CHAPTER 1General Principles & Energy Production in Medical Physiology 19METABOLIC FUNCTIONS
OF AMINO ACIDS
In addition to providing the basic building blocks for proteins,
amino acids also have metabolic functions. Thyroid hor-
mones, catecholamines, histamine, serotonin, melatonin, and
intermediates in the urea cycle are formed from specific ami-
no acids. Methionine and cysteine provide the sulfur con-
tained in proteins, CoA, taurine, and other biologically
important compounds. Methionine is converted into S-ade-
nosylmethionine, which is the active methylating agent in the
synthesis of compounds such as epinephrine.
CARBOHYDRATES
Carbohydrates are organic molecules made of equal amounts
of carbon and H 2 O. The simple sugars, or monosaccharides,
including pentoses (5 carbons; eg, ribose) and hexoses (6 car-
bons; eg, glucose) perform both structural (eg, as part of nu-
cleotides discussed previously) and functional roles (eg,
inositol 1,4,5 trisphosphate acts as a cellular signaling mole-
cules) in the body. Monosaccharides can be linked together to
form disaccharides (eg, sucrose), or polysaccharides (eg, gly-
cogen). The placement of sugar moieties onto proteins (glyco-
proteins) aids in cellular targeting, and in the case of some
FIGURE 1–19 Involvement of the citric acid cycle in transamination and gluconeogenesis. The bold arrows indicate the main pathway
of gluconeogenesis. Note the many entry positions for groups of amino acids into the citric acid cycle. (Reproduced with permission from Murray RK et al:
Harper’s Biochemistry, 26th ed. McGraw-Hill, 2003.)
TransaminaseTransaminaseTransaminasePhosphoenolpyruvate
carboxykinase
OxaloacetateAspartate
Citrateα-KetoglutarateSuccinyl-CoAFumaratePhosphoenolpyruvateCO 2CO 2Alanine Pyruvate Acetyl-CoAGlutamateHistidine
Proline
Glutamine
ArginineIsoleucine
Methionine
ValineHydroxyproline
Serine
Cysteine
Threonine
GlycineTyrosine
PhenylalaninePropionateGlucoseTryptophanLactateFIGURE 1–20 Urea cycle. The processing of NH 3 to urea for ex-
cretion contains several coordinative steps in both the cytoplasm (Cy-
to) and the mitochondria (Mito). The production of carbamoyl
phosphate and its conversion to citrulline occurs in the mitochondria,
whereas other processes are in the cytoplasm.NH 2 +NH 3 +NH 3 +NH 4 + NH 3H 3 N+ArgininosuccinateH 2 N
C
HNCOO−COO−HC(CH 2 ) 3(CH 2 ) 3
HCO ——NH 3 +H 2 N
CPiHNCOO−HC(CH 2 ) 3——Aspartate FumarateCitrulline+ NO ArginineCarbamoyl
phosphateUreaOrnithineO
NH 2CNH 2
——CytoMito