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Ketone Bodies/Sphingolipids
Medical School Biochemistry
26
Biochemistry
Graduate
10/27/2011

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Term
What is the affect of high concentrations of Niacin on cholesterol levels?
Definition
Raises HDL and lowers LDL
Term
Where are Ketone bodies in synthethized?
Definition
In the liver
Term
What tissue types can consume ketone bodies?
Definition
Virtually any tissue can consume ketone bodies except for the liver
Term
What fuel source are Ketone bodies made from?
Definition
fatty acids
Term
Why does beta oxidation not work very well in the absence of carbohydrates?
Definition
Oxaloacetate is removed for gluconeogenesis and is not as available for krebs cycle
Term
Ketone body synthesis mirrors what other lipid metabolic pathway?
Definition
Cholesterol synthesis, HMG CoA though is instead catabolized by HMG CoA Lysase (instead of reductase) to form Acetoacetate
Term
What are the by products of Ketone body synthesis?
Definition
Acetone (volatile) and Beta hydroxybutryate
Term
How do Ketone Bodies work with the Krebs cycle to create ATP?
Definition
Ketone bodies are broken back down to Acetoacetate (via hydroxybutyrate dehydrogenase). Aceto acetate is then used in combination with a Krebs cycle step (succinyl-coA -> Succinate) to create Acetoacetyl-CoA via Succinyl-CoA acetoacetate transferase. This results in the production of 2 Acetyl-CoA
Term
Diabeties has what affect on adipose tissue breakdown?
Definition
upregulates the breakdown of triglycerides into fatty acids (HSL is not inhibited)
Term
Diabetic patients have what effect on Beta oxidation?
Definition
upregulate beta oxidation by no longer inhibiting the carnitine pump (since glycolysis is not occuring).
Term
Diabetic patients have what effect on Beta oxidation?
Definition
upregulate beta oxidation by no longer inhibiting the carnitine pump (since glycolysis is not occuring).
Term
Why do diabetics have upregulated Ketone body formation?
Definition
Diabetes upregulates the breakdown of adipose triglycerides and hinders the ability of a cell downregulate Beta oxidation (Carnitine pump). Furthermore intermediates of the krebs cycle are being drawn off for Gluconeogenesis (Due again to the cells inability to uptake blood glucose). The result is a big buildup of Acetyl-CoA that favors synthesis of Ketone bodies
Term
What is the function of Phospholipase A1, A2, C, D?
Definition
A1: cleaves phospholipid at alpha 1 carbon

A2: cleaves phospholipid at Alpha 2 carbon

Phospholipase C: cleave a phospholipid and make an alcohol

Phospholipase D: cleave a phospholipid to regenerate a phospholipid and a sugar

all are used to make signal molecules
Term
What major signalling molecule is created by Phospholipase C?
Definition
IP3
Term
What major products are created by Phospholipase A2?
Definition
Arachodonic acid and Platelet-activating factor (immune response)
Term
What is DPCC important for?
Definition
major component of Surfactant
Term
From what lipids are Glycolipids synthethized?
Definition
Sphingolipids
Term
What enzyme is used to create Glycosphingolipids (ceramide)?
Definition
mixed function oxidase
Term
Where are glycolipids found in relation to the membrane?
Definition
outside of the membrane
Term
Why are Glycosphingolipids important?
Definition
Embryogenesis and differentiation of cells

Cell mediated immunity

Cell adhesion

Surface receptors for cholera and tetanus toxins

signalling
Term
Dsyfunction of Beta galactosidase gives rise to which disease?
Definition
Generalized gangliosidosis
Term
Dysfunction of the enzyme hexoaminidase-A results in which disease?
Definition
Tay-Sachs
Term
Dysfunction in glucocerebrosidase results in which disease?
Definition
Gaucher's Disease
Term
Dysfunction in alpha galacto sidase A results in which disease?
Definition
Fabry's disease
Term
Dysfunction in hexoaminidase A and B results in which disease?
Definition
Sandhoff's disease
Term
Failure of the enzyme Sphingomyelinase results in which disease?
Definition
Niemann-Pick disease
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