phytanic acid produced? chlorphyl breakdown product by plants!
this is the most abundant plant FA
vegetarians do a lot of this
what are the products of alpha oxidation? co2, propionyl-coa, acetly coa
Name the disease when you have a defect in alpha oxidation pathway Refsums disease, a neurological disease
what organelle can we do beta oxidation of very long chain FA in? peroxisomes
Name of disease when theres a defect in the peroxisomal FA oxidation pathway? Zellweger syndrome, a neurological disease

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