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Gamma-butyrobetaine dioxygenase

Gamma-butyrobetaine dioxygenase (also known as BBOX, GBBH or γ-butyrobetaine hydroxylase) is an enzyme that in humans is encoded by the BBOX1 gene. Gamma-butyrobetaine dioxygenase catalyses the formation of L-carnitine from gamma-butyrobetaine, the last step in the L-carnitine biosynthesis pathway. Carnitine is essential for the transport of activated…

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1.14.11.1
↗
Aliases
BBOX1, BBH, BBOX, G-BBH, gamma-BBH, gamma-butyrobetaine hydroxylase 1
Available structures
Available structuresPDBOrtholog search: PDBe RCSB List of PDB id codes3MS5, 3N6W, 3O2G, 4BG1, 4BGK, 4BGM, 4BHF, 4BHG, 4BHI, 4C5W, 4C8R, 4CWD
Band
11p14.2 · 2|2 E3
Bgee
kidney tubule · glomerulus · human kidney · gingival epithelium · metanephric glomerulus
BioGPS
More reference expression data

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Overview

Reaction

Inhibition

Assay

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Map overview Semantic statistics

Gamma-butyrobetaine dioxygenase

Nodes71
Edges70
Triples113
Avg. degree1.97
Density0.028169
Components1

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Gamma-butyrobetaine dioxygenase

Top relations

Bgee · 20
Gamma-butyrobetaine dioxygenase → deep cerebellar nuclei, epidermis, external globus pallidus, gingival epithelium, glomerulus, hair follicle, human kidney, kidney tubule, left lobe of liver, lip, lumbar subsegment of spinal cord, metanephric glomerulus, renal medulla, skin of arm, skin of back, skin of external ear, skin of hip, skin of thigh, substantia nigra, vestibular membrane of cochlear duct
RNA expression pattern · 20
Gamma-butyrobetaine dioxygenase → deep cerebellar nuclei, epidermis, external globus pallidus, gingival epithelium, glomerulus, hair follicle, human kidney, kidney tubule, left lobe of liver, lip, lumbar subsegment of spinal cord, metanephric glomerulus, renal medulla, skin of arm, skin of back, skin of external ear, skin of hip, skin of thigh, substantia nigra, vestibular membrane of cochlear duct
related to Inhibition · 16
Gamma-butyrobetaine dioxygenase → Although, C-4, CH2, Gamma-butyrobetaine, Grindeks, However, Latvia, Meldonium, MET-88, Mildronate, NH, PDB, Quarterine, Some, THP, To
Gene ontology · 14
Gamma-butyrobetaine dioxygenase → carnitine biosynthetic process, cytoplasm, cytosol, dioxygenase activity, extracellular exosome, gamma-butyrobetaine dioxygenase activity, identical protein binding, iron ion binding, metal ion binding, mitochondrion, oxidoreductase activity, oxidoreductase activity, acting on single donors with incorporation of molecular oxygen, incorporation of two atoms of oxygen, protein binding, zinc ion binding
related to Assay · 8
Gamma-butyrobetaine dioxygenase → Early, Enzyme-coupled, However, NMR, Other, Several, The, Using
related to Reaction · 7
Gamma-butyrobetaine dioxygenase → Ascorbic, Fe, Gamma-butyrobetaine, It, IV, L-carnitine, The
Band · 2
Gamma-butyrobetaine dioxygenase → 11p14.2, 2|2 E3
Chr. · 2
Gamma-butyrobetaine dioxygenase → Chromosome 11 (human), Chromosome 2 (mouse)
End · 2
Gamma-butyrobetaine dioxygenase → 110,144,905 bp, 27,127,809 bp
1.14.11.1 · 1
Gamma-butyrobetaine dioxygenase → ↗

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Important terminology

gamma-butyrobetaine dioxygenase mildronate enzyme also human gene bp 2og substrate carnitine bbox1 chr band l-carnitine oxidation acid catalyses activity 11

Entity relationships Subject–Predicate–Object triples

SubjectPredicateObjectConfidenceSrc
Gamma-butyrobetaine dioxygenase1.14.11.1↗1.00infobox
Gamma-butyrobetaine dioxygenaseAliasesBBOX1, BBH, BBOX, G-BBH, gamma-BBH, gamma-butyrobetaine hydroxylase 11.00infobox
Gamma-butyrobetaine dioxygenaseAvailable structuresAvailable structuresPDBOrtholog search: PDBe RCSB List of PDB id codes3MS5, 3N6W, 3O2G, 4BG1, 4BGK, 4BGM, 4BHF, 4BHG, 4BHI, 4C5W, 4C8R, 4CWD1.00infobox
Gamma-butyrobetaine dioxygenaseBand11p14.21.00infobox
Gamma-butyrobetaine dioxygenaseBand2|2 E31.00infobox
Gamma-butyrobetaine dioxygenaseBgeekidney tubule1.00infobox
Gamma-butyrobetaine dioxygenaseBgeeglomerulus1.00infobox
Gamma-butyrobetaine dioxygenaseBgeehuman kidney1.00infobox
Gamma-butyrobetaine dioxygenaseBgeegingival epithelium1.00infobox
Gamma-butyrobetaine dioxygenaseBgeemetanephric glomerulus1.00infobox
Gamma-butyrobetaine dioxygenaseBgeeskin of thigh1.00infobox
Gamma-butyrobetaine dioxygenaseBgeerenal medulla1.00infobox
Gamma-butyrobetaine dioxygenaseBgeeskin of hip1.00infobox
Gamma-butyrobetaine dioxygenaseBgeeexternal globus pallidus1.00infobox
Gamma-butyrobetaine dioxygenaseBgeeskin of arm1.00infobox
Gamma-butyrobetaine dioxygenaseBgeevestibular membrane of cochlear duct1.00infobox
Gamma-butyrobetaine dioxygenaseBgeeleft lobe of liver1.00infobox
Gamma-butyrobetaine dioxygenaseBgeeskin of external ear1.00infobox
Gamma-butyrobetaine dioxygenaseBgeelumbar subsegment of spinal cord1.00infobox
Gamma-butyrobetaine dioxygenaseBgeelip1.00infobox
Gamma-butyrobetaine dioxygenaseBgeeskin of back1.00infobox
Gamma-butyrobetaine dioxygenaseBgeedeep cerebellar nuclei1.00infobox
Gamma-butyrobetaine dioxygenaseBgeeepidermis1.00infobox
Gamma-butyrobetaine dioxygenaseBgeesubstantia nigra1.00infobox
Gamma-butyrobetaine dioxygenaseBgeehair follicle1.00infobox
Gamma-butyrobetaine dioxygenaseBioGPSMore reference expression data1.00infobox
Gamma-butyrobetaine dioxygenaseChr.Chromosome 11 (human)1.00infobox
Gamma-butyrobetaine dioxygenaseChr.Chromosome 2 (mouse)1.00infobox
Gamma-butyrobetaine dioxygenaseDatabasesNCBI: entry; OMA: entry1.00infobox
Gamma-butyrobetaine dioxygenaseEnd27,127,809 bp1.00infobox

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