MCBII

• Fructose-1-Phosphate
• Intestinal Sucrase which yields both glucose and fructose
• Not under Insulin control

• is high (low affinity)
• That very little F-6-P will be made with fructose. Fructose is a poor substrate competitor.

• High (lower affinity)
• Can at times lead to backup of F-1-P. Lack of Aldolase B leads to fructose poisoning

Need to add a Phosphate via ATP.

• "Essential Fructosuria"- Fructose accumulation in the urine
• "Hereditary Fructose Intolerance" - Buildup of F-1-P, leads to phosphates sequestered--> low ATP production.

• Production of Glycoconjugates.
• F-6-P + Glutamine --> Glucosamine-6-phosphate + Glutamate
• This is a irreversible reaction
• N/O linkage. N(asparagine), O (threonine or serine)
• Create Mannose-6-P via isomerization

It is a very long lipid molecule that acts as an oligosaccharide carrier in the ER.

• Trapping sugars into a cell by adding Pi Group by NADPH based reduction.
• Example: in the seminal vessicals (remember fructose is broken down faster).

Increased glucose amounts leads to more sorbitol and the high amounts of intracellular glucose causes water retention and damages the cells. Seen in Diabetes patients.

• Galactokinase Galactose--> galactose-1-P
• Aldose reductase Falactose--> galactitol
• Uridyltransferase Galactose-1-P --> UDP-Galactose

• Production of lactose. (in mammary tissue)
• uses the enzyme Lactose synthase that has 2 parts (A & B)
• A= Galactosyltransferase and B= lactalbumin)

• Inhibits protein B of Lactose Synthase
• Promotes Protein B of Lactose Synthase

• UDP-Glucose --> UDP-Glucouronate--> can lead to Vit C or Proteoglycan production
• Enzyme is UDP-glucose Dehydrogenase

A process that creates polar compounds that can be more easily cleared in the urine.

Norepinephine, Glucocorticoids, GH, TH

Excess Glucocorticoids- Fat tissue reduced in the extremities.

• - Insulin- Inhibits lipolysis ( the breakdown of lipids) by inhibiting HSL via Dephosphorylation
• - Glucagon and Epinephrine- Promotes Lipolysis

• Albumine acts as a plasma carrier of FFA's
• Triacylglycerols travel through the blood via Chylomicrons

Activation (addition of CoA) of the FA must occur.

• Fatty Acyl + ATP + CoASH--> Fatty Acyl-CoA
• * done with the Enzyme Acyl-CoA synthetase

• Carnitine transports Long Chain Fatty Acids through membranes into and out of the matrix (its the shuttle).
• It is made of 2 Essential Amino Acids. Lysine and 3 methionine (CH3) groups.
• The cell has 2 Carnitine-palmitoyl transferases

1. Oxidation 2. Hydration 3. Oxidation 4. Thiolytic cleavage

1FADH2 and 1NADH per cycle

RQ = CO2 released/ O2 consumed

Efficiency= ATP produced/ per carbon

• 1. HS-lipase
• 2. Malonyl-CoA inhibites Carnitine Tranferase I. causes FA buildup.
• 3. Caritine transferase I- activity increases in prolonged fast.

• Takes place in the Peroxisomes.
• Peroxide needed.

• Patients have non-functioning peroxisomes. Cannot break down VLCFA due to defective ladelling(M6P) of new proteins.
• Broad nose, and widely spaced eyes.

• When FA's B-carbon has a CH3 and the Alpha carbon cannot be oxidized. B-oxidation is stymied and cannot proceed. leads to the accumulation of Phytanic acid.
• The Hydroxylation step is prevented in this disorder.
• Phytanic acid (high in green plants).

Proper oxidation of unsaturated FA requires isomerization to an intermediate trans shape.

• Need to add a carbon to make it a even number. This is done with CO2 addition.
• Total process requires 3 steps.
• Process requires Cobalamin (B12) and Biotin.

• Medium chain acyl-CoA dehydrogenase (From the 1st step).
• Gets confused with SIDS

Since there is an increase in Acyl-CoA that cannot get into the mitochondria, they are diverted to TAG synthesis. Will see lipid droplets in muscle cells.