ReviewJournal of inherited metabolic disease2009
Animal models of maple syrup urine disease.
Review in Journal of inherited metabolic disease, 2009. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 21 papers.
What it found
Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.
The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.
Who cites it
21 citing papers in PubMed, 39 citations in OpenAlex.
- Metabolic pathways at the crossroads of diabetes and inborn errors.Journal of inherited metabolic disease · 2018Review
- Neuroprotective Effect of Creatine and Pyruvate on Enzyme Activities of Phosphoryl Transfer Network and Oxidative Stress Alterations Caused by Leucine Administration in Wistar Rats.Neurotoxicity research · 2017Article
- [Maple syrup urine disease and gene mutations in twin neonates].Zhongguo dang dai er ke za zhi = Chinese journal of contemporary pediatrics · 2016Article
- Metformin inhibits Branched Chain Amino Acid (BCAA) derived ketoacidosis and promotes metabolic homeostasis in MSUD.Scientific reports · 2016Article
- Chemically induced acute model of sarcosinemia in wistar rats.Metabolic brain disease · 2016Article
- Multilayered genetic and omics dissection of mitochondrial activity in a mouse reference population.Cell · 2014Article
- Co-administration of creatine plus pyruvate prevents the effects of phenylalanine administration to female rats during pregnancy and lactation on enzymes activity of energy metabolism in cerebral cortex and hippocampus of the offspring.Neurochemical research · 2014Article
- Effects of a co-treatment with pyruvate and creatine on dendritic spines in rat hippocampus and posterodorsal medial amygdala in a phenylketonuria animal model.Metabolic brain disease · 2013Article
- Adipose transplant for inborn errors of branched chain amino acid metabolism in mice.Molecular genetics and metabolism · 2013Article
- Effects of β-alanine administration on selected parameters of oxidative stress and phosphoryltransfer network in cerebral cortex and cerebellum of rats.Molecular and cellular biochemistry · 2013Article
- Effect of leucine administration to female rats during pregnancy and lactation on oxidative stress and enzymes activities of phosphoryltransfer network in cerebral cortex and hippocampus of the offspring.Neurochemical research · 2013Article
- Placental stem cell correction of murine intermediate maple syrup urine disease.Hepatology (Baltimore, Md.) · 2013Article
- Effect of histidine administration to female rats during pregnancy and lactation on enzymes activity of phosphoryltransfer network in cerebral cortex and hippocampus of the offspring.Metabolic brain disease · 2012Article
- Maple syrup urine disease: new insights from a zebrafish model.Disease models & mechanisms · 2012Article
- Administration of histidine to female rats induces changes in oxidative status in cortex and hippocampus of the offspring.Neurochemical research · 2012Article
- Branched-chain amino acid metabolism in heart disease: an epiphenomenon or a real culprit?Cardiovascular research · 2011Review
- Article
- BCATm deficiency ameliorates endotoxin-induced decrease in muscle protein synthesis and improves survival in septic mice.American journal of physiology. Regulatory, integrative and comparative physiology · 2010Article
- Metabolic manipulation of glycosylation disorders in humans and animal models.Seminars in cell & developmental biology · 2010Review
- Alcohol-induced IGF-I resistance is ameliorated in mice deficient for mitochondrial branched-chain aminotransferase.The Journal of nutrition · 2010Article
Corrections and comments
PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.
Authors and funding
1 author at 1 institution in 1 country.
Funding
Abstract
Maple syrup urine disease (MSUD) is an inherited aminoacidopathy resulting from dysfunction of the branched-chain keto acid dehydrogenase (BCKDH) complex. This disease is currently treated primarily by dietary restriction of branched-chain amino acids (BCAAs). However, dietary compliance is often challenging. Conversely, liver transplantation significantly improves outcomes, but donor organs are scarce and there are high costs and potential risks associated with this invasive procedure. Therefore, improved treatment options for MSUD are needed. Development of novel treatments could be facilitated by animal models that accurately mimic the human disease. Animal models provide a useful system in which to explore disease mechanisms and new preclinical therapies. Here we review MSUD and currently available animal models and their corresponding relevance to the human disorder. Using animal models to gain a more complete understanding of the pathophysiology behind the human disease may lead to new or improved therapies to treat or potentially cure the disorder.
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What OpenQuestion holds
Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.