We are analyzing https://link.springer.com/article/10.1007/s10545-005-0114-x.

Title:
Kinetic characterization of human hydroxyacid–oxoacid transhydrogenase: Relevance toD-2-hydroxyglutaric and γ-hydroxybutyric acidurias | Journal of Inherited Metabolic Disease
Description:
We investigated the presence of hydroxyacid–oxoacid transhydrogenase (HOT), which catalyses the cofactor-independent conversion of γ-hydroxybutyrate (GHB) to succinic semialdehyde coupled to reduction of 2-ketoglutarate (2-KG) to D-2-hydroxyglutarate (D-2-HG), in human liver extracts employing [2H6]GHB and 2-KG as substrates. We measured incorporation of 2H in D-[2H]2-HG using GC-MS analyses, providing evidence for HOT activity in humans. Kinetic characterization of HOT was undertaken in forward and reverse directions. We employed [2H6]GHB and [2H4]2-KG as cosubstrates in order to develop a HOT activity assay in cultured human fibroblasts derived from patients with D-2-hydroxyglutaric aciduria. HOT activity was quantified in this system by the measurement of D-[2H5]2-HG production. Fibroblasts derived from patients with D-2-hydroxyglutaric aciduria showed normal HOT activities. Our results provide the first demonstration and preliminary kinetic characterization of HOT activity in human tissues.
Website Age:
28 years and 1 months (reg. 1997-05-29).

Matching Content Categories {📚}

Education
Science
Non-Profit & Charity

Content Management System {📝}

What CMS is link.springer.com built with?

Custom-built

No common CMS systems were detected on Link.springer.com, and no known web development framework was identified.

Traffic Estimate {📈}

What is the average monthly size of link.springer.com audience?

🌠 Phenomenal Traffic: 5M - 10M visitors per month

Based on our best estimate, this website will receive around 5,000,019 visitors per month in the current month.
However, some sources were not loaded, we suggest to reload the page to get complete results.

check SE Ranking
check Ahrefs
check Similarweb
check Ubersuggest
check Semrush

How Does Link.springer.com Make Money? {💸}

We can't figure out the monetization strategy.

Earning money isn't the goal of every website; some are designed to offer support or promote social causes. People have different reasons for creating websites. This might be one such reason. Link.springer.com might have a hidden revenue stream, but it's not something we can detect.

Keywords {🔍}

article, aciduria, cas, google, scholar, dhydroxyglutaric, human, hot, struys, pubmed, privacy, cookies, content, journal, characterization, jakobs, activity, access, publish, search, metabolic, disease, kinetic, hydroxyacidoxoacid, transhydrogenase, verhoeven, ten, brink, gibson, assay, fibroblasts, patients, data, information, log, research, discover, metabolism, medical, inherit, metab, dis, lhydroxyglutaric, kaufman, nelson, chem, clinical, download, author, springer,

Topics {✒️}

gamma-aminobutyric acid-transaminase deficiency month download article/chapter inherited metabolic disease hot activity assay human hydroxyacid–oxoacid transhydrogenase d-[2h5]2-hg production d-2-hydroxyglutarate dehydrogenase gene γ-hydroxybutyric acidurias published direct nonisotopic assay d-2-hydroxyglutaric aciduria carrying gc-ms analyses disease-related metabolites neurotransmitter metabolism full article pdf privacy choices/manage cookies employed [2h6]ghb hydroxyacid–oxoacid transhydrogenase hydroxyacid-oxoacid transhydrogenase l-2-hydroxyglutaric acid clinical chemistry related subjects cofactor-independent conversion biochemical marker clinical disease entity γ-hydroxybutyric acidurias human d-2-hgdh d-2-hydroxyglutaric aciduria l-2-hydroxyglutaric aciduria relevance tod-2-hydroxyglutaric l-2-hydroxyglutaric acidemias preliminary kinetic characterization european economic area reverse directions 3-methylglutaconyl-coa hydratase developmental time courses conditions privacy policy medical genetics kaufman ee check access instant access oxidize γ-hydroxybutyrate accepting optional cookies d-[2h]2-hg succinic semialdehyde coupled rat kidney mitochondria article struys article journal journal finder publish main content log craigen

Questions {❓}

Gibson KM, Craigen W, Herman GE, et al (1993a) D-2-Hydroxyglutaric aciduria in a newborn with neurological abnormalities: a new neurometabolic disorder?
Van der Knaap MS, Jakobs C, Hoffmann GF, et al (1999a) D-2-Hydroxyglutaric aciduria: biochemical marker or clinical disease entity?

Schema {🗺️}

WebPage:
      mainEntity:
         headline:Kinetic characterization of human hydroxyacid–oxoacid transhydrogenase: Relevance toD-2-hydroxyglutaric and γ-hydroxybutyric acidurias
         description:We investigated the presence of hydroxyacid–oxoacid transhydrogenase (HOT), which catalyses the cofactor-independent conversion of γ-hydroxybutyrate (GHB) to succinic semialdehyde coupled to reduction of 2-ketoglutarate (2-KG) to D-2-hydroxyglutarate (D-2-HG), in human liver extracts employing [2H6]GHB and 2-KG as substrates. We measured incorporation of 2H in D-[2H]2-HG using GC-MS analyses, providing evidence for HOT activity in humans. Kinetic characterization of HOT was undertaken in forward and reverse directions. We employed [2H6]GHB and [2H4]2-KG as cosubstrates in order to develop a HOT activity assay in cultured human fibroblasts derived from patients with D-2-hydroxyglutaric aciduria. HOT activity was quantified in this system by the measurement of D-[2H5]2-HG production. Fibroblasts derived from patients with D-2-hydroxyglutaric aciduria showed normal HOT activities. Our results provide the first demonstration and preliminary kinetic characterization of HOT activity in human tissues.
         datePublished:
         dateModified:
         pageStart:921
         pageEnd:930
         sameAs:https://doi.org/10.1007/s10545-005-0114-x
         keywords:
            Public Health
            Internal Medicine
            Reverse Direction
            Metabolic Disease
            Activity Assay
            Metabolic Diseases
            Human Genetics
            Pediatrics
            Biochemistry
            general
         image:
         isPartOf:
            name:Journal of Inherited Metabolic Disease
            issn:
               1573-2665
               0141-8955
            volumeNumber:28
            type:
               Periodical
               PublicationVolume
         publisher:
            name:Kluwer Academic Publishers
            logo:
               url:https://www.springernature.com/app-sn/public/images/logo-springernature.png
               type:ImageObject
            type:Organization
         author:
               name:E. A. Struys
               affiliation:
                     name:VU University Medical Center
                     address:
                        name:Metabolic Unit, Department of Clinical Chemistry, VU University Medical Center, HV Amsterdam, The Netherlands
                        type:PostalAddress
                     type:Organization
               email:[email protected]
               type:Person
               name:N. M. Verhoeven
               affiliation:
                     name:VU University Medical Center
                     address:
                        name:Metabolic Unit, Department of Clinical Chemistry, VU University Medical Center, HV Amsterdam, The Netherlands
                        type:PostalAddress
                     type:Organization
               type:Person
               name:H. J. Ten Brink
               affiliation:
                     name:VU University Medical Center
                     address:
                        name:Metabolic Unit, Department of Clinical Chemistry, VU University Medical Center, HV Amsterdam, The Netherlands
                        type:PostalAddress
                     type:Organization
               type:Person
               name:W. V. Wickenhagen
               affiliation:
                     name:VU University Medical Center
                     address:
                        name:Metabolic Unit, Department of Clinical Chemistry, VU University Medical Center, HV Amsterdam, The Netherlands
                        type:PostalAddress
                     type:Organization
               type:Person
               name:K. M. Gibson
               affiliation:
                     name:Oregon Health & Science University
                     address:
                        name:Molecular and Medical Genetics, Oregon Health & Science University, Portland, USA
                        type:PostalAddress
                     type:Organization
               type:Person
               name:C. Jakobs
               affiliation:
                     name:VU University Medical Center
                     address:
                        name:Metabolic Unit, Department of Clinical Chemistry, VU University Medical Center, HV Amsterdam, The Netherlands
                        type:PostalAddress
                     type:Organization
               type:Person
         isAccessibleForFree:
         hasPart:
            isAccessibleForFree:
            cssSelector:.main-content
            type:WebPageElement
         type:ScholarlyArticle
      context:https://schema.org
ScholarlyArticle:
      headline:Kinetic characterization of human hydroxyacid–oxoacid transhydrogenase: Relevance toD-2-hydroxyglutaric and γ-hydroxybutyric acidurias
      description:We investigated the presence of hydroxyacid–oxoacid transhydrogenase (HOT), which catalyses the cofactor-independent conversion of γ-hydroxybutyrate (GHB) to succinic semialdehyde coupled to reduction of 2-ketoglutarate (2-KG) to D-2-hydroxyglutarate (D-2-HG), in human liver extracts employing [2H6]GHB and 2-KG as substrates. We measured incorporation of 2H in D-[2H]2-HG using GC-MS analyses, providing evidence for HOT activity in humans. Kinetic characterization of HOT was undertaken in forward and reverse directions. We employed [2H6]GHB and [2H4]2-KG as cosubstrates in order to develop a HOT activity assay in cultured human fibroblasts derived from patients with D-2-hydroxyglutaric aciduria. HOT activity was quantified in this system by the measurement of D-[2H5]2-HG production. Fibroblasts derived from patients with D-2-hydroxyglutaric aciduria showed normal HOT activities. Our results provide the first demonstration and preliminary kinetic characterization of HOT activity in human tissues.
      datePublished:
      dateModified:
      pageStart:921
      pageEnd:930
      sameAs:https://doi.org/10.1007/s10545-005-0114-x
      keywords:
         Public Health
         Internal Medicine
         Reverse Direction
         Metabolic Disease
         Activity Assay
         Metabolic Diseases
         Human Genetics
         Pediatrics
         Biochemistry
         general
      image:
      isPartOf:
         name:Journal of Inherited Metabolic Disease
         issn:
            1573-2665
            0141-8955
         volumeNumber:28
         type:
            Periodical
            PublicationVolume
      publisher:
         name:Kluwer Academic Publishers
         logo:
            url:https://www.springernature.com/app-sn/public/images/logo-springernature.png
            type:ImageObject
         type:Organization
      author:
            name:E. A. Struys
            affiliation:
                  name:VU University Medical Center
                  address:
                     name:Metabolic Unit, Department of Clinical Chemistry, VU University Medical Center, HV Amsterdam, The Netherlands
                     type:PostalAddress
                  type:Organization
            email:[email protected]
            type:Person
            name:N. M. Verhoeven
            affiliation:
                  name:VU University Medical Center
                  address:
                     name:Metabolic Unit, Department of Clinical Chemistry, VU University Medical Center, HV Amsterdam, The Netherlands
                     type:PostalAddress
                  type:Organization
            type:Person
            name:H. J. Ten Brink
            affiliation:
                  name:VU University Medical Center
                  address:
                     name:Metabolic Unit, Department of Clinical Chemistry, VU University Medical Center, HV Amsterdam, The Netherlands
                     type:PostalAddress
                  type:Organization
            type:Person
            name:W. V. Wickenhagen
            affiliation:
                  name:VU University Medical Center
                  address:
                     name:Metabolic Unit, Department of Clinical Chemistry, VU University Medical Center, HV Amsterdam, The Netherlands
                     type:PostalAddress
                  type:Organization
            type:Person
            name:K. M. Gibson
            affiliation:
                  name:Oregon Health & Science University
                  address:
                     name:Molecular and Medical Genetics, Oregon Health & Science University, Portland, USA
                     type:PostalAddress
                  type:Organization
            type:Person
            name:C. Jakobs
            affiliation:
                  name:VU University Medical Center
                  address:
                     name:Metabolic Unit, Department of Clinical Chemistry, VU University Medical Center, HV Amsterdam, The Netherlands
                     type:PostalAddress
                  type:Organization
            type:Person
      isAccessibleForFree:
      hasPart:
         isAccessibleForFree:
         cssSelector:.main-content
         type:WebPageElement
["Periodical","PublicationVolume"]:
      name:Journal of Inherited Metabolic Disease
      issn:
         1573-2665
         0141-8955
      volumeNumber:28
Organization:
      name:Kluwer Academic Publishers
      logo:
         url:https://www.springernature.com/app-sn/public/images/logo-springernature.png
         type:ImageObject
      name:VU University Medical Center
      address:
         name:Metabolic Unit, Department of Clinical Chemistry, VU University Medical Center, HV Amsterdam, The Netherlands
         type:PostalAddress
      name:VU University Medical Center
      address:
         name:Metabolic Unit, Department of Clinical Chemistry, VU University Medical Center, HV Amsterdam, The Netherlands
         type:PostalAddress
      name:VU University Medical Center
      address:
         name:Metabolic Unit, Department of Clinical Chemistry, VU University Medical Center, HV Amsterdam, The Netherlands
         type:PostalAddress
      name:VU University Medical Center
      address:
         name:Metabolic Unit, Department of Clinical Chemistry, VU University Medical Center, HV Amsterdam, The Netherlands
         type:PostalAddress
      name:Oregon Health & Science University
      address:
         name:Molecular and Medical Genetics, Oregon Health & Science University, Portland, USA
         type:PostalAddress
      name:VU University Medical Center
      address:
         name:Metabolic Unit, Department of Clinical Chemistry, VU University Medical Center, HV Amsterdam, The Netherlands
         type:PostalAddress
ImageObject:
      url:https://www.springernature.com/app-sn/public/images/logo-springernature.png
Person:
      name:E. A. Struys
      affiliation:
            name:VU University Medical Center
            address:
               name:Metabolic Unit, Department of Clinical Chemistry, VU University Medical Center, HV Amsterdam, The Netherlands
               type:PostalAddress
            type:Organization
      email:[email protected]
      name:N. M. Verhoeven
      affiliation:
            name:VU University Medical Center
            address:
               name:Metabolic Unit, Department of Clinical Chemistry, VU University Medical Center, HV Amsterdam, The Netherlands
               type:PostalAddress
            type:Organization
      name:H. J. Ten Brink
      affiliation:
            name:VU University Medical Center
            address:
               name:Metabolic Unit, Department of Clinical Chemistry, VU University Medical Center, HV Amsterdam, The Netherlands
               type:PostalAddress
            type:Organization
      name:W. V. Wickenhagen
      affiliation:
            name:VU University Medical Center
            address:
               name:Metabolic Unit, Department of Clinical Chemistry, VU University Medical Center, HV Amsterdam, The Netherlands
               type:PostalAddress
            type:Organization
      name:K. M. Gibson
      affiliation:
            name:Oregon Health & Science University
            address:
               name:Molecular and Medical Genetics, Oregon Health & Science University, Portland, USA
               type:PostalAddress
            type:Organization
      name:C. Jakobs
      affiliation:
            name:VU University Medical Center
            address:
               name:Metabolic Unit, Department of Clinical Chemistry, VU University Medical Center, HV Amsterdam, The Netherlands
               type:PostalAddress
            type:Organization
PostalAddress:
      name:Metabolic Unit, Department of Clinical Chemistry, VU University Medical Center, HV Amsterdam, The Netherlands
      name:Metabolic Unit, Department of Clinical Chemistry, VU University Medical Center, HV Amsterdam, The Netherlands
      name:Metabolic Unit, Department of Clinical Chemistry, VU University Medical Center, HV Amsterdam, The Netherlands
      name:Metabolic Unit, Department of Clinical Chemistry, VU University Medical Center, HV Amsterdam, The Netherlands
      name:Molecular and Medical Genetics, Oregon Health & Science University, Portland, USA
      name:Metabolic Unit, Department of Clinical Chemistry, VU University Medical Center, HV Amsterdam, The Netherlands
WebPageElement:
      isAccessibleForFree:
      cssSelector:.main-content

External Links {🔗}(57)

Analytics and Tracking {📊}

Google Tag Manager

Libraries {📚}

Clipboard.js
Prism.js

CDN Services {📦}

Crossref

5.23s.

LINK . SPRINGER . COM {}