Entity Details

Primary name ECHP_HUMAN
Entity type UniProt
Source Source Link

Details

AccessionQ08426
EntryNameECHP_HUMAN
FullNamePeroxisomal bifunctional enzyme
TaxID9606
Evidenceevidence at protein level
Length723
SequenceStatuscomplete
DateCreated1994-10-01
DateModified2021-06-02

Ontological Relatives

GenesEHHADH

GO terms

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GOName
GO:0003857 3-hydroxyacyl-CoA dehydrogenase activity
GO:0004165 dodecenoyl-CoA delta-isomerase activity
GO:0004300 enoyl-CoA hydratase activity
GO:0005777 peroxisome
GO:0005782 peroxisomal matrix
GO:0005829 cytosol
GO:0006635 fatty acid beta-oxidation
GO:0008104 protein localization
GO:0016509 long-chain-3-hydroxyacyl-CoA dehydrogenase activity
GO:0016863 intramolecular oxidoreductase activity, transposing C=C bonds
GO:0019899 enzyme binding
GO:0033540 fatty acid beta-oxidation using acyl-CoA oxidase
GO:0070403 NAD+ binding

Subcellular Location

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Subcellular Location
Peroxisome

Domains

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DomainNameCategoryType
IPR001753 Enoyl-CoA hydratase/isomeraseFamilyFamily
IPR006108 3-hydroxyacyl-CoA dehydrogenase, C-terminalDomainDomain
IPR006176 3-hydroxyacyl-CoA dehydrogenase, NAD bindingDomainDomain
IPR006180 3-hydroxyacyl-CoA dehydrogenase, conserved siteSiteConserved site
IPR008927 6-phosphogluconate dehydrogenase-like, C-terminal domain superfamilyFamilyHomologous superfamily
IPR018376 Enoyl-CoA hydratase/isomerase, conserved siteSiteConserved site
IPR029045 ClpP/crotonase-like domain superfamilyFamilyHomologous superfamily
IPR036291 NAD(P)-binding domain superfamilyFamilyHomologous superfamily

Diseases

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Disease IDSourceNameDescription
615605 OMIMFanconi renotubular syndrome 3 (FRTS3)A form of Fanconi renotubular syndrome, a disease due to a generalized dysfunction of the proximal kidney tubule resulting in decreased solute and water reabsorption. Patients have polydipsia and polyuria with phosphaturia, glycosuria and aminoaciduria. They may develop hypophosphatemic rickets or osteomalacia, acidosis and a tendency toward dehydration. Some eventually develop renal insufficiency. FRTS3 inheritance is autosomal dominant. The disease is caused by variants affecting the gene represented in this entry.

Drugs

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DrugNameSourceType
DB00157 NADHDrugbanksmall molecule

Interactions

120 interactions

InteractorPartnerSourcesPublicationsLink
ECHP_HUMANTS101_HUMANIntAct19549727 details
ECHP_HUMANC102B_HUMANBioGRID, IntAct25416956 31515488 details
ECHP_HUMANNECA2_HUMANBioGRID, IntAct25416956 31515488 details
ECHP_HUMANBHE40_HUMANBioGRID, IntAct25416956 details
ECHP_HUMANTRI18_HUMANBioGRID, IntAct25416956 31515488 32296183 details
ECHP_HUMANSSNA1_HUMANBioGRID, IntAct25416956 31515488 details
ECHP_HUMANTRI27_HUMANBioGRID, IntAct25416956 32296183 details
ECHP_HUMANDESM_HUMANBioGRID, IntAct25416956 32296183 details
ECHP_HUMANTPP2_HUMANBioGRID, IntAct25416956 31515488 details
ECHP_HUMANCBS_HUMANBioGRID, IntAct25416956 31515488 details
ECHP_HUMANKR107_HUMANBioGRID, IntAct25416956 details
ECHP_HUMANACTG_HUMANBioGRID, IntAct25416956 31515488 details
ECHP_HUMANTRAF1_HUMANBioGRID, IntAct25416956 details
ECHP_HUMANTNIP1_HUMANBioGRID, IntAct25416956 31515488 details
ECHP_HUMANKCTD9_HUMANBioGRID, IntAct25416956 31515488 details
ECHP_HUMANFUND1_HUMANBioGRID, IntAct25416956 31515488 details
ECHP_HUMANKCTD6_HUMANBioGRID, IntAct25416956 31515488 details
ECHP_HUMANPNMA1_HUMANBioGRID, IntAct25416956 details
ECHP_HUMANTRI41_HUMANBioGRID, IntAct25416956 31515488 32296183 details
ECHP_HUMANZBTB9_HUMANBioGRID, IntAct25416956 32296183 details
ECHP_HUMANLZTS2_HUMANBioGRID, IntAct25416956 details
ECHP_HUMANTRI54_HUMANBioGRID, IntAct25416956 details
ECHP_HUMANADIP_HUMANBioGRID, IntAct25416956 details
ECHP_HUMANZRAN1_HUMANBioGRID, IntAct32296183 details
ECHP_HUMANCDR2L_HUMANBioGRID, IntAct32296183 details
ECHP_HUMANPP12C_HUMANBioGRID, IntAct32296183 details
ECHP_HUMANPRKRA_HUMANBioGRID, IntAct32296183 details
ECHP_HUMANRED1_HUMANBioGRID, IntAct32296183 details
ECHP_HUMANMTUS2_HUMANBioGRID, IntAct30886144 32296183 details
ECHP_HUMANSMN_HUMANBioGRID, IntAct32296183 details
ECHP_HUMANBOLL_HUMANBioGRID, IntAct32296183 details
ECHP_HUMANGRAP1_HUMANBioGRID, IntAct32296183 details
ECHP_HUMANNACC1_HUMANBioGRID, IntAct32296183 details
ECHP_HUMANZBT8A_HUMANBioGRID, IntAct32296183 details
ECHP_HUMANRO52_HUMANBioGRID, IntAct32296183 details
ECHP_HUMANREL_HUMANBioGRID, IntAct32296183 details
ECHP_HUMANPICK1_HUMANBioGRID, IntAct32296183 details
ECHP_HUMANZBT26_HUMANBioGRID, IntAct32296183 details
ECHP_HUMANZBT10_HUMANBioGRID, IntAct32296183 details
ECHP_HUMANPNMA5_HUMANBioGRID, IntAct32296183 details
ECHP_HUMANREEP6_HUMANIntAct32296183 details
ECHP_HUMANCOF2_HUMANIntAct32296183 details
ECHP_HUMANANM5_HUMANIntAct32296183 details
ECHP_HUMANIKZF3_HUMANIntAct32296183 details
ECHP_HUMANCA050_HUMANIntAct32296183 details
ECHP_HUMANTRAF2_HUMANIntAct32296183 details
ECHP_HUMANSNG1_HUMANIntAct32296183 details
ECHP_HUMANHM20A_HUMANIntAct32296183 details
ECHP_HUMANSYAM_HUMANBioGRID, IntAct32296183 details
ECHP_HUMANDBLOH_HUMANIntAct32296183 details
ECHP_HUMANSYCY2_HUMANIntAct32296183 details
ECHP_HUMANERGI3_HUMANIntAct32296183 details
ECHP_HUMANLERL1_HUMANBioGRID, IntAct32296183 details
ECHP_HUMANSSMM1_HUMANIntAct32296183 details
ECHP_HUMANUBXN1_HUMANIntAct32296183 details
ECHP_HUMANTNR3_HUMANIntAct32296183 details
ECHP_HUMANSFT2A_HUMANIntAct32296183 details
ECHP_HUMANEBP_HUMANIntAct32296183 details
ECHP_HUMANCAN3_HUMANIntAct32296183 details
ECHP_HUMANFGF14_HUMANBioGRID, IntAct32296183 details
ECHP_HUMANRPRM_HUMANBioGRID, IntAct32296183 details
ECHP_HUMANSCAR5_HUMANBioGRID, IntAct32296183 details
ECHP_HUMANSMIM5_HUMANBioGRID, IntAct32296183 details
ECHP_HUMANCISD2_HUMANBioGRID, IntAct32296183 details
ECHP_HUMANTRIM5_HUMANBioGRID, IntAct32296183 details
ECHP_HUMANSGCB_HUMANBioGRID, IntAct32296183 details
ECHP_HUMANS39A2_HUMANBioGRID, IntAct32296183 details
ECHP_HUMANSNG3_HUMANBioGRID, IntAct32296183 details
ECHP_HUMANHTAI2_HUMANBioGRID, IntAct32296183 details
ECHP_HUMANCKLF6_HUMANBioGRID, IntAct32296183 details
ECHP_HUMANRUSF1_HUMANBioGRID, IntAct32296183 details
ECHP_HUMANNACHO_HUMANBioGRID, IntAct32296183 details
ECHP_HUMANMB213_HUMANBioGRID, IntAct32296183 details
ECHP_HUMANDHB11_HUMANBioGRID, IntAct32296183 details
ECHP_HUMANCLD5_HUMANBioGRID, IntAct32296183 details
ECHP_HUMANCLD18_HUMANBioGRID, IntAct32296183 details
ECHP_HUMANERGI1_HUMANBioGRID, IntAct32296183 details
ECHP_HUMANTRI63_HUMANBioGRID, IntAct18157088 32296183 details
ECHP_HUMANEMP1_HUMANBioGRID, IntAct32296183 details
ECHP_HUMANPTGES_HUMANBioGRID, IntAct32296183 details
ECHP_HUMANJAGN1_HUMANBioGRID, IntAct32296183 details
ECHP_HUMANCEP83_HUMANBioGRID, IntAct32296183 details
ECHP_HUMANCYAC3_HUMANBioGRID, IntAct32296183 details
ECHP_HUMANDERL3_HUMANBioGRID, IntAct32296183 details
ECHP_HUMANVAC14_HUMANBioGRID, IntAct32296183 details
ECHP_HUMANGMCL1_HUMANBioGRID, IntAct32296183 details
ECHP_HUMANPPIP1_HUMANBioGRID, IntAct32296183 details
ECHP_HUMANTRI23_HUMANBioGRID, IntAct32296183 details
ECHP_HUMANHIP1_HUMANBioGRID, IntAct32296183 details
ECHP_HUMANACTB_HUMANBioGRID, IntAct25416956 31515488 32296183 details
ECHP_HUMANOPRM_HUMANBioGRID, IntAct32296183 details
ECHP_HUMANKR411_HUMANBioGRID, IntAct32296183 details
ECHP_HUMANPAQR8_HUMANBioGRID, IntAct32296183 details
ECHP_HUMANORML1_HUMANBioGRID, IntAct32296183 details
ECHP_HUMANCCD13_HUMANBioGRID, IntAct32296183 details
ECHP_HUMANDRC4_HUMANBioGRID, IntAct32296183 details
ECHP_HUMANNGAL_HUMANBioGRID, IntAct32296183 details
ECHP_HUMANTM121_HUMANBioGRID, IntAct32296183 details
ECHP_HUMANUT2_HUMANBioGRID, IntAct32296183 details
ECHP_HUMANNO40_HUMANBioGRID, IntAct32296183 details
ECHP_HUMANAN36B_HUMANBioGRID, IntAct32296183 details
ECHP_HUMANCOQ9_HUMANBioGRID, IntAct32296183 details
ECHP_HUMANCLK2_HUMANBioGRID, IntAct32296183 details
ECHP_HUMANHID1_HUMANBioGRID, IntAct32296183 details
ECHP_HUMANLZTL1_HUMANBioGRID, IntAct32296183 details
ECHP_HUMANARMX3_HUMANBioGRID, IntAct32296183 details
ECHP_HUMANLYPD5_HUMANBioGRID, IntAct32296183 details
ECHP_HUMANTMM17_HUMANBioGRID, IntAct32296183 details
ECHP_HUMANRNF4_HUMANBioGRID, IntAct32296183 details
ECHP_HUMANRDHE2_HUMANBioGRID, IntAct32296183 details
ECHP_HUMANARM12_HUMANBioGRID, IntAct32296183 details
ECHP_HUMANCY561_HUMANBioGRID, IntAct32296183 details
ECHP_HUMANTRIM1_HUMANIntAct30886144 details
ECHP_HUMANCATA_HUMANBioGRID, IntAct16781659 29568061 details
ECHP_HUMANPEX5_HUMANBioGRID20178365 22002062 30378028 details
ECHP_HUMANTRI55_HUMANBioGRID18157088 details
ECHP_HUMANCBSL_HUMANBioGRID25416956 details
ECHP_HUMANDAAM2_HUMANBioGRID32296183 details
ECHP_HUMANTMM31_HUMANBioGRID32296183 details
ECHP_HUMANSCP2_HUMANHPRD10856712 details