Homogentisic acid (2,5-dihydroxyphenylacetic acid) is a
phenolic acid
Phenolic acids or phenolcarboxylic acids are types of aromatic acid compounds. Included in that class are substances containing a phenolic ring and an organic carboxylic acid function (C6-C1 skeleton). Two important naturally occurring types of ph ...
usually found in ''
Arbutus unedo
''Arbutus unedo'' is an evergreen shrub or small tree in the family Ericaceae, native to the Mediterranean region and western Europe. The tree is well known for its fruits, which bear some resemblance to the strawberry — hence the common na ...
'' (strawberry-tree) honey. It is also present in the bacterial plant pathogen
''Xanthomonas campestris'' pv. ''phaseoli'' as well as in the yeast ''
Yarrowia lipolytica
''Yarrowia'' is a fungal genus in the family Dipodascaceae. For a while the genus was monotypic, containing the single species ''Yarrowia lipolytica'', a yeast that can use unusual carbon sources, such as hydrocarbons. This has made it of interes ...
'' where it is associated with the production of brown pigments. It is oxidatively
dimerised to form
hipposudoric acid, one of the main constituents of the 'blood sweat' of
hippopotamus
The hippopotamus ( ; : hippopotamuses or hippopotami; ''Hippopotamus amphibius''), also called the hippo, common hippopotamus, or river hippopotamus, is a large semiaquatic mammal native to sub-Saharan Africa. It is one of only two extan ...
es.
It is less commonly known as melanic acid, the name chosen by
William Prout
William Prout FRS (; 15 January 1785 – 9 April 1850) was an English chemist, physician, and natural theologian. He is remembered today mainly for what is called Prout's hypothesis.
Biography
Prout was born in Horton, Gloucestershire in ...
.
Human pathology
Accumulation of excess homogentisic acid and its oxide, named alkapton, is a result of the failure of the enzyme
homogentisic acid 1,2-dioxygenase (typically due to a mutation) in the degradative pathway of
tyrosine
-Tyrosine or tyrosine (symbol Tyr or Y) or 4-hydroxyphenylalanine is one of the 20 standard amino acids that are used by cells to synthesize proteins. It is a non-essential amino acid with a polar side group. The word "tyrosine" is from the G ...
, consequently associated with
alkaptonuria
Alkaptonuria is a rare inherited genetic disease which is caused by a mutation in the ''HGD'' gene for the enzyme homogentisate 1,2-dioxygenase (); if a person inherits an abnormal copy from both parents (it is a recessive condition), the body ac ...
.
Intermediate
It is an
intermediate in the
catabolism of aromatic amino acids such as
phenylalanine and
tyrosine
-Tyrosine or tyrosine (symbol Tyr or Y) or 4-hydroxyphenylalanine is one of the 20 standard amino acids that are used by cells to synthesize proteins. It is a non-essential amino acid with a polar side group. The word "tyrosine" is from the G ...
.
4-Hydroxyphenylpyruvate
4-Hydroxyphenylpyruvic acid (4-HPPA) is an intermediate in the metabolism of the amino acid phenylalanine. The aromatic side chain of phenylalanine is hydroxylated by the enzyme phenylalanine hydroxylase to form tyrosine. The conversion from t ...
(produced by
transamination
Transamination is a chemical reaction that transfers an amino group to a ketoacid to form new amino acids. This pathway is responsible for the deamination of most amino acids. This is one of the major degradation pathways which convert essential ...
of tyrosine) is acted upon by the enzyme
4-hydroxyphenylpyruvate dioxygenase
4-Hydroxyphenylpyruvate dioxygenase (HPPD), also known as α-ketoisocaproate dioxygenase (KIC dioxygenase), is an Fe(II)-containing non-heme oxygenase that catalyzes the second reaction in the catabolism of tyrosine - the conversion of 4-hydro ...
to yield homogentisate. If active and present, the enzyme
homogentisate 1,2-dioxygenase
Homogentisate 1,2-dioxygenase (homogentisic acid oxidase, homogentisate oxidase, homogentisicase) is an enzyme which catalyzes the conversion of homogentisate to 4-maleylacetoacetate. Homogentisate 1,2-dioxygenase or HGD is involved in the cat ...
further degrades homogentisic acid to yield
4-maleylacetoacetic acid
4-Maleylacetoacetate (4-maleylacetoacetatic acid) is an intermediate in the metabolism of tyrosine. It is converted to fumarylacetoacetate by the enzyme 4-maleylacetoacetate cis-trans-isomerase. Gluthathione coenzymatically helps in conversion ...
.
References
{{DEFAULTSORT:Homogentisic Acid
Hydroquinones
Acetic acids
Hydroxy acids