FRUCTOSE INTOLERANCE
\fɹˈʌktə͡ʊz ɪntˈɒləɹəns], \fɹˈʌktəʊz ɪntˈɒləɹəns], \f_ɹ_ˈʌ_k_t_əʊ_z ɪ_n_t_ˈɒ_l_ə_ɹ_ə_n_s]\
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An autosomal recessive fructose metabolism disorder due to deficient fructose-1-phosphate aldolase (EC 2.1.2.13) activity, resulting in accumulation of fructose-1-phosphate. The accumulated fructose-1-phosphate inhibits glycogenolysis and gluconeogenesis, causing severe hypoglycemia following ingestion of fructose. Prolonged fructose ingestion in infants leads ultimately to hepatic failure and death. Patients develop a strong distaste for sweet food, and avoid a chronic course of the disease by remaining on a fructose- and sucrose-free diet.
By DataStellar Co., Ltd
Nearby Words
- fructose biphosphatase deficiencies
- fructose biphosphatase deficiency
- fructose biphosphate aldolase
- fructose bisphosphatase
- fructose bisphosphate aldolase
- Fructose Intolerance
- fructose intolerances
- fructose metabolism, inborn errors
- fructosediphosphatase
- fructosediphosphatase deficiencies
- fructosediphosphatase deficiency