Flashcards in Amino Acid Oxidation Deck (61):
What are the two major categories of amino group carbon skeletons?
What does it mean to be glucogenic?
TCA Cycle intermediates that can be shunted to gluconeogenesis
What does it mean to be ketogenic?
AcetylCoA derivatives that can be used for ketone body synthesis
Can we make glucose from proteins?
Yes, fairly easily
What does breakdown of Methionine synthesize?
What does the breakdown of Phenylalanine synthesize?
What 4 glucogenic amino acids can form pyruvate?
What 2 glucogenic aa's can form Oxaloacetate?
How many carbons are in amino acids that form pyruvate?
3 Carbons form the alpha-keto acid pyruvate
How many carbons are in amino acids that form oxaloacetate?
4 Carbons form the alpha-keto acid oxaloacetate
What 5 glucogenic amino acids can form alpha-KG?
How many carbons are in amino acids that form alpha-KG?
5 Carbons form alpha-KG but they are first funneled to Glu
What kind of glucogenic/ketogenic aa's can form SuccinylCoA and AcetylCoA?
Branched Chain Amino Acids
What are the branched chain amino acids?
What does Val form when oxidized?
What does Ile form when oxidized?
PropionylCoA and AcetylCoA
Both glucogenic and ketogenic
What does Leu form when oxidized?
Acetoacetate and AcetylCoA
Where does transamination of branched chain aa's occur?
In muscle, kidney and brain
NOT THE LIVER
Which steps of oxidation do the branched chain amino acids share?
The first 2 steps
Separate after dehydrogenase complex
What two glucogenic aa's can form SuccinylCoA exclusively?
What do Met and Thr yield before being converted to propionylCoA?
What is the byproduct of SuccinylCoA conversion from alpha-ketobutyrate?
What is PropionylCoA converted too?
What two major cofactors are used when converting PropionylCoA to SuccinylCoA?
Biotin (Vit. B7)
What strictly ketogenic aa's can be used to form AcetylCoA?
What is Lys converted too?
Are Leu and Lys the only strictly ketogenic aa's?
What type of aa's can be used to form Fumarate and AcetylCoA?
Aromatic Amino Acids
What are the aromatic amino acids?
Aromatics are both glycogenic and ketogenic
What is Phenylketonuria?
An autosomal recessive disease resulting from phenylalanine hydroxylase deficiency
How does one treat Phenylketonuria?
With a low phenylalanine diet until adolescence
What does lack of treatment for Phenylketonuria result in?
Severe mental retardation
What is Tetrahydrobiopterin?
A cofactor in many hydroxylase reactions as well as pathways that produce some major NT's
What is Alkaptonuria?
The first described genetic disease
Defect in homogentisate oxidase
What are the symptoms of Alkaptonuria?
Arthritis like symptoms
What is Ochronosis?
A bluish blackish discoloration of connective tissues due to buildup of acid (Can be found in eye)
What deficiency results in Albinism?
A deficiency in tyrosinase which results in decreased melanin production
What is glycine converted into in bacteria?
Gly > Ser > Pyr
What is glycine converted into in humans?
CO2 and NH4+
What 4 aa's are both glycogenic and ketogenic?
3 aromatics: Phe, Tyr, Trp
1 branched chain: Ile
What 2 acids are strictly ketogenic?
What is Phenylketonuria?
Defect in phenylalanine hydroxylase
What is Maple Syrup Urine Disease?
Defect in branched chain aa DH
What is Methylmalonic Acidemia?
Defect in methylmalonylCoA mutase
What is Homocystinuria?
Defect in cystathione synthase
What is carrier status?
Preconception testing of parents
What is pre-implantation screening?
Test IVF embryos prior to implantation
What are the pre-natal forms of screening for inborn errors of metabolism?
When do you use the Chorionic Villus?
10-12th week of pregnancy
When do you use Amniocentesis?
15-18th week of pregnancy
When do you use Cordocentesis?
After the 17th week of pregnancy
What was the first screening used on newborns?
PKU screening in 1966
What percent of CA newborn are screened for 86 different disorders today?
What enzyme cofactors are important in 1-carbon transfers?
How does biotin transfer carbons?
How does THF transfer carbons?
Transfers Carbons in various oxidation states
How does SAM transfer carbons?
Transfers methyl groups
What is the common name of B6 and what is it's function?
What is the common name of B7 and what is it's function?
Carries activated CO2
What is the common name of B9 and what is it's function?
Precursor for THF for methyl group transfers