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34 Cards in this Set

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Serine degradation to 3-Phosphoglycerate:
(Part 1 of 3)

Substrate?
Enzyme?
Products?
Energy?
Cofactor/Coenzyme?
Serine (+ alpha-KA)

Transanimase

3-Hydroxypyruvate (+ alpha-AA)
Serine degradation to 3-Phosphoglycerate:
(Part 2 of 3)

Substrate?
Enzyme?
Products?
Energy?
Cofactor/Coenzyme?
3-Hydroxypyruvate

D-glcyerate Dehydrogenase

D-glycerate

NADH goes in
Serine degradation to 3-Phosphoglycerate:
(Part 3 of 3)

Substrate?
Enzyme?
Products?
Energy?
Cofactor/Coenzyme?
D-glycerate

D-glycerate Kinase

3-Phosphoglycerate

ATP goes in
What are the three reaction types associated with the Serine degradation to 3-Phosphoglycerate?
Transanimation (aminostransferation)
Dehydration
Phophorylation
Serine conversion to pyruvate:

Substrate?
Enzyme?
Products?
Energy?
Cofactor/Coenzyme?
Serine

Serine Dehydratase

Pyruvate (+ NH4)
Serine Conversion to Glycine:

Substrate?
Enzyme?
Products?
Energy?
Cofactor/Coenzyme?
Serine (+ MTHF)

Serine Hydroxymethly Transferase

Glycine (+ N5,N10 Methylene THF)

PLP
Glycine Degradation to Serine?

Substrate?
Enzyme?
Products?
Energy?
Cofactor/Coenzyme?
Glycine (+ N5,N10 Methylene THF)

Serine Hydroxymethly Transferase

Serine (+ MTHF)

PLP
Alternative Glycine Degradation
(not the one that goes to Serine)

Substrate?
Enzyme?
Products?
Energy?
Cofactor/Coenzyme?
Glycine (+ MTHF)

Glycine Cleavage Complex

CO2 and NH4+ (+ N5, N10 Methylene THF)

NADH comes out
Glycine Cleavage System.

What is the P component?
PLP - Dependent

Glycine Decarboxylase
Glycine Cleavage System.

What is the H component?
Aminomethyl transferase

containing Lipoamide
Glycine Cleavage System.

What is the L component?
Lipamide Dehydrogenase

FAD-requiring

NAD-dependent
NFL
DR. D
Glycine Cleavage System.

What is the T component?
THF-containing

Methyl acceptor
Phe catabolism: Step 1:

Type of Reaction
Substrate
Enzyme
Product
Energy
Cofactor/Coenzyme
Additional Note:
Hydroxylation

Phenylalanine + O2 (+ 5,6,7,8 Tetrahydrobiopterin)

Phenylalanine Hydroxylase
(needs O2)

Tyrosine + H2O (+ 7,8 Dihydrobiopterin)

NADH goes in

Simultaneously
Oxidizes Phe
&
Reduces O to H2O
What enzyme replenishes the hydrogens on 7,8-Dihydrobiopterin?

Is anything else required?
Dihydrobiopterin Reductase

NADH must be put in.
Phe catabolism: Step 2:

Type of Reaction
Substrate
Enzyme
Product
Energy
Cofactor/Coenzyme
Additional Note:
Aminotransferation

Tyrosine (+ alpha KG)

Tyrosine Aminotransferase

p-Hydroxyphenylpyruvate (+ Glu)
Phe catabolism: Step 3:

Type of Reaction
Substrate
Enzyme
Product
Energy
Cofactor/Coenzyme
Additional Note:
Oxygenase reaction (2nd one so far)

p-Hydroxyphenylpyruvate (+ O2)

p-Hydroxyphenylpyruvate
Dioxygenase

Homogentistic Acid (+ CO2)
Phe catabolism: Step 4:

Type of Reaction
Substrate
Enzyme
Product
Energy
Cofactor/Coenzyme
Additional Note:
Oxygenase reaction (3rd and final)

Homogentistic acid (+ O2)

Homogentistic acid
1,2-Dioxygenase

Maleyacetoacetate (+ O2)
Phe catabolism: Step 5:

Type of Reaction
Substrate
Enzyme
Product
Energy
Cofactor/Coenzyme
Additional Note:
Isomerization

Maleyacetoacetate

Maleyacetoacetate
Isomerase

Fumarylacetoacetate
Phe catabolism: Step 6:

Type of Reaction
Substrate
Enzyme
Product
Energy
Cofactor/Coenzyme
Additional Note:
Cleavage by Hydrolysis

Fumarylacetoacetate (+ H2O)

Fumarylacetoacetase

Fumerate + Acetoacetate
Phe catabolism: Step 7:

Type of Reaction
Substrate
Enzyme
Product
Energy
Cofactor/Coenzyme
Additional Note:
Transferation

Acetoacetate (+ Succinyl CoA)

3-keto
Acyl-CoA
Transferase

Acetoacetyl CoA (+ Succinate)
In the catabolism of Phe to Acetoacetyl-CoA, which enzyme breaks the ring structure?
Homogentistic acid 1,2 dioxygenase
What is so unique about the enzygmatic properties of the 1st step in Phe catabolism to Tyr?
PAH
(aka - oxygenase aka mixed f(x) oxygenase)

takes the O from O2 and puts it into Phe, thus hydroxylating it

then reduces the remaining O using Tetrahydrobiopterin

then additional re-reduces dihydrobiopterin using NADH.
The catabolism of both Lysine and Leucine yields what two important products?
Acetoacetyl CoA

----> Acetyl CoA
The catabolism of Isoleucine yields what important products? x2
Succinyl CoA

Acetyl CoA
The catabolism of Tryptophan yields what important products? x 4
Acetoacetyl CoA

Pyruvate

Acetyl CoA x2
In the catabolism of Tryptophan, Pyruvate is made from what precursor in the catabolic pathway?
Alanine
In the catabolism of Tryptophan, what intermediate molecule is shared with the catabolic pathway of Lysine?
Alpha-ketoadipate
What two amino acids are both glucogenic, ketogenic, and form fumerate in its catabolic pathway?
Phenylalanine

Tyrosine

(phenylalanine is the precursor to Tyrosine)
List amino acids that can get to pyruvate (via catabolism) in:

1 step x2

2 steps x2

3 steps x1

3+ steps x1
Serine & Alanine

Glycine & Cysteine

Threonine

Tryptophan
THREonine to Pyr in THREe steps
Catabolism of Thr to Pyr: Step 1

Type of reaction
Substrate
Enzyme
Product
Energy
Cofactor/Coenzyme
Additional notes
Transferase

Threonine

Serine hydroxymethyl transferase

Acetaldehyde + Glycine

PLP
Catabolism of Thr to Pyr: Step 2

Type of reaction
Substrate
Enzyme
Product
Energy
Cofactor/Coenzyme
Additional notes
Transferase

Glycine (+ N5, N10 Methylene THF)

Serine Hydroxymethyl Transferase

Serine (+ MTHF)

Reversible reaction
Catabolism of Thr to Pyr: Step 3

Type of reaction
Substrate
Enzyme
Product
Energy
Cofactor/Coenzyme
Additional notes
Dehydratation

Serine

Serine Dehydrotase

Pyruvate and NH4+

PLP

Segmented reaction where water leaves first, then re-enters, thus releasing NH4+
Discuss the alternative pathway Phe takes when there is a PAH deficiency.

Type of reaction
Substrate
Enzyme
Product
Energy
Cofactor/Coenzyme

What gets transferred?
From where? to what?
Transanimation

Phe (+ Pyruvate)

Transanimase

Phenylpyruvate + (Alanine)

PLP required

Amine group (NH4+)
From Phe to Ala
What are the phenylketones? x3

List the precursor for each.
Phenylpyruvate (precursor is Phe)

Phenylacetate (precursor is phenylpyruvate)

Phenyllactate (precursor is phenylpyruvate)