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MON-188 Characterization and Categorization Based on Genotype-Biochemical Phenotype Association in Fructose-1,6-Bisphosphatase Deficiency

Fructose-1,6-bisphosphatase (FBPase) deficiency, caused by an FBP1 mutation, is an autosomal recessive disorder characterized by hypoglycemia and metabolic acidosis. Due to the rarity of FBPase deficiency, elucidating the mechanism by which the mutations cause enzyme activity loss is challenging. We...

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Bibliografische gegevens
Gepubliceerd in:J Endocr Soc
Hoofdauteurs: Sakuma, Ikki, Yao, Yue, Fujimoto, Masanori, Nagano, Hidekazu, Yokote, Koutaro, Tanaka, Tomoaki
Formaat: Artigo
Taal:Inglês
Gepubliceerd in: Endocrine Society 2019
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Online toegang:https://ncbi.nlm.nih.gov/pmc/articles/PMC6551122/
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1210/js.2019-MON-188
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