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2015 ; 24
(ä): 51-66
Nephropedia Template TP
gab.com Text
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English Wikipedia
The Pigment in Alkaptonuria Relationship to Melanin and Other Coloured
Substances: A Review of Metabolism, Composition and Chemical Analysis
#MMPMID26093627
Roberts NB
; Curtis SA
; Milan AM
; Ranganath LR
JIMD Rep
2015[]; 24
(ä): 51-66
PMID26093627
show ga
The pigments found in plants, animals and humic substances are well described and
classified. In humans considerable progress has been made with the main pigment
melanin in defining its biochemistry, the different types and function. However,
analytical techniques to show these differences in vivo are still not readily
available. NMR and IR spectroscopy are relatively insensitive and reveal only
major structural differences. Techniques utilising MS are useful in determining
elemental content but require further studies to optimise conditions for accurate
mass analysis. How the components may be structurally organised seems to be the
most problematic with scanning TEM and the improved FTIR of use in this respect.
As regards understanding the nature of the pigment related to HGA seen in
patients with Alkaptonuria (AKU), it is still thought of as a melaninlike pigment
simply because of its colour and likewise thought to be a polymer of undetermined
size. It is important that detailed analysis be carried out to define more
accurately this pigment. However, observations suggest it to be the same as the
HGA-derived pigment, pyomelanin, produced by bacteria and containing both quinone
and phenolic groups. The interesting developments in alkaptonuria will be to
understand how such a polymer can cause such profound collagen and connective
tissue damage and how best to reverse this process.