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Definition
is mitochondrial
Degradation intermediates carried by CoA |
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Definition
FA synthesis is cytosolic
Synthetic intermediates carried by |
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Definition
When ATP is plentiful (fed state) in the mitochondria, it inhibits _____ ______ (TCA) and citrate accumulates.
This promotes the translocation of citrate |
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Term
| acetyl coA carboxylase;biotin |
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Definition
Step 2 Synthesis of Malonyl CoA
What is the enzyme and coenzyme
Rate limiting step in FA synthesis
Exists as inactive dimers.
Citrate binds allosterically causing ACC to polymerize, activating it. |
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Definition
| deactivates ACC, causing it to depolymerize. |
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Term
| AMP dependent protein kinase (AMPK) |
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Definition
| deactivates ACC by phosphorylation |
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Definition
| activate phosphoylating kinases which can also phosphorylate ACC, deactivating it in the fasting state. |
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Definition
| activates protein phosphatases which can dephosphorylate ACC and activate it in the fed state. |
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Definition
may be an oncogene. It is up regulated in some breast and prostate cancers with poor prognosis.
Consists of a dimeric protein with seven different catalytic domains.
A cysteine residue which acts as a "parking" site for intermediates
Acyl Carrier Protein (ACP) site very similar to CoA |
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Term
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Definition
| functions as a "swinging arm" to deliver the intermediates to the correct domain on the FA synthase |
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Definition
| Malate is oxidized and decarboxylated to pyruvate by ____ ____ which is NADP+ dependent. Makes NADPH |
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Definition
| when can you not synthesize tags |
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Definition
| can synthesize, but not store TAG's |
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Definition
| removes FA from C1 and C3 of TAG |
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Definition
| pathway activates protein kinase which phosphorylates HSL, activating it. |
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Definition
| Insulin and high glucose levels inhibit |
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Definition
| The free fatty acids leave the adipocyte and bind to ____ ____ for transport |
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Term
| fatty acyl CoA synthetase |
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Definition
| LCFA is converted to its CoA derivative by ? which is located in the outer mitochondrial membrane. |
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Term
| Carnitine palmitoyl transferase I (CPT-I, CAT-I) |
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Definition
| in the outer mitochondrial membrane attaches the LCFA to carnitine, releasing CoA |
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Term
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Definition
| inhibits CAT-I so that when FA synthesis is in progress, the product cannot be transported for oxidation. |
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Definition
| this deficiency affects primarily liver – fasting hypoglycemia |
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Definition
| this deficiency affects skeletal and cardiac muscle – muscle weakness and cardiomyopathy |
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Definition
| β-carbon of the acyl group is oxidized by ___ ___ ____FADH2 is produced which yields 2 ATP in electron transport |
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Term
| 3-L-Hydroxyacyl CoA dehydrogenase |
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Definition
| converts OH to a keto group producing NADH which gives 3 ATP in ETC |
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Definition
| cleaves the terminal acetyl CoA. The acetyl CoA can enter the TCA and produce a further 12 ATP |
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Definition
| deficiency leads to urinary excretion of propionate and methylmalonate |
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Definition
| Methylmalonic acidemia and aciduria can also be caused by deficiency of the |
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Term
| NADPH-dependent 2,4 dienoyl CoA reductase |
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Definition
Oxidation of unsaturated FA's
requires 3,2-enoyl CoA isomerase which moves a 3-cis double bond to a 2-trans one.
Additionally, if there is a 4-cis double bond (as in polyunsaturates), it prevents the hydration reaction at the β carbon so this is needed |
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Definition
Failure to target VLCFA's to the peroxisome or peroxisome insufficiency results in this condition. Hepatomrgaly, high iron and copper in blood, viual distrbance, lack of muscle tone, glaucoma
No cure, poor prognosis, death by ~ 6 months |
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Term
| X-linked adrenoleukodystrophy |
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Definition
Failure to transport VLCFA into the peroxisome results in this condition
Symptoms: Seizures, ataxia by age 4-10
Cerebral or spinal neuro degeneration
Poor prognosis, death 1-10 yrs from presentation
Bone marrow transplantation
Lorenzo’s oil, thalidomide |
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Definition
| this disease is a deficiency of α-hydroxylase leads to accumulation of phytanic acid and neurological symptoms |
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Term
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Definition
High FA degradation increases Acetyl CoA
Depletes NAD+
Raises NADH
Forces Acetyl CoA into ketone production
Acetone on breath
Excretion of ketone bodies in urine depletes serum volume – polyuria
Ketoacidosis |
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