Valine metabolites analysis in ECHS1 deficiency Article Swipe
Mari Kuwajima
,
Karin Kojima
,
Hitoshi Osaka
,
Yusuke Hamada
,
Eriko F. Jimbo
,
Miyuki Watanabe
,
Shiho Aoki
,
Ikuko Sato‐Shirai
,
Keiko Ichimoto
,
Takuya Fushimi
,
Kei Murayama
,
Akira Ohtake
,
Masakazu Kohda
,
Yoshihito Kishita
,
Yukiko Yatsuka
,
Shumpei Uchino
,
Masakazu Mimaki
,
Noriko Miyake
,
Naomichi Matsumoto
,
Yasushi Okazaki
,
Tomomi Ogata
,
Takanori Yamagata
,
Kazuhiro Muramatsu
·
YOU?
·
· 2021
· Open Access
·
· DOI: https://doi.org/10.1016/j.ymgmr.2021.100809
YOU?
·
· 2021
· Open Access
·
· DOI: https://doi.org/10.1016/j.ymgmr.2021.100809
Short-chain enoyl-CoA hydratase (ECHS1) is involved in amino acid and fatty acid catabolism in mitochondria and its deficiency causes Leigh syndrome or exercise-induced dystonia. More than 60 patients with this condition have been reported till date. The accumulation of intermediate metabolites of valine is assumed to be responsible for the cytotoxicity. Since protein restriction, including valine reportedly improves neurological symptoms, it is essential to consider the possible incidence of and diagnose ECHS1 syndrome in the earlier stages. This study reported the liquid chromatography with tandem mass spectrometry (LC-MS/MS) urine and plasma metabolite analysis in six cases, including four new cases with ECHS1 deficiency. The values of urine cysteine/cysteamine conjugates from valine metabolites, S-(2-carboxypropyl) cysteine/cysteamine from methacrylyl-CoA, and S-(2-carboxyethyl) cysteine/cysteamine from acryloyl-CoA were separated between six patients and six normal controls. The LC-MS/MS analysis revealed that these metabolites can be used for the early diagnosis and evaluation of diet therapy.
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Metadata
- Type
- article
- Language
- en
- Landing Page
- https://doi.org/10.1016/j.ymgmr.2021.100809
- OA Status
- gold
- Cited By
- 22
- References
- 21
- Related Works
- 10
- OpenAlex ID
- https://openalex.org/W3206667607
All OpenAlex metadata
Raw OpenAlex JSON
- OpenAlex ID
-
https://openalex.org/W3206667607Canonical identifier for this work in OpenAlex
- DOI
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https://doi.org/10.1016/j.ymgmr.2021.100809Digital Object Identifier
- Title
-
Valine metabolites analysis in ECHS1 deficiencyWork title
- Type
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articleOpenAlex work type
- Language
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enPrimary language
- Publication year
-
2021Year of publication
- Publication date
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2021-10-12Full publication date if available
- Authors
-
Mari Kuwajima, Karin Kojima, Hitoshi Osaka, Yusuke Hamada, Eriko F. Jimbo, Miyuki Watanabe, Shiho Aoki, Ikuko Sato‐Shirai, Keiko Ichimoto, Takuya Fushimi, Kei Murayama, Akira Ohtake, Masakazu Kohda, Yoshihito Kishita, Yukiko Yatsuka, Shumpei Uchino, Masakazu Mimaki, Noriko Miyake, Naomichi Matsumoto, Yasushi Okazaki, Tomomi Ogata, Takanori Yamagata, Kazuhiro MuramatsuList of authors in order
- Landing page
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https://doi.org/10.1016/j.ymgmr.2021.100809Publisher landing page
- Open access
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YesWhether a free full text is available
- OA status
-
goldOpen access status per OpenAlex
- OA URL
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https://doi.org/10.1016/j.ymgmr.2021.100809Direct OA link when available
- Concepts
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Cysteamine, Valine, Internal medicine, Endocrinology, Urine, Metabolite, Catabolism, Leucine, Cysteine, Chemistry, Medicine, Biochemistry, Amino acid, Metabolism, EnzymeTop concepts (fields/topics) attached by OpenAlex
- Cited by
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22Total citation count in OpenAlex
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2025: 6, 2024: 6, 2023: 4, 2022: 5, 2021: 1Per-year citation counts (last 5 years)
- References (count)
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21Number of works referenced by this work
- Related works (count)
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10Other works algorithmically related by OpenAlex
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