Biochem: EXAM 2 (Ch. 24) Acylglycerol, Phospholipid & Sphingolipid Metabolism

____ are one of more fatty acids esterified to glycerol

acylglycerol

_____ are major lipids in fat deposits in the body

triacylglycerols

_____ are one or two fatty acids esterified to glycerol plus a phosphate that is bonded in the 3rd position

phosphoacylglycerols

Insert picture of phosphoacylglycerol what is this molecule

phosphoacylglycerol

What is the storage form of fat in the body?

triacylglycerol

What are 2 common glycosphingolipids in the body?

cerebrosides and gangliosides

____ synthesis is important in programming cell death, cell cycle regulation, and synthesis of sphingomyelin and glycosphingolipids

ceramide

Ceramide combined with phosphatidylcholine=

sphingomyeline

Where does shingomyelin synthesis mainly take place?

golgi apparatus

What is shingolipidoses?

group of inherited lysosomal enzyme deficiencies that slow the break down sphingolipids, leading to toxic accumulation within cells, mainly affecting the nervous system

What is Tay-Sachs Disease?

a Hexosaminidase A deficiency that causes GM2 builds up in brain tissue which causes Intellectual disabilities, blindness, muscular weakness (death usually by the age of 4)

What is Gaucher Disease?

a β-Glucosidase deficiency which causes Glucosylceramide (cerebroside) to build up which enlarges the liver and spleen, erodes of long bones, and causes intellectual disabilities in infants

What is Niemann-Pick Disease?

a Sphingomyelinase deficiency that causes Sphingomyelin to build up which enlarges the liver and spleen, causes intellectual disabilities, and can be fetal

during childhood

What is Multiple Sclerosis?

A Demyelinating disease that causes the loss of both phospholipids and sphingolipids from white matter of the brain and causes a build up of cholesteryl esters in brain and increase phospholipid accumulation in spinal fluid

What 3 molecules are needed for the synthesis of ceramides?

palmitoyl CoA, acetyl CoA, and serine

What molecule is the precursor to both triacylglycerol and phosphatidylcholine?

1,2-Diacylglycerol

Where are triacylglycerol (fat) molecules synthesized?

small intestinal cells

Glycerol is broken down by the enzyme _____ to become _____ in the first step of triacylglycerol synthesis

glycerol kinase, glycerol-3-phosphate

Does the activity of Glycerol kinase require ATP and is it reversible?

yes, no

What is needed to convert 1,2-Diacylglycerol into Triacyglycerol in fat synthesis?

acyl-coA

What is needed to convert 1,2-Diacylglycerol into Phosphatidylcholine?

CDP-choline

What is the first most important irreversible step of Triacyglycerol synthesis? What are the 2 ways this can happen?

formation of glycerol 3-phosphate which can happen through the enzyme glycerol-3-phosphate dehydrogenase in adipose tissue or glycerol kinase in the liver

Glycerol-3-phosphate dehydrogenase is found in the ___, while glycerol kinase is found in the___

adipose tissue, liver

What is required for the enzyme glycerol-3-phosphate dehydrogenase to initiation triacyglycerol synthesis?

NADH