Detection of novel visible-light region absorbance peaks in the urine after alkalization in patients with alkaptonuria. [PDF]
BACKGROUND: Alkaptonuria, caused by a deficiency of homogentisate 1,2-dioxygenase, results in the accumulation of homogentisic acid (2,5-dihydroxyphenylacetic acid, HGA) in the urine.
Yasunori Tokuhara +16 more
doaj +1 more source
Alkaptonuria (AKU) is a rare genetic disease associated with the accumulation of homogentisic acid (HGA) and its oxidized/polymerized products in connective tissues up to the deposition of melanin-like pigments (ochronosis).
Braconi, Daniela +7 more
core +5 more sources
Knee osteoarthrosis secondary to ochronosis -clinical case,
Alkaptonuria is a rare metabolic disease in which a deficiency of the enzyme homogentisate dioxygenase causes an accumulation of homogentisic acid. Ochronosis consists of excessive deposition of homogentisic acid in the connective tissue and presents as ...
Andreia Maria da Silva Martins Ferreira +5 more
doaj +1 more source
We conducted our research with the aim of determining whether the “egumi” taste of moso-bamboo shoots differs depending on the production area and how the “egumi” taste of bamboo shoots from Yamagata Prefecture, which is near the northern limit of ...
Yuka Furusawa +2 more
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Determination of Homogentisic Acid in Urine
Abstract A relatively simple method for the quantitative determination of homogentisic acid in urine is presented. Homogentisic acid (2,5-dihydroxyphenylacetic acid) is oxidized by atmospheric oxygen in mild alkali to form 1,4-benzoquinone-2-acetic acid. The latter compound is then conjugated with 2,4-dinitrophenylhydrazine.
R E, Stoner, B, Blivaiss
openaire +2 more sources
Selection and characterization of alanine racemase inhibitors against Aeromonas hydrophila
Background Combining experimental and computational screening methods has been of keen interest in drug discovery. In the present study, we developed an efficient screening method that has been used to screen 2100 small-molecule compounds for alanine ...
Yaping Wang +7 more
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Case Report: Ochronotic Arthropathy
Ochronosis is a rare metabolic disease caused by the deficiency of the homogentisic acid oxidase enzyme. It gives clinical findings related to the accumulation of homogentisic acid in soft tissues and excretion in urine. Patients with chronic arthropathy
Ülkü Dönmez +3 more
doaj +1 more source
Total knee arthroplasty in ochronosis
Alkaptonuria is disorder of tyrosine metabolism due to deficiency of homogentisic oxidase characterized by excretion of homogentisic acid in urine, deposition of oxidized homogensitate pigments in connective tissues and articular cartilages (ochronosis).
Vaibhav G. Patel, MBBS
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Suitability Of Nitisinone In Alkaptonuria 1 (SONIA 1):an international, multicentre, randomised, open-label, no-treatment controlled, parallel-group, dose-response study to investigate the effect of once daily nitisinone on 24-h urinary homogentisic acid excretion in patients with alkaptonuria after 4 weeks of treatment [PDF]
Alkaptonuria (AKU) is a serious genetic disease characterised by premature spondyloarthropathy. Homogentisate-lowering therapy is being investigated for AKU.
Svensson, Lennart +102 more
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Determination of Peroxidase Encapsulated in Liposomes Using Homogentisic Acid .GAMMA.-Lactone Chemiluminescence [PDF]
Homogentisic acid γ-lactone (HAL) chemiluminescence (CL) was applied to the determination of horseradish peroxidase (HRP) encapsulated in liposomes. HRP was detected after the lysis of HRP-trapped liposomes with Triton X-100.
TANI, Hirofumi +3 more
core +1 more source

