BIOCHEM SUPERTABLE

PATHWAY LOCATION RATE-LIMITING SUBSTRATE & END-PRODUCT CLINICAL CORRELATES MNEMONIC

STEP
Glycolysis Cytoplasm Phosphofructokinase-1 Substrate: Glucose Deficiency Process: Gross Annoying Guys Favor Ass Fuck But
(Inhibited by: Citrate, ATP, ↓ End-product: Pyruvate/lactate Dreamy Gorgeous Nerdy Boys Always Prefer Pretty
insulin?)
(inhibited by glucagon) Pyruvate kinase Hemolytic anemia Peppy Awesome Princesses
Yield: 2 ATP, 2 NADH  5/7 PFK (muscle) Low exercise capacity Enzymes: Hot Pretty Pussy Always Takes Great Pussy
ATP Pyruvate DH Congenital lactic acidosis preparation for Eventual Penetration
G3P shuttle: 1 NADH = 1.5 ATP
Malate asparate: 1 NADH = 2.5 ATP
Arsenic poison Inhibits pyruvate DH
Anerobic: 2 ATP
Gluconeogenesi Both (liver, Fructose 1,6- Substrate: Intermediates of
s kidney) bisphosphatase glycolysis and TCA
End-product: Glucose
Krebs Cycle Mitochondria Isocitrate Substrate: Acetyl-CoA Process: Citrate Is Kreb’s Starting Substrate For Making
Dehydrogenase Yield: 2CO2, 1GTP, 3NADH, 2 Oxaloacetate
FADH2  10 ATP
Glycolysis + TCA  30/32 ATP
1 NADH = 2.5 ATP, 1 FADH2 = 1.5 ATP
Glycogenesis Cytosol (liver, Glycogen synthase Substrate: a-D-glucose *Clinical correlates: read glycogen storage diseases
muscle) (Elongation of glycogen End-product: Glycogen
chains)
Glycogenolysis Cytosol (liver, Glycogen phosphorylase Substrate: Glycogen
muscle) (Shortening of glycogen chain) End-product: Glucose – liver,
G6P – muscle

Galactose Cytosol (liver) UDP-hexose-4-epimerase Substrate: Galactose

UDP galactose to UDP glucose
Galactokinase deficiency Galactosemia, galactosuria
GALT deficiency (Classic Galactosemia, galactosuria
galactosemia)

Fructose Cytosol? (liver) NA Substrate: Fructose

Fructokinase deficiency Essential fructosuria: benign, asymptomatic
Aldolase B deficiency Hereditary fructose intolerance: profound hypoglycemia

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Pentose Cytosol (RBC, Glucose-6-phosphate Substrate: Glucose-6-phosphate

Phosphate liver, adipose DH End-product: NADPH, Ribose-
etc) G6PD deficiency ↓NADPH  ↓glutathione reductase  free radicals, peroxide accumulation
Pathway G6P to 6-phosphogluconate 5-phosphate
(HMP) NADPH oxidase Chronic granulomatous disease (neutrophils unable to convert O2 to superoxide,
deficiency hence unable to kill bacteria)

Fatty Acid / TG Cytosol Acetyl-CoA Carboxylase Substrate: Acetyl-CoA

Synthesis Acetyl CoA to Malonyl CoA End-product: Palmitoyl-CoA
Citrate shuttle Yield (Palmitate): 106 ATP
Beta oxidation Mitochondria Carnitine-palmitoyl End-product: Acetyl-CoA,
transferase NADH, FADH2
Carnitine shuttle
Ketogenesis Mitochondria HMG CoA Synthase Substrate: Acetyl-CoA
Acetoacetyl CoA + Acetyl CoA End-product: Acetoacetate, B-
 HMG CoA Diabetic Very high glucagon, very low insulin
hydroxybutyrate, acetone
ketoacidosis
Cholesterol Cytoplasm HMG CoA Reductase Substrate: Acetyl-CoA
Synthesis (SER) HMG CoA  Mevalonate End-product: Cholesterol
Requirement: 2NADPH
Amino Acid See TN
Urea Cycle Both (liver) Carbamoyl phosphate Substrate: NH3, aspartate, CO2 Process: Old Colorful Cars Are Always Fond of Awesome
synthase I End-product: Urea Umbrellas
NH3 + CO2  Carbamoyl Requirement: 3 ATP
PO4
Heme Both (1 and
st
ALA synthase Substrate: Succinyl + Glycine See TN Process: Some Good Doctors Palpate Heart Under Cover
Metabolism last 3 steps in Glycine + Succinyl CoA  End-product: Heme Producing Heme
mitochondria) aminolevulinic acid
Ethanol
Polyol Pathway Conversion of glucose to sorbitol Glucose  Sorbitol (Aldose reductase) Diabetes  No sorbitol dehydrogenase  Accumulation
Sorbitol  Fructose (Sorbitol dehydrogenase) of sorbitol in retina, lens, kidney, schwann cells 
retinopathy, nephropathy, neuropathy

AMINO ACID DISORDERS

Phenylketonuria ↓ phenylalanine ↑ tyrosine Severe mental retardation, failure to walk/talk,
seizure
Alkaptonuria ↑ homogentisic acid Urine turns black on standing
Connective tissue is dark
Albinism Defective melanin synthesis from tyrosine Absence of pigment
Tyrosinemia ↑ fumarylacetoacetate Liver and kidney failure, CNS, cabbage-like odor
Homocystinuria Defect in methionine degradation Ectopia lentis, osteoporosis, mental retardation,
↓ cysteine ↑ homocysteine stroke risk
Cystinuria COLA Defects: Cystine, Ornithine, Lysine, Kidney stones
Arginine
Methylmalonic Methylmalonyl CoA mutase deficiency Seizure, encephalopathy, stroke
Acidemia
Maple Syrup Urine Blocked degradation of Leucine, Isoleucine, Maple-smell of urine
Disease Valine (LIV)
Deficiency in a-ketoacid DH complex
Histidinemia Defective histidase Benign