Xylulose PPP Glycogen synthase NADP+ NADH + H+

GAG Glucuronate
Primed
glycogenin
Branching enz.

Glyc. phosphorylase
Glycogen GLUCOSE
[Osmotic effect]

Sorbitol Fructose nulung

Aldose reductase Sorbitol dehydrogenase
[McArdle disease] Hexokinase ATP G-6-Phosphatase
Debranching enz.
⊕ Ca2+, AMP
Glucokinase [von Gierke disease]
Glycogenin ADP X-5-P
Pi UTP ⊕ insulin ⊖ G-6-P NADPH NADPH TK-TPP
UDP UDP 2 F-6-P
G-1-P G-6-P Ru-5-P X-5-P
GA-3-P
Fructokinase
Glucuronate Glucoseuridyl G-1-P
transferase
G-6-PD
[G-6-PD Deficiency] R-5-P
TA
Phosphoglucoisomerase
PRPP
Bilirubin Epimerase PFK-2 Hexokinase F-1-P GLYCEROL
F-2,6-BP F-6-P
*RATE LIMITING STEP PFK-1 ATP
Bilirubin Gal-1-P ⊕ AMP, ADP, F-2,6-BP F-1,6-Bisphosphatase ATP
UDP uridyl transferase ⊖ ATP, H+, Citrate ADP ⊖ F-2,6-BP Glycerol kinase
diglucuronide
Galactose [Galactosemia] ADP

Lactose synthase
F-1,6-BP NADH NAD+

Lactose DHAP
Glycerol-3-P TRIGLYCERIDE TRIGLYCERIDE
Gal-1-P Glycerol phosphate dehydrogenase
(endogenous - liver)

Lactase
ADP
Galactokinase 2 Glyceraldehyde-3-P Glyceraldehyde Cholesterol
Fatty acyl CoA
[Lactose intolerance] [Galactosemia]
ATP NAD+ 7-α-hydroxylase
Cholesterol ACAT
Galactose NADH + H+ ester LCAT Cholesterol Cholic acid
*RATE LIMITING STEP
Aldose reductase
1,3-BPG ⊕ Cholesterol
⊖ Cholic acid
Chylomicron VLDL
ADP
Glucose Galactitol Mevalonate
[Osmotic effect]
ATP
Bile acid Chylomicron LPL
⊕ Insulin
LPL LPL
Bile salt remnant IDL LDL
Phosphoglycerate *RATE LIMITING STEP HMG CoA reductase
⊕ Insulin
2 NADP+

ADP ⊖ Cholesterol, Glucagon 2 NADPH

ATP HMG CoA

PEP
PK Acetaldehyde Ethanol HMG CoA Triglyceride
PEPCK
⊕ Insulin, AMP
⊖ Acetyl CoA, ATP
2 Acetyl CoA synthase
⊕ Glucagon, Epinephrine, Cortisol (Acetoacetyl CoA) ACC (Biotin) *RATE LIMITING STEP

GDP
PYRUVATE Acetyl CoA
[ACC def. – ARDS]
⊕ Insulin, Citrate
Malonyl CoA Glycerol Gluconeogenesis
PDH NAD+ LDH ⊖ Glucagon, Epinephrine,
Citrate lyase
GTP
⊕ Ca2+, Mg2+, Insulin,
Pyruvate, ADP, NAD
Lactate Cori’s cycle
⊕ Insulin CO2
Palmitoyl CoA
Fatty acid NADPH
ATP NADH + H+ synthase
⊖ Acetyl CoA, ATP, NADH
PC NADP+
(Biotin) ADP OAA Citrate
⊕ Acetyl CoA ACETYL CoA
OAA Palmitoyl CoA Fatty acid
Serine
ATP
Fatty acyl CoA
synthase
Citrate synthase
⊕ ADP
Sphingosine AMP
⊖ ATP, NADH, Citrate, Succinyl CoA DHAP
CITRATE 6C
OAA 4C UDP-Glucose Fatty acyl CoA Plasmalogen

Isocitrate 6C Cerebroside Ceramide CAT I

MDH NAD+ [CAT I def – exercise intolerance]
Phosphatidylcholine ⊖ Malonyl CoA

NADH + H+
Malate 4C CO2
Sphingomyelin
αKG 5C
αKG-DH NAD+ Fatty acyl carnitine
TPP Translocase
NADH + H+ CAT II
CO2
Odd number of Cs
FUMARATE4C SUCCINYL CoA 4C Methyl malonyl CoA Proprionyl CoA Fatty acyl CoA
+ Even number of Cs
Succinate dehydrogenase GDP NAD+, FAD+ Oxidation – Dehydrogenase
Acetyl CoA Hydration – Hydratase

FADH2
Succinate 4C GTP NADH, FADH2
Oxidation – Dehydrogenase
Cleavage – Acetyltransferase
FAD+
Acetyl CoA

2 Acetyl CoA Acetoacetyl CoA HMG CoA

HMG CoA synthase

HMG CoA lyase Acetone

Succinate
Succinyl CoA Acetoacetate

β-Hydroxybutyrate

⊕ ATP
CPS 2 Aspartate transcarbamoylase
Glutamine ⊖ CTP
Carbamoyl aspartate R-5-P
2ATP 2ADP Aspartate ATP
PROTEIN Glutamate Dihydroorotase
PRPP Synthetase
Glutaminase AMP ⊖ AMP, GMP
synthase
Cathepsin ⊖ Cysteine
Cysteine protease
N-Formimino PRPP
Ubiquitin Glutamate Histidine Glutamine
glutamate PRPP Amidotransferase
*COMMITED STEP⊖ AMP, GMP
[High in Folate def.] Glutamate
N-Formimino THF
[High in Orotic aciduria -> anemia] Orotate Glycine + ATP
Amino acid THF
ADP
N10-formyl-THF
α-KG Glutamate OMP
H2O
H2O
Acetyl CoA Proline THF
semialdehyde Glutamine + ATP
NADP+ NAD+
Transaminase GDH Glutamate + ADP
PLP Urea H2O Lysine UMP ATP
NADPH NADH + H+ ATP
Uridylate
NH4+ NH3 + HCO3- ADP
rNDP reductase
kinase
ADP
CO2
Glutamate NAGS
NAG Arginase dUDP
ATP
UDP
ATP
Aspartate
2ATP CPS I Nucleoside diphosphate
⊕ Arginine *RATE LIMITING STEP HMG CoA ADP kinase
2ADP ⊕ GTP ADP
α-keto acid Ornithine Kreb’s Cycle
UTP
Cytidylate synthetase ⊖ CTP
CTP
Carbamoyl Glutamine Glutamate Fumurate
phosphate
Arginine ATP ADP N10-formyl-THF
Acetyl CoA
⊕ High protein diet, THF
Energy depletion,
OTC Starvation Fumarate dUTP dCTP dCDP CDP
rNDP reductase
IMP
Acetoacetate ⊖ GMP NAD+ Aspartate + GTP
⊖ AMP
dUTPase NADH + H+ GDP
Pi Tyrosine
Citruline AMP APRT
XMP Adenylosuccinate
ATP BH2
Glutamine + ATP
Argininosuccinate synthetase BH4 Fumurate
Glutamate + ADP
Asparagine synthetase Phenyl dUMP dCMP
AST
Phenylalanine acetate GMP AMP
OAA Aspartate Asparagine Guanylate Adenylate
[High in PKU]
Asparaginase N5,10-CH2-THF kinase kinase
Glutamate α-KG
rNDP reductase rNDP reductase
dGDP GDP ADP dADP
THF Nucleoside Nucleoside
[High in B6 def. -> B3 def. -> Pellagra] diphosphate diphosphate ETC
Xanthurinate kinase kinase
Gluconeogenesis dTMP dTDP dTTP dCTP UTP dGTP GTP ATP dATP
Succinyl CoA
Cysteine
Aceotoacetyl CoA PLP
Taurine PAPS
B12 Kynureninase
PLP ALT
Methylmalonyl CoA Tryptophan Alanine PYRUVATE
Nucleic acid
3-Phosphoglycerate Endonuclease
Niacin
Propionyl CoA Serine
Glycine
THF Oligonucleotide NTP
Biotin Cysteine
SHMT
PLP GCE PLP
α-Ketobutyrate
N5,10-CH2-THF Phosphodiesterase
PLP
Serine NH4 + CO2
dTMP
Homocysteine
Homocysteine
CH3-B12
N-CH2-THF
THF THF
Nucleotidase
NMP
SAM methyl transferase Uridine
kinase
Methionine B12 N5,10-CH2-THF N5,10-THF
Thymidine kinase Adenosine
Acetyl CoA kinase

BCAA Transaminase BCKA Dehydrogenase Threonine Glycine Nucleoside

PLP Nucleoside phosphorylase
Leucine BCKA Acetyl CoA + Acetoacetyl CoA Pyruvate R-1-P
α-Ketobutyrate Transaminase Alanine
BCAA Transaminase BCKA Dehydrogenase
Isoleucine BCKA Acetyl CoA + Succinyl CoA APRT Thymine
Glyoxylate AMP Adenine Uracil GMP
BCAA Transaminase BCKA Dehydrogenase Cytosine Nucleotidase
Succinyl CoA AMP Deaminase β-Aminoisobutyrate
Valine BCKA Succinyl CoA
β-Alanine
Oxalate Guanosine
IMP α-KG HGPRT

Nucleotidase Glutamate
Malonyl CoA Guanine deaminase
Inosine Guanine

HGPRT
[Low HGPRT - Gout]
XO XO
[HGPRT def. - Lesch-Nyhan Syndrome] Hypoxanthine Xanthine Uric acid
[High in Gout]