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3-Methylglutaconic Aciduria Type IV
SMPDB_ID
SMP0000141
PW_ID
PW000214
图片
主题
Disease
描述
3-Methylglutaconic Aciduria Type IV (MGA, Type IV; MGA4) is an autosomal recessive disease caused by a mutation in an unknown gene. This disease results in an accumulation of cis and trans 3-methylglutaconic acid. Symptoms include, anemia, hyperammonemia, mental retardation, optic atrophy, hypotonia and early death.
代谢物
SMPDB ID代谢物id代谢物的名字
SMP0000141 PW_C040558 2Fe-2S
SMP0000141 PW_C001491 Molybdopterin
SMP0000141 PW_C001783 Hydrogen peroxide
SMP0000141 PW_C001065 Oxygen
SMP0000141 PW_C001401 Adenosylcobalamin
SMP0000141 PW_C000808 Succinyl-CoA
SMP0000141 PW_C001523 R-Methylmalonyl-CoA
SMP0000141 PW_C001104 Phosphate
SMP0000141 PW_C001560 S-Methylmalonyl-CoA
SMP0000141 PW_C001455 (S)-b-aminoisobutyric acid
SMP0000141 PW_C000020 Biotin
SMP0000141 PW_C001034 Adenosine diphosphate
SMP0000141 PW_C000463 Hydrogen carbonate
SMP0000141 PW_C000414 Adenosine triphosphate
SMP0000141 PW_C001420 Water
SMP0000141 PW_C002981 3-Hydroxyisovaleryl-CoA
SMP0000141 PW_C008511 Lipoyl-GMP
SMP0000141 PW_C001099 Coenzyme A
SMP0000141 PW_C001148 Pyridoxal 5'-phosphate
SMP0000141 PW_C001316 Carbon dioxide
SMP0000141 PW_C001188 Pyridoxal
SMP0000141 PW_C000095 L-Glutamic acid
SMP0000141 PW_C000134 Oxoglutaric acid
SMP0000141 PW_C000897 2-Methylacetoacetyl-CoA
SMP0000141 PW_C001047 2-Methyl-3-hydroxybutyryl-CoA
SMP0000141 PW_C001378 Tiglyl-CoA
SMP0000141 PW_C000820 2-Methylbutyryl-CoA
SMP0000141 PW_C002980 S-(2-Methylbutanoyl)-dihydrolipoamide
SMP0000141 PW_C000373 3-Methyl-2-oxovaleric acid
SMP0000141 PW_C000112 L-Isoleucine
SMP0000141 PW_C000988 Propionyl-CoA
SMP0000141 PW_C000130 Methylmalonic acid
SMP0000141 PW_C001500 (S)-Methylmalonic acid semialdehyde
SMP0000141 PW_C000016 (S)-3-Hydroxyisobutyric acid
SMP0000141 PW_C000829 (S)-3-Hydroxyisobutyryl-CoA
SMP0000141 PW_C000800 Methacrylyl-CoA
SMP0000141 PW_C000960 Isobutyryl-CoA
SMP0000141 PW_C002979 S-(2-Methylpropionyl)-dihydrolipoamide-E
SMP0000141 PW_C002977 2-Methyl-1-hydroxypropyl-ThPP
SMP0000141 PW_C000012 alpha-Ketoisovaleric acid
SMP0000141 PW_C000704 L-Valine
SMP0000141 PW_C001142 Acetoacetyl-CoA
SMP0000141 PW_C000042 Acetoacetic acid
SMP0000141 PW_C000940 Acetyl-CoA
SMP0000141 PW_C001063 3-Hydroxy-3-methylglutaryl-CoA
SMP0000141 PW_C000832 3-Methylglutaconyl-CoA
SMP0000141 PW_C001150 3-Methylcrotonyl-CoA
SMP0000141 PW_C000964 FAD
SMP0000141 PW_C040034 Hydrogen Ion
SMP0000141 PW_C001144 NADH
SMP0000141 PW_C000721 NAD
SMP0000141 PW_C000782 Dihydrolipoamide
SMP0000141 PW_C000870 Isovaleryl-CoA
SMP0000141 PW_C001060 Thiamine pyrophosphate
SMP0000141 PW_C002978 S-(3-Methylbutanoyl)-dihydrolipoamide-E
SMP0000141 PW_C000769 Lipoamide
SMP0000141 PW_C002976 3-Methyl-1-hydroxybutyl-ThPP
SMP0000141 PW_C000174 Succinic acid
SMP0000141 PW_C000540 L-Leucine
SMP0000141 PW_C000547 Ketoleucine
蛋白质
SMPDB ID蛋白质id蛋白质的名字
SMP0000141 P55809 Succinyl-CoA:3-ketoacid coenzyme A transferase 1, mitochondrial
SMP0000141 P24752 Acetyl-CoA acetyltransferase, mitochondrial
SMP0000141 P54868 Hydroxymethylglutaryl-CoA synthase, mitochondrial
SMP0000141 Q13825 Methylglutaconyl-CoA hydratase, mitochondrial
SMP0000141 P05091 Aldehyde dehydrogenase, mitochondrial
SMP0000141 Q06278 Aldehyde oxidase
SMP0000141 P22033 Methylmalonyl-CoA mutase, mitochondrial
SMP0000141 Q96PE7 Methylmalonyl-CoA epimerase, mitochondrial
SMP0000141 P05166 Propionyl-CoA carboxylase beta chain, mitochondrial
SMP0000141 P05165 Propionyl-CoA carboxylase alpha chain, mitochondrial
SMP0000141 Q02252 Methylmalonate-semialdehyde dehydrogenase [acylating], mitochondrial
SMP0000141 P42765 3-ketoacyl-CoA thiolase, mitochondrial
SMP0000141 Q99714 3-hydroxyacyl-CoA dehydrogenase type-2
SMP0000141 P80404 4-aminobutyrate aminotransferase, mitochondrial
SMP0000141 P31937 3-hydroxyisobutyrate dehydrogenase, mitochondrial
SMP0000141 Q6NVY1 3-hydroxyisobutyryl-CoA hydrolase, mitochondrial
SMP0000141 Q9UKU7 Isobutyryl-CoA dehydrogenase, mitochondrial
SMP0000141 P45954 Short/branched chain specific acyl-CoA dehydrogenase, mitochondrial
SMP0000141 P16219 Short-chain specific acyl-CoA dehydrogenase, mitochondrial
SMP0000141 Q9HCC0 Methylcrotonoyl-CoA carboxylase beta chain, mitochondrial
SMP0000141 Q96RQ3 Methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial
SMP0000141 P30084 Enoyl-CoA hydratase, mitochondrial
SMP0000141 P11310 Medium-chain specific acyl-CoA dehydrogenase, mitochondrial
SMP0000141 P26440 Isovaleryl-CoA dehydrogenase, mitochondrial
SMP0000141 P11182 Lipoamide acyltransferase component of branched-chain alpha-keto acid dehydrogenase complex, mitochondrial
SMP0000141 P54687 Branched-chain-amino-acid aminotransferase, cytosolic
SMP0000141 P35914 Hydroxymethylglutaryl-CoA lyase, mitochondrial
SMP0000141 P09622 Dihydrolipoyl dehydrogenase, mitochondrial
SMP0000141 P12694 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial
SMP0000141 P21953 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial

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