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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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Detaylı Bibliyografya
Yayımlandı:J Endocr Soc
Asıl Yazarlar: Sakuma, Ikki, Yao, Yue, Fujimoto, Masanori, Nagano, Hidekazu, Yokote, Koutaro, Tanaka, Tomoaki
Materyal Türü: Artigo
Dil:Inglês
Baskı/Yayın Bilgisi: Endocrine Society 2019
Konular:
Online Erişim: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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