Harper's 24: Metabolism of Acylglycerols & Sphingolipids,

BIOCHEM: Metabolism of Acylglycerols and Sphingolipids,

Metabolism of Acylglycerols and Sphingolipids, EoM test 4 -
Metabolism of Acylglycerols and Sphingolipids (Lipids 4),
EoM 4- Metabolism…
Study online at quizlet.com/combine/349095443,332517683,220727882,319541028,226163740

1. majority of lipids in the body Acylglycerols 14. The utilization of glycerol depends on Glycerol kinase
whether such tissues
2. major lipids in fat deposits and in food Triacylglycerols
have the enzyme -----------
lipid transport and storage
15. liver, kidney, intestine, brown adipose glycerol kinase
3. main lipid component of cell phospholipids and
tissue, and the lactating mammary
membranes. sphingolipids
gland contain significant amounts of ---
amphipathic
--------
4. major component of lung surfactant dipalmitoyl lecithin
16. most of the tissues readily uptake FFA brain
respiratory distress syndrome
expect
5. act as precursors of hormone second Inositol
17. TRIACYLGLYCEROLS & acylation of
messengers phospholipids
PHOSPHOGLYCEROLS ARE FORMED triose
6. activate neutrophils, platelet platelet-activating BY: phosphates
aggregation factor (PAF)
18. triacylglycerols, glycerol 3-
alkylphospholipid.
phosphatidylcholine, phosphate
7. contain sphingosine and sugar Glycosphingolipids phosphatidylethanolamine,
residues as well phosphatidylinositol, and
as a fatty acid are found in the outer cardiolipin
leaflet of the plasma membrane with
their oligosaccharide chains facing are formed from__________
outward
19. Significant branch points in the pathway phosphatidate
8. cell adhesion and cell recognition Glycosphingolipids, occur at diacylglycerol
receptors for bacterial toxins like the ---------------and ---------------
cholera steps
ABO blood group substances
20. phosphoglycerols containing ether link plasmalogens
9. INITIATES CATABOLISM OF hydrolysis (-C-O-C-) PAF
TRIACYLGLYCEROLS
21. plasmalogens and PAF are derived from dihydroxyacetone
10. Triacylglycerols must be hydrolyzed lipase phosphate
by a ------ to their constituent fatty
22. ------------------ and -------------- are glycerol-3-
acids and glycerol before further
intermediates in glycolysis, making a phosphate
catabolism can proceed.
very important connection between dihydroxyacetone
11. Much of this hydrolysis (lipolysis) adipose tissue carbohydrate and phosphate
occurs in ------- with release of free lipid metabolism
fatty
23. ------------- is the Common Precursor Phosphatidate
acids into the plasma,
in the Biosynthesis of Triacylglycerols,
12. combines with FFA released through serum albumin Many Phosphoglycerols, & Cardiolipin
hydrolysis
24. Both glycerol and fatty acids must be ATP
13. after hydrolysis of FFA it will be oxidized activated by ---- before they can be
bound by albumin in the plasma and reesterified incorporated into acylglycerols.
transported to the tissues where it
25. Glycerol kinase catalyzes the activation sn-glycerol 3-
will be :
of glycerol to-------------. phosphate
1. ------- to obtain energy
2. ----------
26. when the activity of glycerol kinase is glycerol 3 35. Free Fatty Acid
low or absent, most of the glycerol 3 phosphate
PO4 is formed from dihydroxyacetone dehydrogenase
phosphate
NADH dependent 36. Major components of plasma and PHOSPHOLIPIDS AND
other membranes GLYCOSPHINGOLIPIDS
27. Two molecules of acyl-CoA, formed phosphatidate
by the activation of fatty acids by 37. Phospholipid
acyl-CoA synthetase combine with
glycerol-3-phosphate to form --------
aka: 1,2-diacylglycerol phosphate
28. two enzymes needed to convert sn- glycerol-3-
glycerol-3-phosphate into the phosphate
intermediate phosphatidate acyltransferase
1-acylglycerol-3- 38. Sphingolipids
phosphate
acyltransferase
29. a family of three proteins that have lipins
PAP activity and they also act as
transcription factors which regulate
the expression of genes involved in 39. component of lung surfactant dipalmitoyl lecithin
lipid metabolism.
40. precursor of hormone second Inositol phospholipid
30. only step specific for TAG synthesis diacylglycerol messenger
rate limiting in most circumstances acyltransferase
41. involved in inflammation and Platelet activating
31. in the intestinal mucosa ER chemotaxis factor
monoacylglycerol acyltransferase cytosol
42. important in intercellular Glycosphingolipids
activity usually happens in the --------
communication and contact, as
although phosphatidate
receptors for bacterial toxins and as
phosphohydrolase protein is found
ABO blood group substance
mainly in the------, the active form
of the enzyme is membrane bound 43. Catabolism of
Triacylglycerols
32. Major storage lipid in the adipose TRIACYLGLYCEROL
tissue
FFA- Goes to the
33. Glycerol plasma and bound to
albumin --> Tissue
uptake --> B-oxidation
or reesterification

Glycerol --> Glycerol

Kinase (enzyme) -->
Glycerol 3-phosphate --
> Reesterification

34. Triglyceride 44. In the absence of this enzyme, glycerol kinase

glycerol 3- phosphate is derived from
dihydroxyacetone phosphate (DHAP)
45. Enzyme used in Dihydroxyacetone Glycerol 3-phosphate
phosphate --> Glycerol 3- phosphate Dehydrogenase
46. Activation of Fatty Acids Fatty acid + ATP + CoA -
Involves hydrolysis of two high- -> Acyl CoA + PPi +
energy bonds AMP
47. Enzymes used in hydrolysis of two Acyl CoA Synthetase
high-energy bonds and activation of
FA
48. Two types of SYNTHESIS OF 1. Phosphatidic Acid 58. Phosphatidylinositol 4,5-
TRIACYLGLYCEROLS: Synthesis bisphosphate
2. Triacylglycerol
Synthesis
49. Phosphatidic Acid
Synthesis

59. can undergo progressive methylation Phosphatidylethanolamine

with SAM as methyl donor to form
phosphatidylcholine
60. Phosphatidylserine + Ethanolamine Phosphatidylethanolamine
(((BY SERINE)))
61. Phospholipid in the mitochondria Plasmalogen
50. Triacylglyerol Synthesis
62. Aggregates platelets - Hypotensive PAF
Ulcerogenic
Involved in inflammation, chemotaxis
and protein phosphorylation
63. ACYLGLYCEROL
BIOSYNTHESIS

64. The common intermediate in the PHOSPHATIDATE

synthesis of triacylglycerol and
51. Enzyme used in DHAP --> Glycerol-3- glycerol-3-phosphate
glycerophospholipids
phosphate dehydrogenase
65. - Driven by availability of free fatty REGULATION OF
52. Enzyme used in Glycerol-3-phosphate acyltransferase
acids TRIACYLGLYCEROL AND
--> Monoacylglycerol-3-phosphate
- Fatty acids that escapes oxidation PHOSPHOLIPID
53. Enzyme used in Monoacylglycerol-3- acyltransferase are preferentially converted to SYNTHESIS
phosphate --> Phosphatidic acid phospholipids, and when this
54. Enzyme used in Phosphatidic acid --> Phosphatic Acid requirement is satisfied they are used
1,2-Diacylglycerol Phosphatase for triacylglycerol synthesis

55. Enzyme used in 1,2-Diacylglycerol --> acyltransferase 66. Different portions of the molecule CATABOLISM OF
Triacylglycerol turnover at different rates PHOSPHOLIPIDS

56. ALTERNATIVE PATHWAY OF 67.

TRIACYLGLYCEROL SYNTHESIS IN
THE INTESTINAL MUCOSA
57.
2-Monoacylglycerol -Acyl PHOSPHOLIPASES AND
CoA-> 1,2-Diacylglycerol - THEIR SITES OF ACTION
Acyl CoA->
Triacylglycerol
PHOSPHATIDYLINOSITOL
68. Catalyze transfer of a fatty acid LECITHIN:CHOLESTEROL 74. Demyelinating disease MULTIPLE
residue from position 2 of ACYLTRANSFERASE SCLEROSIS
lecithin to cholesterol to from (LCAT) Involves the loss of both
cholesteryl ester phospholipids and sphingolipids from
white matter
Responsible for cholesteryl
esters in plasma lipoproteins Leads to slowing and eventual
cessation of nerve impulse
69. LECITHIN:CHOLESTEROL Lecithin + Cholesterol -
transmission
ACYLTRANSFERASE LCAT-> Lysolecithin +
Cholesteryl ester
Manifest as weakness, lack of
70. Parent compound of all CERAMIDE coordination, speech and vision
sphingolipids problems
75. Due to absence of enzymes that SPHINGOLIPID
An important signaling
degrade glycosphingolipids in the STORAGE
molecule regulating pathways
lysosomes DISEASES
including programmed cell
death (apoptosis), the cell
Rate of synthesis of stored lipids is
cycle, and cell differentiation
normal
and senescence

Complex lipids containing ceramide

Synthesized in the endoplasmic
accumulate in cells, particularly
reticulum from serine and
neurons, causing neurodegeneration
palmitoyl CoA
and shortening of life span
71. Present in plasma membranes; SPHINGOMYELIN
76. SPHINGOLIPID
especially prominent in myelin,
STORAGE
a membranous sheath that
DISEASES
surrounds and insulates the
axons of some neurons
72. Include cerebrosides and GLYCOSPHINGOLIPIDS
gangliosides

Constituents of outer leaflet of

the plasma membrane 77. glycerol phosphate pathway, what are the 2
monoglycerol pathway pathways for TG
Important in intracellular synthesis
communication and contact, as 78. phosphatidate for there to be
antigens such as ABO blood synthesis of TG in
groups and as receptors for adipose tissue, you
bacterial toxins have to go through

Made up of C24 fatty acids 79. the phosphate is cleaved and we form what happens to
an diglycerol that phosphatidate
73. C24 fatty acids 1. lignoceric acid as we move
2. cerebronic acid forward with the
3. nervonic acid rxn
80. an activated fatty acid what do we add to
the diglycerol to
get to TG
81. lipoprotein lipase fatty acid uptake is
regulated by
82. diglyceride acyltransferase, DGAT what is the rate
limiting enzyme in
TG synthesis
83. glucose and insulin what stimulates DGAT1 in 103. they can go directly into the what is significant about
adipose tissue blood to be used for energy MCT
by every tissue in the body,
84. insulin what stimulated DGAT2 in
including the brain
adipose tissue
104. bile for digestion MCT do not require
85. they can't become obese what happens when we knock
out the gene for DGAT1 in 105. lipase human milk contains what
mice that helps babies
breakdown the fat
86. no does adipose tissue have
glycerol kinase 106. pancreas babies also use bile from
the ____ to breakdown milk
87. DHAP what does adipose tissue use
as a source for glycerol-3- 107. they have an ether bond plamogens are different in
phosphate in TG synthesis instead of an ester bond that
88. glycerol, because it does adipose tissue cannot use ____ 108. DHAP ether lipids come from
not have glycerol kinase as a source for glycerol-3-p,
109. choline or ethanolamine the head alcohol group of
and why?
plasmalogens is typically
89. glycerol and DHAP the liver uses what as a source
110. plasmalogens 50% of the hearts
for glycerol-3-p
phospholipids are
90. dihydroxyacetone DHAP stands for
111. they help make the why is it significant that
phosphate
membranes more stable the heart has so many
91. liver, kidney, intestine where can glycerol plasmalogens
metabolism occur
112. plasmalogens and PAF what 2 things come
92. diglyceride DGAT stands for directly from DHAP
acyltransferase
113. reduce DHAP to G3P we can get PAF directly
93. glucose in the liver, only ___ stimulates from DHAP, whereas is
DGAT1 and 2 the synthesis of other
lipids, we must
94. DGAT what is the rate limiting
step/committed step in TG 114. PAF potent molecular signal
synthesis in all tissues that stimulates platelet
aggregation and serotonin
95. lipid droplets TG in the liver is stored as
release
96. ketone bodies lipids droplets are used to
115. inflammation and play a role in PAF can also stimulate
form
the allergic response
97. VLDL TG that we do not store in the
116. PAF what comes from
liver is put into
phospholipase A2
98. yes, and it is stimulated by does muscle and brain tissue remodeling of membrane
exercise and starvation have GK activity lipids
respectively
117. phosphatidylcholine, 6 glycerophospholipids
99. stay in the muscle to be TG made in the muscle will do phosphatidylethanolamine,
burned for energy by slow what phosphatidylserine,
twitch muscle fibers phosphatidylinositol,
100. milk TG made in mammary glands phosphatidylglycerol,
will end up in diphosphatidylglycerol

101. mammary glands where are medium chained 118. cephalin phosphatidylethanolamine
fatty acids synthesized is also known as

102. in mammary glands the 2nd thioesterase is found 119. cardiolipin diphosphatidyl glycerol is
where also known as
120. lecithin phosphatidylcholine is
also known as
121. PS, PC, PE which glycerolphospholipid 140. diacylglycerokinase, what enzymes are used for
can be formed without energy phosphatase the degradation of second
messengers back to PI
122. CTP what energy do we use to form
glycerophospholipids 141. ER sphingolipid synthesis occurs
where
123. base exchange with PE PS formed by
142. serine and palmitic acid sphingosine is formed using
124. phosphatidyl- PS formation uses the enzyme
ethanolamine-serine 143. every lipid that has a ceramide is a precursor for
transferase sphingosine backbone
125. CDP-choline + DG PC formed by 144. adding a very long chain ceramide is formed by
(energy) fatty acid to sphinganine to
or form N-acyl-sphinganine
SAM methylation of PE this is then oxidized using
(no energy) FAD to give up ceramide
126. methyltransferase enzyme used for SAM 145. ER sphingomyelin synthesis
methylation of PE to PC occurs where
127. CDP-ethanolamine + DG PE formed by 146. ceramide and sphingomyelin is made up of
(energy) phosphocholine
or
147. diglyceride what is a byproduct in the
base exchange with PS
formation of sphingomyelin
(no energy)
148. sphingomyelin synthetase what enzyme is used in the
128. CDP-DG + inositol PI formed by
formation of sphingomyelin
129. CDP-DG + G3P PG formed by
149. golgi complex where does glycolipid
130. CDP-DG + PG DPG formed by synthesis occur
131. lung surfactant, formed dipalmitoyllecithin is what 150. glucosylceramide, what are the 5 glycolipids
using remodeling galactosylceramide,
lactosylceramide,
132. phospholipase A2 what enzyme is used to form
trihexosylceramide,
dipalmitoyllecithin
globoside
133. the removal of a FA from what happens during the rxn to
151. N-acetylgalactosamine globoside always has
position 2 and replacing it form dipalmitoyllecithin
with palmitate 152. 2 how many cerebrosides do
we have
134. phospholipase A2, A1, D, C what are the 4 enzymes
associated with remodeling 153. sialic acid gangliosides always contain
135. -acts on PI, and releases phospholipase A2 actions 154. carbohydrates all the cells in our bodies are
arachidonic acid coated with
-arachidonic acid is a
155. glycocalyx the carbohydrate coating is
precursor for
called the
prostaglandins
156. to prevent the alveoli from what is the function of lung
136. second messenegers phospholipase C plays a role
collapsing surfactant
in producing
157. dipalmitoyl- what phospholipid decreases
137. IP3 PI can be converted to second
phosphatidylcholine tension at the air liquid
messenger
interface and decrease work
138. PI 4-kinase, PI 5-kinase what are the enzymes used to of breathing
activate PI to be converted to
158. deficiency in lung IRDS is caused by
second messengers
surfactant
139. phospholipase C what enzyme is used to
159. dipalmitoyl- what is DPPC
convert activated PI into
phosphatidylcholine, it is
second messenger
the major lung surfactant
160. phospholipid remodeling how is DPPC formed
161. LPC, PG, cholesterol, what are some other 177. liver: glycerol 3-phosphate added describe
surfactant proteins components of lung with fatty acid to make triglyceride cycle
surfactant traicyglycerol
162. taking amniotic fluid levels how do we know if there if
blood: free FAs from adipose liver
of DPPC enough lung surfactant
released for fuel and lipoprotein
163. when DPPC exceeds at what point do we know lipase cleaves glycerol from
sphingomyelin by 2 lungs are fully mature triacylglycerol to be released in
164. synthetic surfactant, bovine what are 2 FDA blood stream
surfactant approved/highly available
methods to prevent RDS adipose tissue: free FAs from cleaved
triaglycerol from liver are joined with
165. 24 weeks gestation when do we begin synthesis
glycerol 3-phosphate to make
of lung surfactant
triacylglycerol, which is then cleaved
166. type II pneumonocytes where is lung surfactant to release glycerol and free FAs,
synthesis carried out where FAs then enter blood for fuel
167. 35 weeks at what point in gestation are 178. enzyme: PEP carboxykinase important enzyme
lungs mature enough product: glycerol 3-phosphate, which and product
168. corticosteroids how can we speed lung then leads to triacylglycerol involved in
maturity synthesis glyceroneogenesis?

169. blood gas, pulse ox how could you diagnose RDS 179. in liver: leads to activation of PEPCK, how do
which leads to more production of glucocorticoids
170. placed on o2 besides replacing lung glycerol 3-phosphate and production interact with
surfactant, what is the other and release of TGs from liver triglyceride cycle?
intervention that needs to
take place in adipose tissue: leads to inhibition
171. 60% of babies born before how often do we see this in of PEPCK, which leads to less
28 weeks develop RDS clinic production of TGs, so free FAs can't
be incorporated into TGs, and free
172. 5% once babies reach 35 weeks
FAs subsequently enter blood, which
gestation, what are their
leads to increase insulin resistance
chances of developing RDS
and disruption of how muscles
173. 1. incorporation into 2 possible fates of handle glucose
triacylglycerides for synthesized or ingested FAs
180. upregulation of PEPCK in adipose how do
storages
tissue which leads to increased thiazolidinediones
2. incorporation into
production of glycerol 3-phosphate interact with
phospholipid components
and TGs, so there is less free FAs triglyceride cycle?
of cells
circulating in the blood
174. phosphatidate fates of FAs begin with the
181. acetyl-CoA and FAs insulin activates
synthesis of?
production of ______
175. phosphatidate common intermediate in the and _______
synthesis of triacylglycerides
182. ketone bodies acetyl-CoA can
and phospholipids?
produce ______ _______,
176. phosphatidate has 2 FAs difference between which is increased
and a phosphorus group phosphatidate and in diabetes
while triacylglycerol has 3 triacyglyceride?
183. 1. FA 4 components of
FAs
2. platform to attach the fatty acid PLs?
3. a phosphate
4. an alcohol
184. 1. synthesis of backbone (glycerol or 4 steps of 197. internalized by endocytosis then fused degradation of
sphingosine) biosynthesis of with lysosomes for degradation by sphingolipids
2. attachment of FAs membrane PLs? specific enzymes
3. addition of a hydrophilic head
198. accumulation of ceremide Farber disease
group through a phosphodiester
bond 199. failure to degrade gangliosides due to Tay-Sachs
4. alteration of the head group to lack of hexosamininidase A, which results disease
yield the final phospholipid product in accumulation of a fatty substance in
the nervous system
185. phosphatidate; alcohol; CTP (loses 2 either the __________ or
phosphates) __________ must be 200. accumulation of sphingomyelin due to Niemann-Pick
activated with ______ defect in sphingomyelinase disease
for synthesis of PL 201. - disease begins during infancy type A
186. CDP-diacylglycerol phosphatidate - enlarged liver and spleen Niemann-Pick
becomes _________ - failure to thrive disease
after CTP activation - progressive deterioration of the
nervous system
187. cleavage of bond between lone activated
phosphate group and phosphate diacyglycerol has 202. less severe type B
group of CMP energy for Niemann-Pick
attachment of disease
results in phosphatidyl-(alcohol) and alcohol because? 203. usually not diagnosed until adulthood type C
CMP what are the Niemann-Pick
resultants? disease
188. alcohol activated by ATP --> CTP steps of alcohol 204. - macrophages become filled with Gaucher
added with release of PPi (so CMP activation undigested glucocerebroside disease
added) --> cleavage between ATP - spleen and liver enlarged
phosphate group and CTP phosphate - fatigue, bleeding problems, fragile
group --> addition of diacyglycerol bones
and release of CMP
205. heoxsaminidase A defect in Tay-
189. phosphatidylcholine most common PL in Sachs
mammals?
206. a-galactosidase defect in
190. phosphatidylethanolamine phosphatidylcholine Fabry's disease
is methylation of
207. arylsulfatase A defect in
what?
metachromatic
191. phosphatidylserine 10% of PLs in leukodystrophy
mammals are ?
208. B-galactosidase defect in
192. sphingosine sphingolipids Krabbe's
backbone? disease
193. amide linkage good identifier for 209. B-glucosidase defect in
sphingolipids? Gaucher's
194. ceramide initial molecule in disease
sphingolipids 210. sphingomyelinase defect in
synthesis for further Niemann-Pick
synthesis?
211. ceramidase defect in
195. sugar units head group of Farber's
glycolipids? disease
196. sphingomyelin head group: attachment of polar 212. ether-linked alkene linkage at C1 in
phosphocholine head group to make plasmalogen?
sphingomyelin or
213. ether-linked alkene linkage at C1 in
cerebroside head group: glucose cerebroside from
platelet-
ceramide?
activating
factor?
214. acetyl ester molecular group at C2 in 226. phospholipid Diacylglycerol and activated
platelet-activating factor? alcohol to synthesize
__________________
215. - Incorporation into Fatty acids synthesized or
triacylglycerides for ingested have two possible 227. phosphatidylcholine What is the most common
storage fates, name those fates. phospholipid in mammals?
- Incorporation into
228. phosphatidylcholine What is the product of the
phospholipid
methylation of
components of cells
phosphatidylehtanolamine?
216. Phosphatidate ___________________ is a common
229. base exchange reaction How is phosphatidylserine
intermediate in the synthesis of
with phosphatidylcholine synthesized?
triacylglycerides and
or
phospholipids
phosphotidylethanolamine
217. phosphatidate Fatty acids synthesized or
230. backbone is sphingosine How are sphingolipids
ingested have two possible
instead of glycerol different than phospholipids?
fates, Both fates begin with the
synthesis of _________________ 231. glycerol, spingosine Sphingomyelin is a
phospholipid that is not
218. they stimulate PEPCK How do Glucorticoids affect the
derived from _____________. The
transcription in the liver, Triglyceride cycle?
backbone is made of
but inhibit PEPCK in
_______________.
adipose tissue
232. Fatty acid unit, glycolipid What are the components of
219. They stimulate the How do Thiazolidinediones
and then a sugar unit Glycolipids?
transcription of PEPCK affect the Triglyceride cycle?
(glucose, galactose, etc.)
in adipose tissue
233. attachment of polar head How are sphingomyelin or
220. stimulates Insulin ___________ the conversion of
group cerebroside made from
Acetyl-CoA into fatty acids.
ceramide?
221. Fatty acid, Platform to What are the 4 components of
234. Sphingolipids _________________ are internalized by
attach the fatty acid, a phospholipids?
endocytosis then fused with
phosphate and an
lysosomes for degradation by
alcohol
specific enzymes.
222. 1. Synthesis of a What are the 4 steps required
235. Farber What disease is associated
backbone (glycerol or for the synthesis of membrane
with accumulation of ceramide
sphingosine) phospholipids?
due to deficiency in the
2. Attachment of fatty
specific lysosomal enzymes?
acids
3. Addition of a 236. accumulation of ceramide What is Farber Disease?
hydrophillic head group 237. Tay-sachs disease What disease is due to failure
through a to degrade gangliosides due
phosphodiester bond to lack of hexosaminidase A?
4. Alteration of the head
238. Tay-Sachs Disease What disorder is related to
group to yield the final
accumulation of a fatty
phospholipid product
substance in the nervous
223. phosphatidate, alcohol Either the ________________ or the system, results in disability and
____________ must be activated with death?
CDP for synthesis of a
239. Riemann-Pick Disease What disease is related to the
phospholipid
accumulation of
224. before Phosphatidate activated with sphingomyelin due to defect in
CTP ___________ attachment of the sphingomyelinase?
polar head group (alcohol)
225. Diacylglycerol Activated ________________ has energy
for attachment of alcohol
240. Type A Niemann- What disease begins during infancy enlarged liver and spleen (hepatosplenomegaly), failure to thrive
Pick progressive deterioration of the nervous system?
241. Type B What form of Niemann-Pick is a less severe form, that begins during infancy enlarged liver and spleen
(hepatosplenomegaly)?
242. Type C What form of Niemann-Pick is usually not diagnosed until adulthood?
243. Gaucher What disease is associated with macrophages become filled with undigested glucocerebroside,
spleen and liver enlarge
fatigue, bleeding problems, fragile bones?
244. Hexosaminidase A What is the defect in Tay-Sachs disease?
245. alpha- What is the defect in Fabry's Disease?
galactosidase
246. arylsulfatase A What is the defect in Metachromatic Leukodystrophy?
247. beta- What is the defect in Krabbe's disease?
galactosidase
248. beta-glucosidase What is the defect in Gaucher's disease?
249. spingomyelinase What is the defect in Neimann-Pick?
250. ceramidase What is the defect in Farber's Disease?