Lecture 34 Flashcards
(12 cards)
Triglyceride synth involves addition of two fatty acids transferred from fatty acyl CoA to ____-__-_____ to yield phosphatidic acid. The phosphate group is hydrolyzed, and finally the third fatty acyl group is added.
Glycerol-3-phosphate
Glycerol-3-phosphate can be formed in adipocytes from the reduction of _______ phosphate (a glycolytic intermediate) using ____ as a coenzyme. In the liver, glycerol can be phosphorylated by glycerol _____ to form Glycerol-3-phosphate.
Dihydroxyacetone phosphate
NADH
Glycerol Kinase
There are two mechanisms for formation of phosphatidyl compounds that contain a phosphatidyl group and a polar head group. One involves the activating of the _______ group, which leads to production of phosphatidyl inositol, and the other involves activation of the _____ ____ group with CDP, which is the mechanism that produces phosphatidyl choline or ethanoliamine.
Diacyglycerol group
Polar head group
Phospholipase ____ is present in many tissue types and in pancreatic juice. It acts on ________, releasing arachidonic acid, which is a precursor of ______ (pain molecules.) This phospholipase is inhibited by ______.
Phospholipase A2
Phosphotidyl Inositol
Arachidonic acid
Glucocorticoids
Phospholipase ___ is found in _____ lysosomes and the alpha-toxin of clostridia and other bacilli. Products of this phospholipase are important in signal transduction pathways.
Phospholipase C
Liver lysosomes
Sphingosine is synthesized from palmitoyl CoA and the amino acid _____. Addition of choline phosphate yields _______, while the addition of sugars yields cerebrosides and globosides. Glycolipids that contain ____ ____ are known as gangliosides.
Serine
Sphingomyelin
Sialic Acid
Sphingolipids are important molecules in cell _____ and are particularly rich in ___ tissue (think myelin.) Hereditary defects in _____ required for their degradation results in their accumulation and frequently involve ______ impairment as in Tay-Sachs disease.
Cell membranes
Nervous
Hydrolases
Neurological impairment
_____ serve as precursors to Sphingomyelin and Glycosphingolipids
CERAMIDES
Tay-Sachs disease results from a deficiency in the enzyme Beta-_________. It is characterized by accumulation of _______ and has symptoms such as cherry-red _____, muscular weakness, and blindness. Often, these patients regress in development, so parents don’t see their children progress through developmental checkpoints; instead, they see them regress.
Beta-HEXOSAMINIDASE A
GANGLIOSIDES
MACULA
Gaucher disease (the most abundant among sphingolipidoses, especially in Ashkenazi Jews) results from a deficiency in Beta-______. It is characterized by accumulation of gluco_______ and has symptoms such as hepato_______ and osteoporosis of long bones. It can be treated with ________ injections.
Beta-GLUCOSIDASE
Glucocerebrosides
hepatoSPLENOMEGALY
Glucocerebrosidase
Nieman-Pick disease results from a deficiency in _______. This results in accumulation of ______, leading to symptoms such as cherry-red _____, neurodegeneration, and hepato______.
SPHINGOMYELINASE
SPHINGOMYELIN
MACULA
hepatoSPLENOMEGALY.
Fabry disease (the only ___-_____ inherited among sphingolipidoses) results from a deficiency in ____-_______. This results in accumulation of ________, leading to symptoms such as red-purple skin rash, burning in _____ extremities, and kidney and heart failure. It can be treated with ______ replacement therapy (Galafold).
X-Linked
Alpha-galactosidase
Globosides
Lower
Enzyme replacement therapy (Galfold.)
