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MCBM Exam 4 Kat > Amino Acid Oxidation > Flashcards

Amino Acid Oxidation Flashcards

1
Q

What are the two major categories of amino group carbon skeletons?

A

Glucogenic

Ketogenic

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2
Q

What does it mean to be glucogenic?

A

TCA Cycle intermediates that can be shunted to gluconeogenesis

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3
Q

What does it mean to be ketogenic?

A

AcetylCoA derivatives that can be used for ketone body synthesis

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4
Q

Can we make glucose from proteins?

A

Yes, fairly easily

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5
Q

What does breakdown of Methionine synthesize?

A

Cysteine

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6
Q

What does the breakdown of Phenylalanine synthesize?

A

Tyrosine

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7
Q

What 4 glucogenic amino acids can form pyruvate?

A

Ala
Ser
Cys
Trp

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8
Q

What 2 glucogenic aa’s can form Oxaloacetate?

A

Asn

Asp

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9
Q

How many carbons are in amino acids that form pyruvate?

A

3 Carbons form the alpha-keto acid pyruvate

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10
Q

How many carbons are in amino acids that form oxaloacetate?

A

4 Carbons form the alpha-keto acid oxaloacetate

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11
Q

What 5 glucogenic amino acids can form alpha-KG?

A 
Gln
Pro
Arg
His
Glu
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12
Q

How many carbons are in amino acids that form alpha-KG?

A

5 Carbons form alpha-KG but they are first funneled to Glu

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13
Q

What kind of glucogenic/ketogenic aa’s can form SuccinylCoA and AcetylCoA?

A

Branched Chain Amino Acids

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14
Q

What are the branched chain amino acids?

A

Ile
Val
Leu

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15
Q

What does Val form when oxidized?

A

PropionylCoA

Strictly glucogenic

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16
Q

What does Ile form when oxidized?

A

PropionylCoA and AcetylCoA

Both glucogenic and ketogenic

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17
Q

What does Leu form when oxidized?

A

Acetoacetate and AcetylCoA

Strictly ketogenic

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18
Q

Where does transamination of branched chain aa’s occur?

A

In muscle, kidney and brain

NOT THE LIVER

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19
Q

Which steps of oxidation do the branched chain amino acids share?

A

The first 2 steps

Separate after dehydrogenase complex

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20
Q

What two glucogenic aa’s can form SuccinylCoA exclusively?

A

Met

Thr

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21
Q

What do Met and Thr yield before being converted to propionylCoA?

A

alpha-ketoburyate

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22
Q

What is the byproduct of SuccinylCoA conversion from alpha-ketobutyrate?

A

Cysteine

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23
Q

What is PropionylCoA converted too?

A

SuccinylCoA

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24
Q

What two major cofactors are used when converting PropionylCoA to SuccinylCoA?

A

Biotin (Vit. B7)

Cobalamin

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25
What strictly ketogenic aa's can be used to form AcetylCoA?
Leu | Lys
26
What is Lys converted too?
AcetoacetocylCoA
27
Are Leu and Lys the only strictly ketogenic aa's?
YES!!!
28
What type of aa's can be used to form Fumarate and AcetylCoA?
Aromatic Amino Acids
29
What are the aromatic amino acids?
Phe Tyr Trp Aromatics are both glycogenic and ketogenic
30
What is Phenylketonuria?
An autosomal recessive disease resulting from phenylalanine hydroxylase deficiency
31
How does one treat Phenylketonuria?
With a low phenylalanine diet until adolescence
32
What does lack of treatment for Phenylketonuria result in?
Severe mental retardation
33
What is Tetrahydrobiopterin?
A cofactor in many hydroxylase reactions as well as pathways that produce some major NT's
34
What is Alkaptonuria?
The first described genetic disease | Defect in homogentisate oxidase
35
What are the symptoms of Alkaptonuria?
Ochronosis | Arthritis like symptoms
36
What is Ochronosis?
A bluish blackish discoloration of connective tissues due to buildup of acid (Can be found in eye)
37
What deficiency results in Albinism?
A deficiency in tyrosinase which results in decreased melanin production
38
What is glycine converted into in bacteria?
Gly > Ser > Pyr
39
What is glycine converted into in humans?
CO2 and NH4+
40
What 4 aa's are both glycogenic and ketogenic?
3 aromatics: Phe, Tyr, Trp | 1 branched chain: Ile
41
What 2 acids are strictly ketogenic?
Leu | Lys
42
What is Phenylketonuria?
Defect in phenylalanine hydroxylase
43
What is Maple Syrup Urine Disease?
Defect in branched chain aa DH
44
What is Methylmalonic Acidemia?
Defect in methylmalonylCoA mutase
45
What is Homocystinuria?
Defect in cystathione synthase
46
What is carrier status?
Preconception testing of parents
47
What is pre-implantation screening?
Test IVF embryos prior to implantation
48
What are the pre-natal forms of screening for inborn errors of metabolism?
Chorionic villus Amniocentesis Cordocentesis
49
When do you use the Chorionic Villus?
10-12th week of pregnancy
50
When do you use Amniocentesis?
15-18th week of pregnancy
51
When do you use Cordocentesis?
After the 17th week of pregnancy
52
What was the first screening used on newborns?
PKU screening in 1966
53
What percent of CA newborn are screened for 86 different disorders today?
99%
54
What enzyme cofactors are important in 1-carbon transfers?
Biotin THF SAM
55
How does biotin transfer carbons?
Transfers CO2
56
How does THF transfer carbons?
Transfers Carbons in various oxidation states
57
How does SAM transfer carbons?
Transfers methyl groups
58
What is the common name of B6 and what is it's function?
Pyridoxine | Transaminations
59
What is the common name of B7 and what is it's function?
Biotin Carries activated CO2 Carboxylase reactions
60
What is the common name of B9 and what is it's function?
Folate | Precursor for THF for methyl group transfers
61
What is the common name of B12 and what is it's function?
Cobalamin MMACoA > SucCoA homocys > met

MCBM Exam 4 Kat (10 decks)

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