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FA genetics/ Biochem > Metabolism > Flashcards

Metabolism Flashcards

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1
Q

Where do FA synthesis, HMP shunt, steroid synthesis, and cholesterol synthesis occur?

A

Cytoplasm

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2
Q

What cycles occur in both the mitochondria and cytoplasm?

A

Urea
GLuconeogenesis
Heme

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3
Q

What is the rate limiting enzyme of glycolysis? Regulators?

A

Phosphofructokinase 1
+= F2,6-BP, AMP
- = CITRATE, ATP

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4
Q

What is the rate limiting enzyme of Gluconeogenesis? regulators?

A

F-1,6-BPase
+ = ATP,
- = F2,6BP, AMP

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5
Q

What is the rate limiting enzyme of TCA cycle? regulators?

A

Isocitrate dehydrogenase

- = NADH

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6
Q

What is the rate limiting enzyme of HMP shunt?

A

G6PD

- = NADPH

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7
Q

What is the rate limiting enzyme of de novo pyrimidine synthesis?

A

Carbamoyl phosphate synthetase II

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8
Q

What is the rate limiting enzyme of de novo Purine synthesis?

A

Glutamine-PRPP amidotransferase

- = IMP, AMP, GMP

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9
Q

What is the rate limiting enzyme of Urea cycle?

A

Carbamoyl phosphate synthetase I

+ = N- acetylglutamate

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10
Q

What is the rate limiting enzyme of FA synthesis? regulators?

A

Acetyl-CoA carboxylase

- = Palmitoyl CoA, glucagon

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11
Q

What is the rate limiting enzyme of FA oxidation?

A

Carnitine acytransferase I

- = Malonyl CoA

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12
Q

What is the rate limiting enzyme of Ketogenesis?

A

HMG-CoA synthetase

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13
Q

What is the rate limiting enzyme of cholesterol synthesis? regulators?

A

HMG CoA reductase

+ = Insulin, T3

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14
Q

What enzyme is def in SEVERE galactosemia?

A

Galactose-1-phosphate uridyltransferase

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15
Q

What enzyme is def in von Gierke’s disease?

A

Glucose-6-phosphatase= turns G6P–> Glucose for release during gluconeogenesis

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16
Q

What enzyme is deficient in Fructose intolerance?

A

Aldolase B = Fructose-> G3P/ DHAP

17
Q

What enzymes require Thiamine as a cofactor?

A 
Transketolase (ribulose 5P->F6P)
Pyruvate dh (pyruvate-> acetyl CoA)
aKetogluterate dh (aketo--> Succinyl-CoA)
18
Q

Difference btwn Hexokinase & Glucokinase?

A 
Hexo= muscles/Fat, low Km, low Vmax, uninduced by insulin
Glucokinase= Liver/ Pancreatic beta cells, High Vmax/Km
19
Q

What molecules carry CO2, 1 carbon units, or methyl group?

A

CO2= Biotin
1carbon unit= THF
Methyl group= SAM

20
Q

What are the KETOGENIC AAs?

A

Lysine

Leucine

21
Q

benign asymptomatic enzyme def with Fructose appearing in urine?

A

Fructokinase def

22
Q

Enzyme def causing Hypoglycemia, Jaundice, cirrhosis, vomiting and build up of F-1-P?

A

Aldolase B def= Fructose intolerance

23
Q

Enzyme def causing Galactitol accumulation, galactose in urine and infantile cataracts?

A

Galactokinase Def

24
Q

Infant with failure to thrive, jaundice, Hepatomegaly, cataracts, and retardation, with Excess Galactitol accumulation?

A

Galactose 1-phosphate Uridyltransferase def

25
Q

Def in Galactosemia vs Fructose intolerance?

A 
Fructose= Aldolase B
Galactose= Uridyltransferase
26
Q

What cells have only Aldose reductase and NOT sorbitol dehydrogenase?

A

Retina
Schwann
Kidney

27
Q

What are the Ketogenic & glucogenic essential AAs?

A 
Keto= Leu/ Lys
Gluco= Met, Val, His
28
Q

What is the treatment for Hyperammonemia?

A

Low protein

Lactulose to Acidify GI and trap NH4

29
Q

What is Ornithine transcarbamyolase def?

A

MC Uraa cycle enzyme def= Hyperammonia + High Orotic acid in blood/urine
**Infant with Tremor, somnolence, vomiting, and lactic acidosis.

30
Q

Production of DA/NE/ Epi require what 2 AAs?

A

Pheylalanine–> Tyrosine-> DA–> NE –> EPi

31
Q

Conversion of Dopa to DA requires?
DA–> NE requires?
NE–> Epi requires?

A

B6
Vit C
SAM

32
Q

How is glycogenesis and muscle activity coordinated?

A

Ca/ Calmodulin in muscle activate Glycogen phosphorylase to breakdown glycogen

33
Q

Epi & glucagon have what similar actions?

A

increase cAMP
activate PKA
activate Glycogen phosphorylase

34
Q

Function of ApoE?

A

remnant uptake (all but LDL)

35
Q

Function of ApoA1?

A

Activate LCAT on HDL

36
Q

Function of ApoCII

A

Lipoprotein lipase cofactor (HDL, VLDL, chylomicrons)

37
Q

Function of ApoB48?

A

chylomicron secretion

38
Q

ApoB100?

A

Binds LDL receptor

FA genetics/ Biochem (10 decks)

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