62 DAIRY CHEMISTRY AND BIOCHEMISTRY
lack of Gal-1-P : uridyl transferase leads to the accumulation of Gal and
Gal-1-P. The latter interferes with the synthesis of glycoproteins and
glycolipids (important for membranes, e.g. in the brain) and results in
irreversible mental retardation within 2-3 months if the consumption of
galactose-containing foods is continued. The incidence of this disease, often
called 'classical galactosaemia', is about 1 in 60 000.
The ability to metabolize galactose decreases on ageing (after 70 years),
leading to cataract; perhaps this, together with the fact that mammals
normally encounter lactose only while suckling, explains why many people
lose the ability to utilize lactose at the end of childhood.
2.7 Determination of lactose concentration
Lactose may be quantified by methods based on one of five principles:
- polarimetry;
- oxidation-reduction titration;
- colorimetry;
- chromatography;
- enzymatically.
2.7.1 Polarimetry
The specific rotation, [a]?, of lactose in solution at equilibrium is + 55.4"
expressed on an anhydrous basis (+52.6" on a monohydrate basis). The
specific rotation is defined as the optical rotation of a solution containing
1 gml-' in a 1 dm polarimeter tube; it is affected by temperature (20°C is
usually used; indicated by superscript) and wavelength (usually the sodium
D line (589.3 nm) is used; indicated by subscript).
where a is the measured optical rotation; 1, the light path in dm; and c, the
concentration as g m1-I. It is usually expressed as:
where c is in g perlOOml.
The milk sample must first be defatted and deproteinated, usually by
treatment with mercuric nitrate (Hg(NO,),). In calculating the concentra-
tion of lactose, a correction should be used for the concentration of fat and
protein in the precipitate.