Entity Details

Primary name MAAI_HUMAN
Entity type UniProt
Source Source Link

Details

AccessionO43708
EntryNameMAAI_HUMAN
FullNameMaleylacetoacetate isomerase
TaxID9606
Evidenceevidence at protein level
Length216
SequenceStatuscomplete
DateCreated1998-12-15
DateModified2021-06-02

Ontological Relatives

GenesGSTZ1

GO terms

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GOName
GO:0004364 glutathione transferase activity
GO:0004602 glutathione peroxidase activity
GO:0005739 mitochondrion
GO:0005759 mitochondrial matrix
GO:0005829 cytosol
GO:0006559 L-phenylalanine catabolic process
GO:0006572 tyrosine catabolic process
GO:0006749 glutathione metabolic process
GO:0010510 regulation of acetyl-CoA biosynthetic process from pyruvate
GO:0016034 maleylacetoacetate isomerase activity
GO:0042802 identical protein binding
GO:0042803 protein homodimerization activity
GO:1901687 glutathione derivative biosynthetic process

Subcellular Location

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Subcellular Location
Cytoplasm

Domains

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DomainNameCategoryType
IPR004045 Glutathione S-transferase, N-terminalDomainDomain
IPR004046 Glutathione S-transferase, C-terminalDomainDomain
IPR005955 Glutathione S-transferases, class ZetaFamilyFamily
IPR010987 Glutathione S-transferase, C-terminal-likeDomainDomain
IPR034330 Glutathione S-transferases, class Zeta , C-terminalDomainDomain
IPR034333 Glutathione S-transferases, class Zeta , N-terminalDomainDomain
IPR036249 Thioredoxin-like superfamilyFamilyHomologous superfamily
IPR036282 Glutathione S-transferase, C-terminal domain superfamilyFamilyHomologous superfamily
IPR040079 Glutathione Transferase familyFamilyFamily

Diseases

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Disease IDSourceNameDescription
617596 OMIMMaleylacetoacetate isomerase deficiency (MAAID)An autosomal recessive inborn error of metabolism characterized by mild elevations in succinylacetone in blood and urine, usually identified by newborn screening. Liver function and coagulation are normal. MAAID is a benign disorder. The disease is caused by variants affecting the gene represented in this entry.

Drugs

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DrugNameSourceType
DB00143 GlutathioneDrugbanksmall molecule
DB04447 1,4-DithiothreitolDrugbanksmall molecule
DB08809 Dichloroacetic acidDrugbanksmall molecule
DB09462 GlycerinDrugbanksmall molecule