GLYCOLYSIS / EMBDEN MEYERHOF PATHWAY

 Major pathway for glucose metabolism in all cells, nearly universal o Malate-Aspartate Shuttle
 In cytosol, non-compartmentalized, primitive CYTOSOL MITOCHONDRIA
 Sets the stage for complete oxidation of glucose into CO2 and H2O (via TCA: pyruvic acid to acetyl CoA) OAA  ASP     ASP  OAA
 Integrated into many other metabolic processes, with intermediates common to other pathways ↓ NADH NAD ↑ NAD  NADH  ETC
 3 Types of Chemical Transformations Mal           Mal
o Degradation of carbon skeleton of glucose to yield pyruvate o α-glycerophosphate Shuttle
o Phosphorylation of ADP to ATP by high energy PO4 compounds CYTOSOL MITOCHONDRIA
o Transfer of H+ atoms or electrons to NAD  NADH+H DHAP        DHAP
 Glycolytic intermediates are phosphorylated ↓ NADH NAD ↑ FAD  FADH  ETC
o PO4 negative charge at ph 7 traps intermediate in the cell α-GP         α-GP
o PO4 groups essential for enzymatic conservation of energy  Energetics
o PO4 binds to active site of enzymes providing binding energy to lower activation energy  Aerobic: G + 2 Pi + 2 ADP  2 lactate + 2 ATP + 2 H2O;
Property Hexokinase Glucokinase o net of 2 ATP / G; @ oxygen debt or lacking mitochondria, no net NADH+H consumption
Tissue Distribution Most tissues Liver, pancreas  Anaerobic: G+ 2Pi + 2 NAD + 2 ADP  2 pyruvate + 2 ATP + NADH + 2H+ + H2O;
Glucose Km Low (0.1mM) High (10mM) o net of 2 ATP / G; 2 NADH+H / G, NADH into ETC = 2 or3 ATP
Vmax Low High Reaction Moles ATP produced or consumed / mole G
Substrate specificity D-glucose, hexoses D-glucose only G  G6P -1
Inhibition by G6P yes no F6P  F1,6BP -1
Insulin induction no yes GA3P  1,3BPG +4/+6
 Shuttle System: NADH+H not used in pyr reduction to lac  transported across mitochondrial membrane 1,3BPG  3-PG +2
 Reduction of organic molecule by NADH+H  go to mito  reoxidation with NAD in mito back to cytosol PEP  Pyr +2
Net +2 (+6/+8 if aerobic)

Step Substrate Product Enzyme Action Stimulators / Activators Inhibitors / Deactivators Notes
Glucose Hexokinase, Effectively traps G Irreversible, 1st regulatory step, requires ATP with
Phosphorylation Glucose-6-phosphate (G6P) 2-deoxyglucose
(G) glucokinase inside the cell Mg2+
PREPARTORY STAGE

Phosphoglucose Aldose PO4 

Isomerization G6P Fructose-6-phosphate (F6P) Reversible
isomerase ketose PO4
Irreversible, 2nd regulatory step, committed step,
Fructose-1,6-bisphosphate Adds another PO4 Allosterically by F6P, ATP, citrate, long chain fatty acids
Phosphorylation F6P Phosphofructokinase-I requires ATP, PFK-II reserves excess F6P as F2,6P
(F1,6BP) group AMP, ADP and F2,6BP (LCFA)
for PFK-I
Glyceraldehyde-3-phosphate
Forms 2 triose
Cleavage F1,6BP (GA3P) dihydroxyacetone aldolase Reversible
phosphates
phosphate (DHAP)
Phosphotriose Converts DHAP to
Isomerization DHAP GA3P Reversible
isomerase GA3P
1st redox, requires NAD from NADH from pyruvate to
1,3-bisphosphoglyceric acid GA3P dehydrogenase lactate, maybe linked to ETC if aerobic, 2,3-BPG in
Oxidation GA3P Reduces GA3P Arsenate, iodoacetate
(1,3BPG) (DH) RBC (Rapoport-Luebering Shunt) for O2 transfer by
mutase
Transfers high energy
1,3BPG, ATP, 3-phosphoglycerate Phosphoglycerate Reversible, substrate level phosphorylation,
Formation group from 1,3BPG +
PAY OFF STAGE

ADP (3PG) kinase produces 2 moles ATP / mole glucose

ADP
Phosphoglycerate Shifts PO4 from C3 to
Isomerization 3PG 2-phosphoglycerate (2PG)
mutase C2
Enolase (Mg2+ at Yields high energy
Dehydration 2PG Phophoenolpyruvate (PEP) Fluoride (binds with Mg2+) Reversible
active site) compound
ATP, acetyl CoA, LCFA, covalent
Produces 2ATP via Allosterically by F-1,6-
modification of pyr kinase,
Formation PEP Pyruvate (Pyr) Pyruvate kinase Substrate level BP, insulin stimulates Irreversible, 3rd regulatory step
glucagon inhibits pyr kinase via
phosphorylation pyr kinase
cAMP dependent phosphorylation
Lactic acid DH (LDH1: Reversible, lactic acid fermentation, anaerobic part
Reduction Pyr Lactate (Lac) heart, to lactate; LDH2: of EMP, major fate of Pyr in skel musc and RBC,
skel musc, to pyruvate) involves reoxidation of NADH, ensures NAD supply
 TRICARBOXYLIC ACID CYCLE / KREBS CYCLE / CITRIC ACID CYCLE

 ensures complete oxidation of glucose to CO2 and H2O  Anaplerotic Reactions: refill / replenish depleted intermediates in TCA which have been used for biosynthetic
 in mitosol, amphibolic reactions; continuous functioning
 provides intermediates for biosynthesis of other compounds o Carboxylation of pyruvate
 final oxidative pathway for CHO, FA, AA  Principal anaplerotic reaction, in mitochondria
 oxidation of molecules accounts for greatest fraction(2/3) of O2 consumption and ATP production  Catalyzed by pyruvate carboxylase, ATP-dependent, allosterically activated by aCoA and LCF
 principal source of reducing equivalents that enter ETC acylCoA
 Energetics: acetyl CoA + 3NAD + FAD + GDP + Pi + H2O  2CO2 + 3NADH + 3H+ + FADH2 + GTP + CoASH o Reductive carboxylation of pyruvate to malate
Reactions Moles ATP / Mole Pyr Moles ATP / Mole G  In cytosol
 Catalyzed by NADPH-linked malate enzyme (NOT identical to mitochondrial malate DH)
Pyr  aCoA 3 (NADH) 6
 Provides NADPH for FA synthesis
Ict  oxalosuccinate / αKG 3 (NADH) 6
o Transamination of aspartic acid to OAA
αKG  sCoA 3 (NADH) 6 o Transamination of glutamic acid to αKG
sCoA  suc 1 (ATP) 2 o Degradation of C skeletons of glucogenic AAs
Suc  fum 2 (FADH2) 4  Met, Val, Ile  sCoA
Mal  OAA 3 (NADH) 6  Tyr, Phe  Fum
Total 15 30 (+6/+8 from aerobic glycolysis)

Step Substrate Product Enzyme Action Stimulators / Activators Inhibitors / Deactivators Notes
Pyruvate dehydrogenase (PDH) enzyme Allosterically: AMP,
Oxidation Allosterically: ATP, acetyl
CoASH, NAD, Ca2+,
Preliminary Oxidation

pyruvate Acetyl CoA complex (E1-3); active when Links EMP to TCA Irreversible, in mitosol
(3 Steps) CoA, FA, NADH, high energy
dephosphorylated (kinase / phosphatase) low energy
Decarboxylation pyruvate Hydroxyethyl derivative E1: Pyruvate decarboxylase + TPP
Hydroxyethyl derivative /
Oxidation / Disulfide lipoic acid / E2: Dhidrolipoyl transacetylase + lipoic
Acetyl group as thioester
Transfer acetyl CoA acid
in lipoic acid
Oxidation / Sulfhydryl lipoic acid / Regenerated lipoic E3: Dihydrolipoyl DH + FAD, NAD,
Reoxidation FADH2 acid / FAD CoASH
Oxaloacetate (OAA), ATP, NADH, succinyl CoA, Rate-limiting / regulatory
Condensation Citrate (Cit) Citrate synthase Aldol condensation OAA, aCoA
acetyl CoA (aCoA) citrate, LCF acylCoA step
Isomerization Cit Isocitrate (ict) aconitase Dehydration, hydration Ca2+, ADP Fluorocitrate, ATP
Isocitrate, AMP, ADP,
Oxidation and Releases 1st of 3 NADH ATP, NADH, high energy Irreversible, rate-limiting
Ict α-ketoglutarate (αKG) Ict DH Ca2+, low energy
carboxylation and 1st CO2 charge step
charge
TCA Cycle

Oxidative Releases 2nd NADH and ATP, GTP, NADH, SCoA,

αKG Succinyl CoA (sCoA) α-KG DH AMP, NAD+
decarboxylation CO2 Ca2+, arsenite
Cuts high-energy Substrate level
Cleavage sCoA Succinate (Suc) Suc thiokinase, sCoA synthase
thioester bond  GTP phosphorylation
Suc dehydrogenase (only FAD linked Produces 2 ATP via
Oxidation Suc Fumarate (Fum) ATP, Pi, Suc Malonate, OAA
enzyme in TCA) FADH2 in ETC
Adds H2O to double
Hydration Fum L-Malate (L-Mal) Fumarase Reversible
bond of Fum
Releases 3rd NADH,
Oxidation L-Mal OAA Mal DH NAD+ NADH
regenerates OAA
 HEXOSE MONOPHOSPHATE SHUNT / PENTOSE PHOSPHATE SHUNT
 1st alternative route for glucose oxidation  Summary
 Primarily anabolic o Oxidative: 3 G6P + NADP  3 pentose5P + 6 NADPH + 6 H+ + 3 CO2
 In cytosol, most active in adipose, RBC, adrenal cortex, testes and lactating mammary glands o Non-oxidative: 2 X5P + Rbs5P  GA3P + 2 F6P
 Regulation o Balanced: 3 G6P + 6 NADP  2 F6P + GA3P + 6 NADPH + 6 H+ + 3 CO2
o Based on demands for NADPH, Rbs5P and ATP o For 1 mole G: 6 G6P + 12 NADP  5 hexose6P + 12 NADPH + 12 H+ + 6 CO2
o If NADPH need, non-oxidative phase generates compounds that can be easily reconverted to G6P  Clinical Disorders
o Most important regulator: NADP for G6PDH activity; high NADPH inhibits G6PDH o G6PDH deficiency
o G6PDH inhibited by ↑ levels of reduced glutathione; ↑ CHO intake = G6PDH activity  ↓ NADPH production from deficiency of G6PDH, may be genetically absent or partially active variant
 failure to maintain reduced glutathione (using glutathione reductase) for RBC CM integrity =
Mode Substrate Need for hemolysis
1 Rbs5P Nucleic acid synthesis  failure to maintain normal oxidation state of iron in hemoglobin = methemoglobinemia = RBC cell
2 NADPH + Rbs5P Steroid and lipid synthesis membrane weakening = hemolysis
3 NADPH Reducing agent, detox o Wenicke-Korsakoff Syndrome
4 GA3P  Pyruvate Energy production  Loss of memory, partial paralysis secondary to thiamin deficiency, vitamin B1 deficiency
 ↓ affinity of transketolase for TPP

Step Substrate Product Enzyme Action Notes

Dehydration
G6P 6-phosphogluconolactone (6PGL) G6P DH Produces 1st NADPH Rate-limiting step
Oxidative

Hydrolysis
6PGL lactone group 6-phosphogluconic acid (6PGA) gluconolactonase
Oxidative decarboxylation
6PGA Ribulose-5-phosphate (Rbls5P) 6-Phosphogluconate DH (NADP-linked) Produces 2nd NADPH
Conversion
Rbls5P Ribose-5-phosphate (Rbs5P) Phosphopentose isomerase (ketoisomerase)
Non-Oxidative

(Isomerization)
Conversion Rbs5P Xylulose-5-phosphate (X5P) Phosphopentose epimerase
GA3P + sedoheptulose-7-phosphate
Transketolation 1 X5P + Rbs5P Transketolase Donor: Ketose, Receiver: Aldose
(S7P)
Transaldolation S7P + GA3P Erythrose-4-P (E4P) + F6P Transaldolase Donor: Aldose, Receiver: Ketose
Transketolation 2 X5P + E4P GA3P + F6P Transketolase

URONIC ACID PATHWAY / GLUCURONIC ACID PATHWAY

 2nd alternative pathway for glucose oxidation  Needed for pentose formation and metabolism of non-phosphorylated sugar derivatives
 Catabolic, produces UDP-glucuronate for:  Clinical Conditions:
o GAG synthesis o Essential Pentosuria – ↑ L-xylulose secretion, genetic absence of LX DH, no physiologic consequences
o Glucuronide formation through conjugation o Oxalosis – too much xylitol, deposition of oxalate in brain and kidneys; following parenteral xylitol
o Some polysaccharide synthesis administration; xylitol in carrot, plum, spinach, as sweetener
 Source of ascorbic acid except for man and guinea pigs
Step Substrate Product Enzyme Action Notes
Isomerization G6P Glucose-1-phosphate (G1P) Phosphoglucomutase
Formation G1P + UTP UDP-glucose (UDPG) UDPG pyrophosphorylase
Oxidation UDPG @ C6 UDP-glucuronate (UDPGU) (active glucuronate) UPG DH (NAD-dependent)
Conversion UDPGU D-glucuronate (DGU) hydrolase
Reduction DGU L-gulonate (LG) LG DH (NADPH-dependent) Change from D- to L- isomer Most highly oxidized C is C1 of LG
Formation (3 steps) LG Ascorbic acid
Dehydration LG L-gulonolactone (LGL) Aldonolactonase
Oxidation LGL 2-keto-L-gulonolactone (2KLGL) Gulonolactone oxidase
Conversion 2KLGL L-ascorbic acid Spontaneous
Oxidation L-ascorbic acid L-dehydro-ascorbic acid Ascorbic acid oxidase (plants), heavy metals (humans)
Oxidation LG 3-keto-L-gulonate (3KLG) Keto-L-gulonate DH (NAD-dependent)
Decarboxylation 3KLG L-xylulose (LX)
Conversion (2 steps) LX D-xylulose (DX)
Reduction LX Xylitol LX DH (NADPH-dependent)
Oxidation xylitol DX Xylulose DH
Phosphorylation DX D-xylulose-5-phosphate (DX5P) xylulokinase DX5P can be metabolized in HMP shunt
 GLUCONEOGENESIS (GNG)

 Synthesis of glucose from non-carb sources (lac, pyr, glycerol, glucogenic AA, odd-chain FA)
 Both in cytosol and mitochondria in liver (90%) and renal cortex (10%)
 Not a reverse of glycolysis/EMP; but 8 out of 11 glycolysis steps are reversible and are shared
o Direct reversal of EMP = ΔG°’ = +20 Kcal/mol = very unfavorable thermodynamically
o GNG = ΔG°’ = -9 Kcal/mol = feasible
 4 unique rxns (pyruvate carboxylase (pyr CX), PEP CX, F1,6BP 1-phosphatase, and G6Pase) w/c circumvent the 3 irreversible glycolytic rxns (Hexokinase, PFK1, pyruvate kinase)
 All inhibitors of EMP are activators of GNG and vice versa
 Fxn: maintains blood sugar concentration, uses lactate and glycerol (end products of glycolysis and glycerol), excretes excess protons by kidneys during metabolic acidosis, recycles C skeletons of deaminated AA
 Energetics: couples cleavage of cleavage of 6 high-energy phosphate bonds per glucose molecule
Reaction Change in ATP / Glucose
Pyr  OAA -2 ATP
OAA  PEP -2 GTP (or ATP)
3PGA  1,3BPG -2 ATP
Total -6 ATP
o Summary: 2 pyr + 4ATP + 2GTP + NADH +2H+  glucose + 2 NAD+ + 4ADP + 2GDP + 6Pi
 Regulation
o Enzyme compartmentation – pyr kinase (cytosol), pyr CX (mito)
o Substrate availability – glucogenic AA and lactate
o Allosteric regulation and feedback control
 ↑ Pyr CX by aCoA, ATP
 Pyr kinase if inhibited directs to GNG; ↑ by F1,6BP; ↓ ATP, alanine, free FA, aCoA (high energy)
 ↑ F1,6BP1Pase by citrate, ATP; ↓ by AMP, ADP, F2,6BP
 ↓ G6Pase by Pi and glucose (product inhibition)
o Hormonal control
 Induction of enzyme synthesis – key GNG enzymes ↑ by glucocorticoids, ↓ by insulin (also induces EMP enzymes)
 Covalent modification
 Glucagon ↓ pyr kinase by phosphorylation, ↑ F2,6BPase by phosphorylation
 Insulin ↓ GNG by ↓cAMP levels = ↓ phosphorylation of F2,6BPase

Step Substrate Product Enzyme Action Notes

Conversion Lac Pyr LA DH, NADH
Carboxylation Pyr OAA Pyr CX, biotin, aCoA, ATP, Mg2+, Mn2+ In mitochondria, circumvents conversion of pyr to PEP
***Reduction OAA Malate Mito mal DH Translocates mito OAA to cytosol
*** Reoxidation Malate OAA Cytosol mal DH
Conversion OAA PEP Cytosolic PEP CX, GTP, Mn2+ Liberates CO2 Circumvents conversion of pyr to PEP
Conversion PEP F1,6BP Enolase, phosphoglycerate mutase, 1,3-BPglycerate kinase, GA3P Reversal of glycolysis
DH, Phosphotriose isomerase, aldolase
Dephosphorylation F1,6P F6P F1,6BP 1-phosphatase Allosterically activated by ATP, inhibited by AMP
Isomerization F6P G6P Phosphoglucoisomerase
Dephosphorylation G6P Glucose G6Pase Absent in skel musc, brain to trap free glucose

 GNG from Glycerol o succinyl CoA  malate

o Glycerol (product of TAG hydrolysis) phosphorylated + ATP by glycerokinase (not found in adipose) to  Cori cycle
Glycerol-3P o Lactic acid reaches kidney and liver via blood
o Gly3P oxidized to DHAP by Gly3P DH + NAD o Lactate  glucose
o DHAP enters EMP o Glucose is released to blood  glycolysis  pyruvate
 GNG from AA  Glucose-Alanine Cycle
o C skels of glucogenic AA are degraded into pyr and TCA intermediates o Muscle Alanine transaminates with αKG = Glutamate, pyr in liver
o Mito malate diffuses into cytosol then converted to OAA o Liver pyr  glucose
 GNG from Odd-# FA o Glucose leaves liver, used by extrahepatic tissues in glycolysis, forming pyr
o Forms propionyl CoA (proCoA) after β-oxidation o Alanine may also be released directly by muscles in muscle protein breakdown
o proCoA  methylmalonyl CoA  succinyl CoA  TCA
 GLYCOGEN METABOLISM

 synthesized and stored in cystolic granules in liver and muscle, higher conc in liver, greater % in muscle
 polymeric nature – easier sequestration of energy stores (unlike glucose)
 liver glycogen maintains blood glucose via breakdown; 10% of weight is glycogen (higher in well-fed state), lasts 12-24 hours
 muscle glycogen for fuel reserve; 2% weight as glycogen

GLYCOGENESIS (Synthesis)
Step Substrate Product Enzyme Notes / Action
Isomerization G6P Glucose-1-phosphate (G1P) Phosphoglucomutase
Formation G1P + UTP UDP-glucose (UDPG) + PPi UDPG pyrophosphorylase
Hydrolysis UPDG + PPi Pyrophosphatase
Formation Old glycogen fragment or glycogenin Glycogen primer Glycogen initiator synthase free G cannot accept a molecule of G from UDPG to initiate chain synthesis
Elongation via G from UDPG + primer Elongated primer with G, UDP Glycogen synthase (GlyS) Forms α14 glycosidic bonds,
Transfer
Branching 7G chain Connects to C6 Branching enzyme (α-D-glucsyl-4:6 transferase / Forms α16 glycosidic bonds, irreversible, at least 11G before transfer (4G
oligo 1,41,6 glucantransferase) remains between branching), ↑ solubility, ↑ non-reducing ends = ↑ synthesis

GLYCOGENOLYSIS (Degradation)
Step Substrate Product Enzyme Notes / Action
Phosphorolysis Terminal α1,4 glycosidic bonds at G1P Glycogen phosphorylase Rate limiting step, ends if 4G remaining on chain before branch
non-reducing end
Debranching Glycogen chain Shorter branch Glucose 4:4 transferase Transfers outer 3G of 4G of a branch to non-reducing end of another chain
Glycogen chain G, glycogen chain Amylo α1,6 glucosidase Breaks α1,6 linkage, makes chain available for glycogen phosphorylase

Regulation
Enzyme Activity State (Phosphorylated/ Action/Notes
Dephosphorylated)
GlyS D Inactive P G6P dependent
GlyS I Active D G6P independent
Glycogenesis cAMP-dependent protein kinase Interconverts GlyS D/I
Phosphoprotein phospatase Dephosphorylates GlyS D
Phosphoprotein phospatase inhibitor Active/Inactive P/D
GlyP a Active P Liver: dimer with pyridoxal PO4; Muscle: allosterically activated by ATP, G6P (↑ energy)
GlyP b Inactive D By phosphatase; Muscle: allosterically activated by AMP (↓ energy)
Glycogenolysis
Phosphorylase kinase Active/Inactive P/D Activates GlyP b to a
cAMP-dependent protein kinase With cAMP activates phosphorylase, 2R/2C tetramer

Phosphorylation Cascade and Hormonal Control

 Hormonal status influences glycogen metabolism by affecting cAMP levels
 cAMP  activates cAMP-dependent protein kinase  [1] activates phosphorylase b kinase  phosphorylase b to a = glycogenolysis; [2] GlyS I  GlyS D = ↓ glycogenesis
 phosphorylated phosphoporotein phosphatase inhibitor  inhibits phosphoprotein phosphatase  GlyS I maintained
 ↑ blood G  ↑ insulin  glucose use (EMP, TCA)
 ↓ blood G  ↑ glucagon  create glucose (glycogenolysis, gluconeogenesis)
 cAMP cascade promotes glycogenolysis / energy use
o pro-cAMP: glucagon / epinephrine
o anti-cAMP: Insulin is stimulates phosphodiesterase  cAMP  5’AMP
 FRUCTOSE METABOLISM
 Product of sucrose hydrolysis; 2nd most abundant dietary sugar

Pathway Step Substrate Product Enzyme Notes / Action

Phosphorylation Fructose, ATP Fructose-1-phosphate (F1P) Fructokinase
Cleavage F1P DHAP, glyceraldehyde Aldolase B Isoenzyme of aldolase A of EMP
DHAP For glycolysis
Major Pathway
Phosphorylation Glyceraldehyde, ATP G3P Triokinase For glycolysis or gluconeogenesis
(F1P Pathway)
Oxygenation Glyceraldehyde D-glycerate Glyceraldehyde DH (NAD linked) Serine production via hydroxypyruvate
Reduction and… Glyceraldehyde Glycerol Glyceraldehyde DH (NADH linked) For triglyceride synthesis or gluconeogenesis
Phosphorylation Glycerol Glycerol-3-phosphate Glycerol kinase
Minor Pathway Phosphorylation Fructose, ATP F6P Hexokinase In adipose & muscle, during ↑ fructose conc, enters EMP (pyruvate), or GNG (glucose)
Reduction Glucose D-sorbitol For spermatozoa nutrition, eye lens, Schwann cells
Sorbitol
Oxidation D-sorbitol D-fructose Sorbitol impermeable to cell membrane, step impaired in diabetics  ↑ soribtol = ↑ osmotic pressure,
Pathway
cataract formation, peripheral neuropathy

Clinical Disorders
 Hereditary Fructose Intolerance – genetic deficiency of aldolase B = ↑ F1P = inhibits fructokinase, hepatic glycogen phosphorylase, aldolase A
 Essential Fructosuria – deficient hepatic fructokinase  ↑ blood/urine fructose
 Fructosemia – deficient F1,6BP1P = ↑ F1,6BP in blood and urine

GALACTOSE METABOLISM

 Product of lactose hydrolysis, from complex carb digestion

Step Substrate Product Enzyme Notes / Action
Phosphorylation Galactose (Gal), ATP Gal1P galactokinase
Transfer Gal1P + UDPG UPDGal, G1P uridyltransferase UDPGal may donate Gal in synthetic pathways
Isomerization UPDGal UPDG Gal-4 epimerase UDPG used in uridyltransferase again
Disorders
 Galactosemia – excessive Gal; autosomal recessive
o Galactokinase Deficiency - ↑ blood/urine/tissue Gal, in lens: reduced to galactilol  cataract formation
o Gal1P uridyltransferase Deficiency – classic galactosemia, ↑Gal1P and galactilol; liver cirrhosis, mental retardation, cataracts; omit milk @ infancy

LACTOSE METABOLISM

Step Substrate Product Enzyme Notes / Action

Synthesis Conversion UDPG UDPGal Gal-4 epimerase
Transfer UDPGal, glucose β1,4 linkage Lactose synthase (galactosyl In mammary gland
UDPGal, N-acetyl glucosamine N-acetylgalactosamine transferase)
Degradation Degradation lactose G, Gal lactase In intestinal brush border