ArticleMolecular genetics and metabolism reports2014
Medium-chain triglyceride supplementation under a low-carbohydrate formula is a promising therapy for adult-onset type II citrullinemia.
Article in Molecular genetics and metabolism reports, 2014. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 25 papers.
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Who cites it
25 citing papers in PubMed, 45 citations in OpenAlex.
- Challenges in dietary management: a qualitative study on caregivers of preschool and school-age children with citrin deficiency.Orphanet journal of rare diseases · 2025Article
- ASS1 is a hub gene and possible therapeutic target for regulating metabolic dysfunction-associated steatotic liver disease modulated by a carbohydrate-restricted diet.Molecular diversity · 2025Article
- Review
- Deciphering the Mutational Background in Citrin Deficiency Through a Nationwide Study in Japan and Literature Review.Human mutation · 2025Article
- Clinical landscape of citrin deficiency: A global perspective on a multifaceted condition.Journal of inherited metabolic disease · 2024Review
- The therapeutic landscape of citrin deficiency.Journal of inherited metabolic disease · 2024Review
- [Full-cycle, multidisciplinary and systematic management of citrin deficiency].Zhonghua gan zang bing za zhi = Zhonghua ganzangbing zazhi = Chinese journal of hepatology · 2024Article
- Rare cause of recurrent acute pancreatitis in teenage man.eGastroenterology · 2024Article
- Pathogenesis and Management of Citrin Deficiency.Internal medicine (Tokyo, Japan) · 2024Review
- Adult-onset Type II Citrullinemia Developed under Dietary Restrictions during Imprisonment.Internal medicine (Tokyo, Japan) · 2024Article
- Aspartate-glutamate carrier 2 (citrin): a role in glucose and amino acid metabolism in the liver.BMB reports · 2023Review
- Citrin Deficiency: Clinical and Nutritional Features.Nutrients · 2023Review
- Obstacles to home-based dietary management for caregivers of children with citrin deficiency: a qualitative study.Orphanet journal of rare diseases · 2022Article
- Food Preferences of Patients with Citrin Deficiency.Nutrients · 2021Article
- Lessons for the clinical nephrologist: dietary management of adult-onset type II citrullinemia in chronic kidney disease: a nutritional dilemma.Journal of nephrology · 2020Article
- Successful treatment of adult-onset type II citrullinemia with a low-carbohydrate diet and L-arginine after DNA analysis produced a definitive diagnosis.Clinical journal of gastroenterology · 2020Article
- Hypoketotic hypoglycemia in citrin deficiency: a case report.BMC pediatrics · 2020Article
- AGC2 (Citrin) Deficiency-From Recognition of the Disease till Construction of Therapeutic Procedures.Biomolecules · 2020Review
- Overexpression of the peroxin Pex34p suppresses impaired acetate utilization in yeast lacking the mitochondrial aspartate/glutamate carrier Agc1p.FEMS yeast research · 2019Article
- Bi-allelic GOT2 Mutations Cause a Treatable Malate-Aspartate Shuttle-Related Encephalopathy.American journal of human genetics · 2019Article
Corrections and comments
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Authors and funding
14 authors at 4 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundCitrin, encoded by
methodsAn MCT supplementation therapy under a low-carbohydrate formula was administered to five patients with CTLN2. Four of the patients had episodes of hyperammonemic encephalopathy, and one patient had postprandial hyperammonemia with no symptoms.
resultsOne of the patients displaying hyperammonemic encephalopathy completely recovered with all normal laboratory findings. Others notably improved in terms of clinical and or laboratory findings with no hyperammonemic symptoms; however, the patients displayed persistent mild citrullinemia and occasionally had postprandial mild hyperammonemia most likely due to an irreversible change in the liver.
conclusionsAn MCT supplement can provide energy to hepatocytes and promote hepatic lipogenesis, leading to a reduction in the cytosolic NADH/NAD
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