BC2002 lecture 11

ATP, higher levels of ATP, glucose

glucose metabolism yields ____ but fatty acid metabolism yields _______ whilst regenerating ______

carbons

Fatty acids yield higher levels of energy- the more _____ that there are

Acetyl-CoA dehydrogenase (ADs)

specific for different length fatty acid.. if deficient, the first enzyme of β-oxidation cause serious disease

acetyl-CoA dehydrogenase

3 isoenzyme encoded by different genes

very long

3-Hydroxyacyl-CoA dehydrogenase deficiency VLCHAD is a _____ chain

medium

Acyl-CoA dehydrogenase deficiency MCAD is a _____ chain

short

Acyl-CoA dehydrogenase deficiency SCAD is a _____ chain

debilitating, low, accumulation

medium chain Acyl-CoA dehydrogenase deficiency can be _____, ___ energy levels, fat mass _____

medium chain acyl-coa dehydrogenase deficiency

treatment is low fat/high carb diet helps and eating little but often

1:66,000

The most common acyl-CoA dehydrogenase deficiency affects _____ (MCAD), tested for at birth!

deposition of excess TGs in liver, dybiosis

Non alcoholic fatty liver disease is ___________, ________ between accumulation and removal

NAFLD, liver scarring, liver disease, liver failure

causes of ______ include hepatitis B virus, poisoning by excess lipid, modern malnutrition, mobilization of non esterified FA from adipose tissue, and decreased oxidation of fat by hepatic cells, which results in _______ and _______ and ______ can result in longer term

wolmans disease

lysosomal acid lipase deficiency, genetic-autosomal recessive- mutations

abnormally accumulate

Wolmans disease, certain fats may ______ in the tissues and organs of the body

Wolmans disease

Disease causing bloating or swelling of the stomach (abdominal distention), vomiting, and significant enlargement of the liver or spleen (hepatosplenomegaly), has life-threatening complications often develop during early childhood

ketones, ketone bodies

formation of ketone bodies, acetyl-CoA formed from fatty acid breakdown fed into stage 2 and 3 of metabolism for energy but can also form _______ which can be used to transfer metabolic energy- known as _______

carbohydrate unavailable, low carbohydrate, untreated

ketone body formation generally happen to provide energy when ______ e..g. _________ diet. particularly pronounced in: starvation, fasting, and _____ diabetes

Acetyl-CoA, liver, acetone

______ from β-oxidation is synthesized into ketone bodies (mainly in the ______). _____ exhaled in the breath

Acetoacetate and β-hydroxybutyrate, energy x2

blood soluble amalgamations of acetyl-CoA, they are exported in blood to organs that need ______ where they reconvert to acetyl-CoA—>

forces, oxidize/burn, carbohydrates

A ketogenic diet _____ body to ______ fats rather than _____. used in several diet plans, can be used to treat epilepsy

acidosis, ketosis, coma, acidifying

Excessive overproduction of ketone bodies causes the blood pH to decrease —>______→ ______—>_______ , so _______ the blood!

measuring acidity of urine, sampling exhaled air for acetone via gas chromatography

ketosis can be quantified by ________ and _______

Type I diabetes

Type of diabetes that is insulin dependent, detected in early life, autoimmune destruction pancreases β cells, and no insulin is produced

plasma membrane

for type I diabetes, Glut4 isn’t released to the _______

isn’t, acetyl-CoA

for type I diabetes, glucose ____ taken up in sufficient quantities, fatty acids undergo oxidation’s to yield ______

inhibits, citrate

for type I diabetes, NADH produced here ______ the TCA cycle, _____ is not produced- cells need energy

ketones bodies produced, high, urine

for type I diabetes they’re sent to tissues and the brain, these cause a pH drop and ketoacidosis- possible coma, glucose levels remain _____, excreted in ____

Type II diabetes, insulin, glucose

type of diabetes that is non-insulin dependent, detected in later life, dietary dependent, and ______ is produced but continuous challenge to insulin receptors alters _____ uptake

type II diabetes

type of diabetes associated with increased glucose levels and hyperinsulinemia

regulation of fat catabolism

occurs mainly at level of carnitine acyl-transferase-1 enzyme

inhibited, will not

carnitine acyl-transferase-1 is ______ when fat breakdown at sufficient levels for energy output and when fats start to be synthesized, fat synthesis and degradation ______ happen at same time

allosterically, malonyl-CoA

Carnitine acyl-transferase-1 inhibited ______ by high concentration of the first product in fatty acid synthesis = _______ blocks fatty acid import into mitochondria

acetyl-CoA carboxylase

3 C malonyl-CoA is made by an enzyme called _________ which adds a carboxyl to acetyl-CoA to form malonyl-CoA

high energy indicators

fatty acid β-oxidation enzymes are inhibited by

dehydrogenase

high (NADH)/(NAD+) ratio inhibits enzyme 2 of betaoxidation-

dehydrogenase, thiolase

high (acetyl-CoA) inhibits enzyme 2 ____ and enzyme 4 _______

AMP-activated kinase (AMPK), AMP/ATP, AMP

is a key regulator of metabolism, when energy is low ______ ratio high and ____-kinase turned on

Acetyl-CoA carboxylase (ACC), malonyl-CoA, on, on, energy

AMPK phosphorylated and inactivates _______—> no ____ produced —> so carnitine acyl-transferase turned ____—> β-oxidation ____—> _____

transcription factors

up-regulate transcription of fat catabolism genes

CREB

glucagon activates the transcription factor ______, which turns on fatty acid catabolism genes and regulates genes in glucose metabolism

PPAR

family of nuclear receptors transcription factors, respond to low energy fasting and starvation, activate genes essential for fatty acid oxidation including: fatty acid transporter, carnitine acyltransferases 1 and 2, the fatty acyl-CoA dehydrogenases

Acetyl-CoA carboxylase

the first rate-limiting enzyme that starts fatty acid synthesis creating malonyl CoA