Dear Biochemistry
Students,
Dear Biochemistry
Students,
First of all, thank you
for taking your time to be interested in Thiolase. Thiolase is a dual function
enzyme. First, thiolase is used in the last step of β oxidation of fatty acid
metabolism. Second, it regulates the production of ketone bodies. It uses
CoEnzyme A as a cofactor!
Thiolase is important in
our fatty acid metabolism and production of ketone bodies. But some people can
be deficient in Thiolase (known as β-ketothiolase deficiency).
Watch out!
• β-ketothiolase deficiency is an inherited disorder in
which the body cannot effectively process a protein building block (amino acid)
called isoleucine. This disorder also impairs the body's ability to process
ketones, which are molecules produced during the breakdown of fats.
• The signs and symptoms
of beta-ketothiolase deficiency typically appear between the ages of 6 months
and 24 months. Affected children experience episodes of vomiting, dehydration,
difficulty breathing, extreme tiredness (lethargy), and, occasionally,
seizures. These episodes, which are called ketoacidotic attacks, sometimes lead
to coma. Ketoacidotic attacks are frequently triggered by infections, periods
without food (fasting), or increased intake of protein-rich foods.
But,
• It is estimated to affect fewer than 1 in 1 million
newborns.
Genetics,
• Mutations in the ACAT1 gene cause β-ketothiolase
deficiency.
Mutations in the ACAT1
gene reduce or eliminate the activity of the ACAT1 enzyme. A shortage of this
enzyme prevents the body from processing proteins and fats properly. As a
result, related compounds can build up to toxic levels in the blood. These
substances cause the blood to become too acidic (ketoacidosis), which can
damage the body's tissues and organs, particularly in the nervous system.
Vote for Thiolase!
Sincerely
- Jin
http://ghr.nlm.nih.gov/condition/beta-ketothiolase-deficiency
This is a good post that gets right to the point. However, it would be good to see the actual reactions that thiolase is involved in rather just telling us that it is involved in that specific reaction. When seeing something visually it adds a another dimension to the story you are trying to sell.
ReplyDeleteI really like your protein. It sounds cool and the images of its structure from the first assignment are awesome! However, I too am curious about more of the biochemistry of thiolase. I think it would be a nice addition to add some of the information from your article summaries as well as diagrams of reactions or interactions with thiolase (these can be from the articles too). It seems like there is a lot of information out there about thiolase, so your protein presentation has the potential to be really compelling.
ReplyDeleteI appreciated the succinctness of your post. Much like Amanda and Shelby, however, I would be interested to see the actual chemistry performed by Thiolase--maybe a mechanism or a diagram that lays it out for the viewers? I feel that additional pictures or details about the chemistry performed by Thiolase would be very effective in helping me understand this enzyme more!
ReplyDeleteCan you expand on this?
ReplyDelete