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Congenital Bile Acid Synthesis Defect Type III
smpdb.ca/view/SMP0000318DiseaseSymptoms include severe cholestasis, cirrhosis, and liver failure. … Congenital Bile Acid Synthesis Defect Type III (CBASIII) is caused by a defect in 25-hydroxycholesterol 7-alpha-hydroxylase, which plays a role in synthesis of bile acids.
Glycogenosis, Type III. Cori Disease, Debrancher Glycogenosis
smpdb.ca/view/SMP0000553DiseaseGSD III is divided into the types IIIa, IIIb, IIIc, and IIId, which are distinguished by their pattern of signs and symptoms. GSD types IIIa and IIIc affect primarily the liver and muscles. … Glycogen storage disease type III, which is also known as GSD III or Cori disease, is a rare inherited inborn error of metabolism (IEM). GSD-III has an incidence of about 1 in 100 000.
Drugs: Pyridoxal phosphate · NADH · ATP · Calcium · Magnesium cation · Uridine diphosphate glucose +4 moreGlycogenosis, Type III. Cori Disease, Debrancher Glycogenosis
smpdb.ca/view/SMP0125759DiseaseGSD III is divided into the types IIIa, IIIb, IIIc, and IIId, which are distinguished by their pattern of signs and symptoms. GSD types IIIa and IIIc affect primarily the liver and muscles. … Glycogen storage disease type III, which is also known as GSD III or Cori disease, is a rare inherited inborn error of metabolism (IEM). GSD-III has an incidence of about 1 in 100 000.
Drugs: Pyridoxal phosphate · NADH · ATP · Calcium · Magnesium cation · Uridine diphosphate glucose +4 more17-beta Hydroxysteroid Dehydrogenase III Deficiency
smpdb.ca/view/SMP0000356Disease17-beta hydroxysteroid dehydrogenase III deficiency, also known as 17-KSR deficiency or male pseudohermaphroditism with gynecomastia (MPH), is as rare inborn error of metabolism (IEM) and autosomal recessive … disorder of the androgen and estrogen metabolism pathway.
Congenital Bile Acid Synthesis Defect Type III
smpdb.ca/view/SMP0125690DiseaseSymptoms include severe cholestasis, cirrhosis, and liver failure. … Congenital Bile Acid Synthesis Defect Type III (CBASIII) is caused by a defect in 25-hydroxycholesterol 7-alpha-hydroxylase, which plays a role in synthesis of bile acids.
Glutaminolysis and Cancer
smpdb.ca/view/SMP0002298DiseaseThe transportation of glutamine in and out regulates mTOR activation to coordinate cell growth and proliferation. … Once it reaches malic acid, it is transported outside the mitochondria and transformed into pyruvic acid and lactate.
Drugs: Pyridoxal phosphate · Pyruvic acid · Biotin · Aspartic acid · L-Glutamine · Succinic acid +9 moreNicotinate and Nicotinamide Metabolism
smpdb.ca/view/SMP0000048MetabolicNicotinate (niacin) and nicotinamide - more commonly known as vitamin B3 - are precursors of the coenzymes nicotinamide-adenine dinucleotide (NAD+) and nicotinamide-adenine dinucleotide phosphate (NADP … The precursors are fed into the NAD+ biosynthetic pathwaythrough adenylation and phosphoribosylation reactions.
Fructose and Mannose Degradation
smpdb.ca/view/SMP0000064MetabolicFructose causes less of an insulin response compared to glucose and thus may be a preferred sugar for diabetics. … Fructose and mannose are monosaccharides that can be found in many foods. Fructose can join with glucose to form sucrose. Mannose can be converted to glucose. Both may be used as food sweeteners.
Androgen and Estrogen Metabolism
smpdb.ca/view/SMP0000068MetabolicThis pathway describes the inactivation and catabolism of male (androgen) and female (estrogen) hormones. … Many steroid hormones are transformed by sulfatases, dehydrogenases and glucuronide transferases to enhance their solubility and to facilitate their elimination.
NAD+ Signalling and Aging
smpdb.ca/view/SMP0084271SignalingAn interesting link has emerged between NAD+ metabolism, SIRT1, SIRT3, and mitochondrial function. … NR and PARP inhibitors increased life span in worms via activation of the mitochondrial unfolded protein response UPRmt by sir2.1.