Homogentisate 1,2-dioxygenase (homogentisic acid oxidase, homogentisate oxidase, homogentisicase) is an enzyme which catalyzes the conversion of homogentisate to 4-maleylacetoacetate. (wikipedia.org)
Homogentisate 1,2-dioxygenase or HGD is involved in the catabolism of aromatic rings, more specifically in the breakdown of the amino acids tyrosine and phenylalanine. (wikipedia.org)
homogentisate 4-maleylacetoacetate The active site of Homogentisate 1,2-dioxygenase was determined through the crystal structure, which was captured through the work of Titus et al. (wikipedia.org)
ES-MS spectra of tryptic digested SDS-PAGE band and bioinformatics studies revealed that C12O share 81% homology to homogentisate 1,2-dioxygenase reported in other Pseudomonas chlororaphis strains. (preprints.org)
A deficiency of the hepatic enzyme homogentisate 1,2-dioxygenase (HGO) forces the accumulation of homogentisic acid, which is rapidly cleared in the kidney and excreted. (medscape.com)
Alkaptonuria (AKU) is a rare autosomal recessive condition with a prevalence of 1 in 250 000-1 000 000 1 caused by the deficiency of homogentisate 1,2 dioxygenase, the enzyme responsible for breaking down homogentisic acid (HGA). (bmj.com)
A (p.Gly361Arg), of the homogentisate 1,2-dioxygenase (HGD) gene. (omia.org)
Alkaptonuria (aku), an inborn error of metabolism caused by the loss of homogentisate 1,2-dioxygenase (HGD), has been described in a mouse model created by ethylnitrosourea mutagenesis but the mutation in these mice has not previously been identified. (elsevierpure.com)
Deficiency of homogentisate 1,2-dioxygenase leads to increased homogentisic acid which is deposited as ochronotic pigment. (qxmd.com)
The mutation causes defects in the homogentisate 1,2-dioxygenase enzyme which hampers the breakdown of tyrosine. (hxbenefit.com)
Alkaptonuria is a rare inherited disorder in which a deficiency of an enzyme called homogentisate 1,2 dioxygenase - HGD, results in the accumulation of homogentisic acid in connective tissues . (osmosis.org)
Similar to Homogentisate 1,2-dioxygenase (EC 1.13.11.5) (Homogentisicase) (Homogentisate oxygenase) (Homogentisic acid oxidase). (go.jp)
Identification of 11 Novel Homogentisate 1,2 Dioxygenase Variants in Alkaptonuria Patients and Establishment of a Novel LOVD-Based HGD Mutation Database. (medscape.com)
Homogentisate 1,2-dioxygenase (HGD) gene variants, their analysis and genotype-phenotype correlations in the largest cohort of patients with AKU. (cdc.gov)
Enzyme called homogentisate1
The HGD gene provides instructions for making an enzyme called homogentisate oxidase. (medlineplus.gov)
Conversion of homogentisate1
The mild antioxidant nature of ascorbic acid helps to retard the process of conversion of homogentisate to the polymeric material that is deposited in cartilaginous tissues. (medscape.com)
Oxidase1
The HGD gene controls the instruction for making the homogentisate Oxidase enzyme. (hxbenefit.com)
Pathway2
HGD appears in the metabolic pathway of tyrosine and phenylalanine degradation once the molecule homogentisate is produced. (wikipedia.org)
Homogentisate reacts with HGD to produce maleylacetoacetate, which then is further used in the metabolic pathway. (wikipedia.org)
Bestellen1
Homogentisate, 'bestellen metformax dianorm almere glucophage' residually even though heterovaccine cemented of whose pseudosocial Locilex. (blindman.be)
Precursor2
4-Hydroxyphenylpyruvate, a homogentisate precursor, was found to be upregulated by 26% (p = 7.20E-3). (bvsalud.org)
While tocochromanol synthesis occurs in plastids, converging genetic data in Arabidopsis and soybean demonstrate that the synthesis of the polar precursor homogentisate is located in the cytoplasm. (unifr.ch)
Metabolic1
Since the availability of both homogentisate and polyprenyl pyrophosphates are currently accepted as major mechanisms controlling the type and amount of tocochromanols produced in plant tissues, we summarized our current knowledge and research gaps concerning the biosynthesis, metabolic origins and transport of tocochromanol biosynthetic precursors in plant cells. (unifr.ch)
Process1
The opening of the aromatic ring in homogentisate is a multi-step process. (wikipedia.org)
Disorder1
This disorder is due to the inability of the body to deal with homogentisate, which when oxidized by the body will produce the compound known as the ochronotic pigment, which causes a black color, and has several negative effects. (wikipedia.org)
Active1
Homogentisate binds in the active site to Glu341, His335, and His371 via the Fe2+ atom. (wikipedia.org)
Homogentisic Acid2
A deficiency of the hepatic enzyme homogentisate 1,2-dioxygenase (HGO) forces the accumulation of homogentisic acid, which is rapidly cleared in the kidney and excreted. (medscape.com)
In our body, the dis-ease, Alkaptonuria is caused by a mutation in the HGD gene which encodes for the enzyme homogentisate 1,2-dioxygenase, characterized by accumulation of homogentisic acid (a brown pigment) in the urine , ochronosis in various tissues, and arthritis . (wellnessadvantage.com)
Catalyzes the conversion2
A mononuclear Fe(II)-dependent oxygenase, this enzyme catalyzes the conversion of homogentisate to 4-maleylacetoacetate, the third step in the pathway for the catabolism of TYROSINE. (umassmed.edu)
The encoded protein catalyzes the conversion of 4-hydroxyphenylpyruvate to homogentisate. (nih.gov)
Tyrosine1
HGD appears in the metabolic pathway of tyrosine and phenylalanine degradation once the molecule homogentisate is produced. (wikipedia.org)
Maleylacetoacetate1
Homogentisate reacts with HGD to produce maleylacetoacetate, which then is further used in the metabolic pathway. (wikipedia.org)
Pigment2
This disorder is due to the inability of the body to deal with homogentisate, which when oxidized by the body will produce the compound known as the ochronotic pigment, which causes a black color, and has several negative effects. (wikipedia.org)
This pigment can be an oxidation item of homogentisate [27], [29]. (thetechnoant.info)
Conversion1
The mild antioxidant nature of ascorbic acid helps to retard the process of conversion of homogentisate to the polymeric material that is deposited in cartilaginous tissues. (medscape.com)
Active site1
Homogentisate binds in the active site to Glu341, His335, and His371 via the Fe2+ atom. (wikipedia.org)
Therapy1
Homogentisate-lowering therapy is being investigated for AKU. (uea.ac.uk)