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BIOCHEMISTRY 2 > Amino Acid Oxidation Pathways > Flashcards

Amino Acid Oxidation Pathways Flashcards

1
Q

Fates of Carbon skeleton

A
  1. Complete oxidation in TCA cycle to CO2 and H2O
  2. Gluconeogenesis
  3. Ketoneogenesis
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2
Q

AAs forming pyruvate

A 
ALA
CYS
GLY
SER
THR
TRP
-glucogenic
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3
Q

AAs forming OAA

A

ASN
ASP
-glucogenic

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4
Q

AAs forming Fumarate

A

PHE
TYR
-glucogenic

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5
Q

AAs forming Succinyl CoA

A 
ILE
MET
THR
VAL
-glucogenic
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6
Q

AAs forming alpha-KG

A 
GLU
ARG
GLN
HIS
PRO
-glucogenic
(all first to Glu)
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7
Q

AAs forming Acetyl CoA

A 
PHE
THR
ILE
TRP
TYR
-both glycogenic and ketogenic
LEU
LYS
-ketogenic
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8
Q

Serine

A

sphingosine - sphingolipids
ethanolamine
choline

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9
Q

Glycine

A

purine synthesis
heme biosynthesis
conjugated bile acids

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10
Q

Glycine cleavage enzyme deficiency

A

nonketotic hyperglycinemia
mental deficiency
early death

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11
Q

Phenylketonuria PKU

A

deficiency in Phe hydroxylase
ketoacidosis
mental retardation
–Phe free diet

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12
Q

Histamine

A

His -> histamine + CO2
enzyme: His decarboxylase
vasodilation, allergic reaction, stimulation of HCl secretion to stomach

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13
Q

Cystathione synthase deficiency

A

hyperhomocysteinemia

atherosclerosis

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14
Q

Methylmalonic aciduria

A

deficiency of vitamin B12

deficieny of methylmalonyl mutase

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15
Q

Branched chain AAs

A

breakdown in muscle, kidney, adipose tissue, brain
special enzyme: BCAT - branched chain aminotransferase
-not in liver
-simple transamination, producing alpha-ketoacids
Val, Ile, Leu

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16
Q

Breakdown of Valine

A

Val- BCAT - 2ketoisovalerate - BCalphaKADH -Isobutyryl CoA - Proprionyl CoA
requires TPP, lipoid acid, CoASH, FAD and NAD+

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17
Q

Breakdown of Isoleucine

A

Ile - BCAT - 2keto3methyl valerate - BCalphaKADH - 2methylbutyryl CoA - Proprionyl CoA + Acetyl CoA
requires TPP, lipoid acid, CoASH, FAD and NAD+

18
Q

Breakdown of Leucine

A

Leu - BCAT - 2ketoiso caproate - BCalphaKADH - Isovaleryl CoA - Acetoacetate + Acetyl CoA
requires TPP, lipoid acid, CoASH, FAD and NAD+

19
Q

Regulation of BC alpha KADH

A

covalent:
activation - protein phosphatase (activated by high BCAA in diet)
inhibition - protein kinase (activated by low BCAA in diet)

20
Q

Deficiency in BCKD

A

maplesyrupurine
ketoacidosis
mental retardation

21
Q

Asparagine -> OAA

A

Asn - ASPARAGINASE - Asp - ASPARTATE AMINOTRANSFERASE - OAA

requires PLP

22
Q

Threonine -> Pyruvate

A

Thr - THREONINE DH - 2amino3ketobutyrate - Gly - SERINE HYDROXYMETHYL TRANSFERASE - Ser - SERINE DH - Pyruvate
requires MTHF

23
Q

Tryptophan -> Pyruvate

A

Trp - Ala - ALANINE AMINO TRANSFERASE - pyruvate

requires PLP

24
Q

Tryptophan + Lysine -> Acetyl CoA

A

Trp / Lys - alpha ketoadipate - glutaryl Coa - Acetoacetyl Coa - B KETOACYL COA TRANSFERASE - Acetyl CoA

25
Q

Phenylalanine -> Acetyl CoA

A

Phe - PHENYLALANINE HYDROXYLASE - Tyr - Acetoacetate - Acetoacetyl CoA - B KETOACYL COA TRANSFERASE - Acetyl Coa
requires THF and O2

26
Q

Leucine -> Acetyl CoA

A

Leu - Acetoacetate - Acetoacetyl CoA - B KETOACYL COA TRANSFERASE - Acetyl CoA
or straight to acetyl coa

27
Q

Isoleucine -> Acetyl CoA

A

straight to acetyl Coa

28
Q

Threonine -> Acetyl CoA

A

Thr - THREONINE DH - 2amino3ketobutyrate - 2AMINO 3KETOBUTYRATE COA LIGASE - Acetyl Coa

29
Q

Arginine -> alpha Ketoglutarate

A

Arg - ARGINASE - ornithine - ORNITHINE delta AMINOTRANSFERASE - glutamate gamma semialdehyde - GLUTAMATE SEMIALDEHYDE DH - Glu - GLUTAMATE DH - alpha KG
occurs primary in liver

30
Q

Proline -> alpha Ketoglutarate

A

Pro - PROLINE OXIDASE - Pyrroline 5 carboxylate - glutamate gamma semialdehyde - GLUTAMATE SEMIALDEHYDE DH - Glu - GLUTAMATE DH - alpha KG

31
Q

Glutamine -> alpha Ketoglutarate

A

Gln - GLUTAMINASE - Glu - GLUTAMATE DH - alphaKG

requires PLP, NADPH/NADH

32
Q

Histidine -> alpha Ketoglutarate

A

His - HISTIDINE AMMONIA LYASE - urocanate - UROCANATE HYDRATASE - 4 imidazolone 5 proprionate - IMIDAZOLONE PROPRIONASE - N-formimino glutamate - GLUTAMATE FORMIMINO TRANSFERASE - Glu - GLUTAMATE DH - alpha KG
reguires THF

33
Q

Methionine -> Succinyl CoA

A

Met - METHIONINE ADENOSYLTRANSFERASE - 5adenosyl methionine sam - 5adeonosyl homocysteine sah - HYDROLASE - homocysteine - CYSTATHIONINE B SYNTHASE - cystathionine - CYSTATHIONINE GAMMA LYASE - alpha ketobutyrate - a KETOACID DH - proprionyl coa - d-methylmalonyl coa -l-methylmalonyl coa -Succinyl coa

34
Q

Threonine -> Succinyl CoA

A

Thr - THREONINE DEHYDRATASE - a ketobutyrate - aKETOACID DH - proprionyl coa - methylmalonyl coa - succinyl coa

35
Q

Isoleucine -> Succinyl CoA

A

Ile - proprionyl coa - methylmalonyl coa - succinyl coa

36
Q

Valine -> Succinyl CoA

A

Val - proprionyl coa - methylmalonyl coa - succinyl coa

37
Q

Metabolism of Glu related neurotransmitters

A

Glu - GLUTAMATE DECARBOXYLASE - GABA - AMINO TRANSFERASE - succinate semialdehyde - SUCCINATE SEMIALDEHYDE DH - succinate

38
Q

Arginine related compounds

A

Arg -> NO synthesis
Arg -> Orn -> polyamines
Arg - Creatine -CREATINE KINASE - creatine P - creatinine

39
Q

Biogenic amine of tyrosine

A

Tyr - TYROSINE HYDROXYLASE - DOPA - DOPA DECARBOXYLASE - dopamine - DOPAMINE B HYDROXYLASE - norepinephrine - NE N METHYL TRANSFERASE - epinephrine

40
Q

Biogenic amine of Tryptophan

A

Trp - TRP HYDROXYLASE - 5hydroxy Trp - AROMATIC AA DECARBOXYLASE - serotonin

41
Q

MAO (monoamine oxidase)

A

in neural + other tissues

deaminates/ inactivates neurotransmitters

42
Q

Serotonin

A

synthesized mostly in intestinal mucosa
degraded by MAO
pain perception, regulate sleep, apetite, temperature, blood pressure, cognitive functions, mood

BIOCHEMISTRY 2 (41 decks)

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