SMPDB_ID

SMP0000219

PW_ID

PW000188

图片

主题

Disease

描述

Orotic Aciduria (Orotic acidemia) is caused by a defect in uridine monophosphate synthetase (UMPS) (orotate phosphoribosyl transferase and orotidine-5’-decarboxylase), an enzyme that catalyses the formation of uridine monophosphate (UMP), an energy carrying molecule in many important biosynthetic pathways. This disease is marked by very high accumulation of orotic acid in the urine, sometimes causing urinary obstruction. Symptoms include megaloblastic anemia as well as failure to thrive.

代谢物

SMPDB ID	代谢物id	代谢物的名字
SMP0000219	PW_C001441	Phosphoric acid
SMP0000219	PW_C001993	3-Aminoisobutanoic acid
SMP0000219	PW_C000792	dUDP
SMP0000219	PW_C000927	Deoxyuridine triphosphate
SMP0000219	PW_C001363	Ureidoisobutyric acid
SMP0000219	PW_C000056	Dihydrothymine
SMP0000219	PW_C000179	Thymine
SMP0000219	PW_C000187	Thymidine
SMP0000219	PW_C001035	Thymidine 5'-triphosphate
SMP0000219	PW_C000987	dTDP
SMP0000219	PW_C000831	Dihydrofolic acid
SMP0000219	PW_C000950	5-Thymidylic acid
SMP0000219	PW_C001178	5,10-Methylene-THF
SMP0000219	PW_C009795	Zinc
SMP0000219	PW_C001087	dUMP
SMP0000219	PW_C001042	Deoxyribose 1-phosphate
SMP0000219	PW_C000007	Deoxyuridine
SMP0000219	PW_C000008	Deoxycytidine
SMP0000219	PW_C000937	dCMP
SMP0000219	PW_C000544	Fe2+
SMP0000219	PW_C000962	dCDP
SMP0000219	PW_C001316	Carbon dioxide
SMP0000219	PW_C000040	beta-Alanine
SMP0000219	PW_C000018	Ureidopropionic acid
SMP0000219	PW_C000964	FAD
SMP0000219	PW_C000146	NADPH
SMP0000219	PW_C000053	Dihydrouracil
SMP0000219	PW_C000143	NADP
SMP0000219	PW_C001146	Ribose 1-phosphate
SMP0000219	PW_C000204	Uracil
SMP0000219	PW_C000035	Ammonia
SMP0000219	PW_C000202	Uridine
SMP0000219	PW_C000061	Cytidine
SMP0000219	PW_C000064	Cytidine monophosphate
SMP0000219	PW_C001189	CDP
SMP0000219	PW_C000790	dCTP
SMP0000219	PW_C000057	Cytidine triphosphate
SMP0000219	PW_C000170	Pyrophosphate
SMP0000219	PW_C001918	3'-AMP
SMP0000219	PW_C000423	Magnesium
SMP0000219	PW_C000192	Uridine triphosphate
SMP0000219	PW_C000353	Calcium
SMP0000219	PW_C000201	Uridine 5'-diphosphate
SMP0000219	PW_C000194	Uridine 5'-monophosphate
SMP0000219	PW_C000144	Orotidylic acid
SMP0000219	PW_C000190	Phosphoribosyl pyrophosphate
SMP0000219	PW_C001170	Flavin Mononucleotide
SMP0000219	PW_C000151	Orotic acid
SMP0000219	PW_C001860	Quinone
SMP0000219	PW_C001852	L-Dihydroorotic acid
SMP0000219	PW_C000662	Ureidosuccinic acid
SMP0000219	PW_C001005	Zinc (II) ion
SMP0000219	PW_C001104	Phosphate
SMP0000219	PW_C001034	Adenosine diphosphate
SMP0000219	PW_C000862	Carbamoyl phosphate
SMP0000219	PW_C000463	Hydrogen carbonate
SMP0000219	PW_C001420	Water
SMP0000219	PW_C000414	Adenosine triphosphate
SMP0000219	PW_C000500	L-Glutamine

蛋白质

SMPDB ID	蛋白质id	蛋白质的名字
SMP0000219	P33316	Deoxyuridine 5'-triphosphate nucleotidohydrolase, mitochondrial
SMP0000219	P04818	Thymidylate synthase
SMP0000219	P04183	Thymidine kinase, cytosolic
SMP0000219	P32321	Deoxycytidylate deaminase
SMP0000219	P19971	Thymidine phosphorylase
SMP0000219	P32320	Cytidine deaminase
SMP0000219	Q9NWZ5	Uridine-cytidine kinase-like 1
SMP0000219	Q7LG56	Ribonucleoside-diphosphate reductase subunit M2 B
SMP0000219	P31350	Ribonucleoside-diphosphate reductase subunit M2
SMP0000219	Q9UBR1	Beta-ureidopropionase
SMP0000219	Q14117	Dihydropyrimidinase
SMP0000219	Q12882	Dihydropyrimidine dehydrogenase [NADP(+)]
SMP0000219	O95045	Uridine phosphorylase 2
SMP0000219	Q9Y2T3	Guanine deaminase
SMP0000219	P49902	Cytosolic purine 5'-nucleotidase
SMP0000219	P17812	CTP synthase 1
SMP0000219	Q9BY32	Inosine triphosphate pyrophosphatase
SMP0000219	Q9UIJ7	GTP:AMP phosphotransferase, mitochondrial
SMP0000219	O75414	Nucleoside diphosphate kinase 6
SMP0000219	Q5EBM0	UMP-CMP kinase 2, mitochondrial
SMP0000219	Q8WVQ1	Soluble calcium-activated nucleotidase 1
SMP0000219	A8K5J1	Uridine monophosphate synthetase isoform A
SMP0000219	Q02127	Dihydroorotate dehydrogenase (quinone), mitochondrial
SMP0000219	P27708	CAD protein