Protein Metabolism

Created by njawors3 

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Essential AA (9)

Lys, Thr, Trp, Met, Ile, Leu, Val, Phe, His, Arg*

Non-Essential (11)

Ser, Gly, Cys, Ala, Asp, Asn, Arg, Glu, Gln, Pro (from Glucose); Tyr (from Phe)

Transamination RXNs (3)

Pyruvate to Alanine; a-KG to Glu; OAA to Asp; require PLP

Gly & Ser Synthesis

Phosphoglycerate from glycolysis to Serine / Gly

Cys Synthesis

Met donates S and Serine donates N & C

Tyr Synthesis

Phe to Tyr; BH4 cofactor & enzyme PAH; PKU disease = no PAH enzyme activity = build up of Phe in blood & Phenolic Pyruvate

Asn Synthesis

Derivative of Asp; Gln donates amide group

Synthesis of Glu, Gln, Pro, Arg

Formed from a-KG; Glu is precursor

Glucogenic Essential

Val, Thr, Met, His; can produce pyruvate or intermediates of the TCA cycle

Glucogenic Non-Essential

Ala, Arg, Asn, Asp, Cys, Glu, Gln, Gly, Pro, Ser

Glucogenic & Ketogenic (5)

Ile, Thr, & Phenolic (Phe, Trp, Tyr); produce acetyl CoA or ketone bodies and cannot produce OAA which is necessary for gluconeogenesis

Ketogenic (2)

Lys, Leu

Converted to Acetoacetate

Leu, Lys, Phe, Trp, Tyr

Converted to Acetyl CoA

Ile, Thr

Converted to Pyruvate

Ala, Cys, Gly, Ser, Thr, Trp

Converted to Glu then aKG

Gln, Pro, His, Arg

Converted to Succinyl CoA

Ile, Met, Thr, Val

Converted to Fumarate

Phe & Tyr

Converted to OAA

Asn, Asp

BCAA

Val, Ile, Leu; greatest potential for E use in muscle; make up 25% of protein; Leu increases insulin; Ile forms hemoglobin; Val

BCAA Degradation Step 1

Transamination = a-KG converts (Muscle)

BCAA Degradation Step 2

a-Keto groups undergo oxidative decarboxylation by a-keto acid dehydrogenase (Liver)

Val Degradation Products

FADH2, NADH, Gluconeogenic

Ile Degradation Products

NADH, both Gluco & Keto

Leu

FADH2, NADH, Keto

MSUD

maple syrup disease; a-keto acid dehydrogenase enzyme is defective in liver so you get a lot of build up of Glutamate & Glutamine

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