Shuttles Produce Atp, Enzymes

Front 1

Malate - Aspartate Shuttle

Back 1

= 32 moles of ATP / mole of Glucose
2.5 moles of cytosolic NADH

Front 2

Glycerol phosphate Shuttle

Back 2

= 30 moles of ATP / mole of Glucose oxidazed
1.5 moles of ATP / cytosolic NADH

Front 3

All the 20 amino acids can be degraded to TCA cycle intermediates except 2:

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Lysine
Leucine

Front 4

Hyperammonemia is the condition caused by insufficient removal of NH4+ resulting in the following?

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Blood ammonia concentration above normal range (30 -60uM) may cause coma , retardation, seizure and death

Front 5

Maple-syrup urine disease due to branched chain keto acids derivative from isoleucine, leucine, and valine appear in the urine.

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Branched-chain -2-keto acid decarboxylase deficiency leads to maple-syrup urine disease

Front 6

Homocystinuria accumulates homocysteine in blood and body fluids which is due deficiency

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Homocystinuria may result from several defects one of which is deficiency of Cystathionine synthase.

Front 7

A patient suffering from Phenyketonuria has organ damage, unusual posture, and mental retardation.

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Phenylalanine hydroxylase (PAH) converts amino acid phenylalanine to tryosine, another amino acid.
Mutation in both copies of the gene for PAH means that the enzyme is inactive or is less efficient, and the concentration of phenylalanine in the body can be build up to toxic levels.

Front 8

Which of the following molecules may carry amino groups from skeletal muscle cells to the liver?

Back 8

Glutamine

Front 9

When the energy charge in liver cells is low, and oxaloacetate levels are limiting, excess mitochondrial acetyl CoA is converted into which?

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Ketone bodies are produced as a result of starvation when oxaloacetate levels are limiting but energy requirment are hight (or diabetes)

Front 10

A 13 year old boy who had testicular feminization before puberty because the lack of sufficient levels of DHT, but started developing masculine characters. This body underdeveloped testes produced sufficient amount of testosterone to result in androgen surge thus promote masculine charaters. Which enzyme is the young boy?

Back 10

5- alpha-reductase which is required to convert testosterone to dihyrotestosterone(DHT) which is more potent androgenic hormone. A deficiency in this enzyme causes testicular feminization in this young boy. However at the puberty sufficient amounts of testosterone are produced by the underdeveloped testes that promote masculine characters.

Front 11

5 major classes of apolipoproteins

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* A (apo A-I, apo A-II, apo A-IV, and apo A-V)
* B (apo B48 and apo B100)
* C (apo C-I, apo C-II, apo C-III, and apo C-IV)
* D
* E

Front 12

APOLIPOprotein synthesis in the intestine

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Is regulated principally by the fat content of the diet

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APOLIPOprotein synthesis in the liver is controlled by a host of factors:

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_ Dietary composition
_ Hormones (insulin, glucagon, thyroxin, estrogens, androgens
_ Alcohol intake
_ Various drugs (statins, niacin,and fibric acids)

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Apolipoprotein B (APOB) is the primary apolipoprotein of low density lipoproteins (LDL or "bad cholesterol")

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which is responsible for carrying cholesterol to tissues
apo B48 and apo B100

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Apolipoprotein A-I (ApoA-I)is the major protein component of high density lipoprotein (HDL) in plasma

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The protein promotes cholesterol efflux from tissues to the liver for excretion.
Activity associated with high HDL-C and protection from heart disease.
Protection from Alzheimer disease by ApoA1 may rely on a synergistic interaction with alpha-tocopherol.
A wide variety of amyloidosis symptoms are associated with rare ApoA-I mutants.
ApoA-I binds to lipopolysaccharide or endotoxin, and has a major role in the anti-endotoxin function of HDL.

Front 16

Apolipoprotein A-I (ApoA-I)

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It is a cofactor for lecithin cholesterolacyltransferase (LCAT) which is responsible for the formation of most plasma cholesteryl esters.

Front 17

Apolipoprotein A-I (ApoA-I)

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ApoA-I was also isolated as a prostacyclin(PGI2) stabilizing factor, and thus may have an anticlotting effect

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Defects in the gene encoding Apolipoprotein A-I are associated with?

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_ HDL deficiencies,
_ Tangier disease
_ systemic non-neuropathic amyloidosis

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Factors affecting ApoA-I activity

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ApoA-I production is decreased by Vitamin D, and increased by a drug that antagonizes it
Exercise or statin treatment may cause an increase in HDL-C levels by inducing ApoA-I production, but this depends on the G/A promoter polymorphism.

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Apolipoprotein C2

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responsible for the activation of lipoprotein lipase (LPL) in capillaries and thus begins the catabolism of the chylomicrons and VLDL

Front 21

Apolipoprotein C3

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Is a very low density lipoprotein (VLDL) protein.
APOC3 inhibits lipoprotein lipase and hepatic lipase;
it is thought to delay catabolism of triglyceride-rich particles

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APOA1, APOC3 and APOA4 genes are closely linked in human genomes.
The A1 and A4 genes are transcribed from the same strand, while the A1 and C3 genes are convergently transcribed

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An increase in apoC-3 levels induces the development of hypertriglyceridemia

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Apolipoprotein E (APOE)

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A main apoprotein of the chylomicron, binds to a specific receptor on liver cells and peripheral cells.
Essential for the normal catabolism of triglyceride-rich lipoprotein constituents.

Front 24

Apolipoprotein E (APOE) function

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APOE was initially recognized for its importance in lipoprotein metabolism and cardiovascular disease.
Alzheimer's disease (astroglia and microglia cells), immunoregulation, and cognition.

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The APOE gene, ApoE, is mapped to chromosome 19 in a cluster with Apolipoprotein C1 and Apolipoprotein C2

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Defects in APOE result in familial dysbetalipoproteinemia, or type III hyperlipoproteinemia (HLP III).
ApoE is a target gene of liver X receptor, a nuclear receptor member that play role in metabolism regulation of cholesterol, fatty acid, and glucose homeostasis.

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The gene is polymorphic, with three major alleles, ApoE2, ApoE3, ApoE4

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* E2 is associated with the genetic disorder type III hyperlipoproteinemia and with both increased and decreased risk for atherosclerosis.

* E4 has been implicated in atherosclerosis and AD, impaired cognitive function, and reduced neurite outgrowth.